Atlas of Clinical Dermatology in Coloured Skin

This Atlas of Dermatology has a unique clinical orientation and lays emphasis on morphology. The disease entities are arranged
according to the morphology of lesions, not etiologically. This arrangement of disease entities is more useful for both teaching
and learning and serves as an important aid in making a diagnosis, as this approach is based on the way patients present them-
selves in out-patient departments. Key diagnostic clinical pointers too have been provided with images, which help one suspect
the diagnosis and exclude clinical differentials. This Atlas would serve as a desk reference for residents and practitioners.

Key Features

● An atlas and text covering skin, mucosa, hair and nail diseases in skin of color
● Emphasis on key diagnostic clinical pointers
● 3000+ clinical images—multiple images of a disease to cover the clinical spectrum
● Dedicated section on Regional Dermatology and Skin changes in systemic diseases
● Clinician’s desk reference for OPD practice
Atlas of Clinical Dermatology in
Coloured Skin
A Morphological Approach

Edited by
PC Das and Piyush Kumar
First edition published 2023
by CRC Press
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and by CRC Press
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Library of Congress Cataloging‑in‑Publication Data

Names: Das, P. C. (Dermatologist), editor. | Kumar, Piyush, 1982- editor.

Title: Atlas of clinical dermatology in coloured skin : a morphological approach / edited by P C Das and
Piyush Kumar.
Description: First edition. | Boca Raton : CRC Press, 2023. | Includes bibliographical references and index.
Identifiers: LCCN 2022033961 (print) | LCCN 2022033962 (ebook) | ISBN 9781138483682 (hardback) |
ISBN 9781032399669 (paperback) | ISBN 9781351054225 (ebook)
Subjects: MESH: Skin Diseases--diagnosis | Skin Diseases--ethnicity | Skin Pigmentation | Skin Manifestations |
Diagnosis, Differential | Atlas
Classification: LCC RL71 (print) | LCC RL71 (ebook) | NLM WR 17 | DDC 616.5--dc23/eng/20221128
LC record available at https://lccn.loc.gov/2022033961
LC ebook record available at https://lccn.loc.gov/2022033962

ISBN: 9781138483682 (hbk)

ISBN: 9781032399669 (pbk)
ISBN: 9781351054225 (ebk)

DOI: 10.1201/9781351054225

Typeset in Minion
by KnowledgeWorks Global Ltd.

Access the companion website: https://routledgetextbooks.com/textbooks/9781032399669/

Dedicated to my father, late Sri Surya Narayan Das, who had inherent scientific temperament,
exceptional observational capacity and intent learning interest
– PC Das
Dedicated to my parents and teachers, who showed me the path of learning, and to my patients,
who kept me walking on this path
– Piyush Kumar
Contents

Foreword by Debabrata Bandyopadhyay xii

Foreword by Eckart Haneke xiii
Preface xiv
Acknowledgements xvi
Editors xvii
Assistant editors xviii
Contributors xix

SECTION 1  INTRODUCTION TO CLINICAL DERMATOLOGY 1

E1 Overview of skin lesions 2

Chandra Sekhar Sirka, Swetalina Pradhan, Kananbala Sahu, Arpita Nibedita Rout

SECTION 2  APPROACH TO CLINICAL DIAGNOSIS 3

Piyush Kumar, PC Das

1 Hypopigmented and depigmented macules and patches: Localized 5

PC Das, Priya Rajbansh

E2 Hypopigmented and depigmented macules and patches: Generalized 18

PC Das, Mamta Yadav

2 Hyperpigmented macules and patches: Localized 19

PC Das, Danish Akhtar

3 Acquired facial melanosis 39

Preeti Sharma, Anupam Das

E3 Hyperpigmented macules and patches: Generalized 56

PC Das, Talat Fatima, Niharika Ranjan Lal

E4 Diffuse hyperpigmentation 57
Rampal Sharma

4 Reticulate and mottled pigmentation 58

Saumya Panda, Rashid Shahid

5 Papules: Localized 76
Niharika Ranjan Lal

6 Facial papules 100

Piyush Kumar, Rizwana Barkat
E5 Papules: Generalized 141
Niharika Ranjan Lal

vii
viii Contents

E6 Follicular papules 142

Ramesh Chandra Gharami

7 Plaques: Localized 143

Anirban Das

8 Plaques: Generalized 180

Dhiraj Kumar

9 Nodules: Localized 202

Aseem Sharma, Trupti Agarwal, Manish Khandare, Madhulika Mhatre

E7 Nodules: Generalized 233

Swetalina Pradhan, Kananbala Sahu

E8 Tumors 234
PC Das

10 Vesicobullous lesions: Localized 235

Niharika Ranjan Lal

11 Vesicobullous lesions: Generalized 254

Riti Bhatia and Vishal Gupta

12 Pustules: Localized 276

Madhulika Mhatre, Aseem Sharma, Trupti Agarwal

E9 Pustules: Generalized 291

Aseem Sharma, Madhulika Mhatre, Trupti Agarwal

E10 Clinical approach to ulcers 292

Sourabh Jain

13 Ulcer: Single or few 293

B Shiny Shulamite, Ch Venkata Krishna, Sri Harsha Kolla, Sai Prasanth Neti, Vignasree A

E11 Leg ulcers 310

Piyush Kumar, Komal Agarwal

E12 Ulcers: Multiple 311

Piyush Kumar, Rashmi Roy

E13 Scales: Localized 312

PC Das

E14 Scales: Generalized 313

Avijit Mondal
E15 Atrophy 314
Chirag Desai, Rashid Shahid

14 Purpura 315
Santoshdev P Rathod

E16 Erythema: Localized 327

Aparajita Ghosh, PC Das

E17 Erythema: Generalized 328

Swetalina Pradhan, Arpita Nibedita Rout

E18 Telangiectasia 329

Sunil K Kothiwala, Kanya Rani Vashisht

E19 Cysts and pseudocysts 330

PC Das

15 Draining sinuses and fistulas 331

Sunil K Kothiwala, Kanya Rani Vashisht
 Contents ix

16 Eschar 343
Sudhir Singh

17 Cutaneous horn 357

Satish S Savant, Sushil S Savant

SECTION 3  REGIONAL DERMATOLOGY 360

Piyush Kumar, PC Das

E20 Scalp 361

PC Das

E21 Periorbital area 362

Anup Kumar Tiwary

E22 Ears 363

Divya Sachdev

E23 Nose 364

Divya Sachdev

18 Oral mucosa 365

Sunil Kothiwala, Kanya Rani Vashisht

E24 Tongue 403

Meenaz Khoja, Sabha Mushtaq

E25 Axillae and groins 404

Shvetha Jain

E26 Umbilicus and periumbilical region 405

Divya Sachdev

E27 Palms and soles 406

Prerna, Rashid Shahid

19 Male genitalia 407

Piyush Kumar, Rashid Shahid

E28 Female genitalia 440

Madhulika Mhatre, Aseem Sharma

E29 Nipple areola complex 441

Amarkant Jha Amar, Shivani Sharma

E30 Scrotum 442

Dilip Kumar SA

E31 Seborrheic area 443

Pooja Nupur

E32 Intertriginous areas 444

Pooja Nupur, Shahid Hassan

20 Nails 445
Piyush Kumar

SECTION 4  SKIN IN SYSTEMIC DISEASES 472

PC Das, Piyush Kumar

21 Nutritional deficiency disorders 473

Chirag Desai, Piyush Kumar

E33 Endocrine disorders 488

Vikas Pathania, Sunmeet Sandhu
x Contents

E34 Renal disorders 489

Dependra Kumar Timshina, Vishal Golay

E35 Autoimmune rheumatic diseases 490

Anup Kumar Tiwary

E36 Hereditary disorders of connective tissue 491

Ravi Hiremagalore, Piyush Kumar

E37 Hepatic diseases 492

Gautam Kumar Singh

E38 Cardiovascular diseases 493

Preema Sinha, Anwita Sinha

E39 Neurocutaneous conditions 494

Satyaki Ganguly

22 Psychocutaneous disorders 495

Swetalina Pradhan, Gaurav Dash

E40 Dermatoses in HIV-infected persons 506

Santoshdev P Rathod

E41 Internal malignancy 507

Shekhar Neema, Dipali Rathod

E42 Nails in systemic disease 508

Balachandar S Ankad, Balakrishna Nikam

SECTION 5  MISCELLANEOUS 509

E43 Head and neck mass 510

Sasi Kiran Attili

E44 Red face 511

Abhishek Jha, Shivani Sharma

E45 Leonine facies 512

Sourabh Jain

E46 Linear lesions 513

Anupam Das, Preeti Sharma

23 Annular lesions 514

Piyush Kumar, Rashmi Roy

24 Alopecia 541
Piyush Kumar, Priya Rajbansh

E47 Hypertrichosis and hirsutism 570

B Shiny Shulamite

25 Vascular lesions 571

Sunil Kumar Gupta

E48 Vasculitis 590

Sunil Kumar Gupta

E49 Neonatal dermatology 591

Manjunath M Shenoy, Amina Asfiya MI, Ghaliyah Aziz Kutty

E50 Geriatric dermatoses 592

Rajesh Kumar Mandal

E51 Dermatoses of pregnancy 593

Swetalina Pradhan, Gaurav Dash
 Contents xi

26 Cutaneous adverse drug reactions 594

Abanti Saha, Amrita Sil, Nilay Kanti Das

E52 Dermatitis artefacta, dermatitis neglecta, and terra firma-forme dermatosis 610
Saumya Panda, Rashid Shahid

E53 Pruritus Sine Materia 611

Anuradha Priyadarshini

E54 Hyperhidrosis 612

Ankan Gupta, Navya Handa

E55 Anhidrosis 613

Sabha Mushtaq

27 Photosensitivity in children 614

Resham Vasani

E56 Photosensitivity in adults 629

Santoshdev P Rathod, Kalgi Baxi

E57 Maculopapular rash 630

Preeti Sharma

E58 Fever with rash in children 631

Bhumesh Kumar Katakam

Index 632
Foreword by Debabrata Bandyopadhyay

During the past couple of decades, sweeping changes have clinical dermatology with the major emphasis on the mor-
occurred in all aspects of medicine. We have witnessed rapid phologic and regional basis of differential diagnosis of the
expansion of knowledge about the molecular biology, genet- dermatoses. The coterie of mostly bright young dermatolo-
ics, and immunology of diseases, resulting in revolution- gists (many of whom I had the opportunity to teach and
ary transformation in the way diseases are diagnosed and learn from) have done a superb job with their contributions,
managed. In this milieu of explosive change, the core com- covering a wide spectrum of clinical topics, the arrangement
petence in the specialty of dermatology has still remained of text reflecting the clinical orientation of the authors.
largely dependent on the clinical aspects of the diseases. I have found the text to be a user-friendly, rapidly acces-
It is a specialty that frequently allows the physician to sible source of information on dermatologic presentations
make a correct diagnosis based solely on history and physi- and diseases. Therefore, the book will be helpful not only
cal examination. The easy visibility and accessibility of the to dermatology residents and practicing dermatologists but
whole organ helps the clinician in this respect. This is the also to physicians in other allied fields of medicine, partic-
beauty, as well as the challenge, of the practice of this branch ularly internal medicine, family medicine, pediatrics, and
of medicine as there exists hundreds of named disease enti- rheumatology.
ties, and the clinician has to interpret a wide variety of clini-
cal signs dependent on the morphology and distribution of Debabrata Bandyopadhyay
the lesions to pinpoint a diagnosis. Former Professor & Head
It is with great pleasure that I welcome a new addition Dept. of Dermatology, Venereology, & Leprosy
to dermatological literature that approaches the subject of Medical College & Hospital, Kolkata

xii
Foreword by Eckart Haneke

Differential diagnosis in dermatology is often very chal- Skin, hair, and nail disorders in systemic disease follow.
lenging, sometimes not even possible without further labo- This will satisfy the needs of all those colleagues who are
ratory investigations. Skin type may be a confounding factor – convinced that the skin is the mirror of the soul and the
dark skin is difficult for practitioners used to fair-skinned internal organs. Indeed, virtually no organ system or tissue
patients as most skin lesions tend to be darkly pigmented or of the body can be seen in isolation. It is well acknowledged
even black, whereas for those treating mainly skin of color that there are not only psychocutaneous but also cutaneo-
everything appears to be strangely red. In addition, in Asia psychological disorders. Virtually nobody with an embar-
dark-complexioned people tend to live in a tropical climate rassing or severe skin disease will not be psychologically
and are more exposed to infections and infestations but stressed; and vice versa, psychological distress worsens or
more protected against the harmful, particularly carcino- even precipitates a variety of skin diseases.
genic action of the sun, of which they have more than plenty. Finally, the miscellaneous chapters cover what is not yet
This atlas is comprised of five sections, with groups of discussed – age- and pregnancy-related dermatoses, drug
skin signs and diseases. In fact, after a brief introduction to reactions, physically induced skin lesions, and pediatric
look at the skin to make a diagnosis, as the title promises, rashes with fever.
the atlas starts with hypo- and hyperpigmentations, which The 85 chapters in five sections will leave no gap. The
are very stigmatizing in dark skin. Even in seemingly black illustrations will help to reach to the correct diagnosis, and
skin, hyperpigmentation is noted as profoundly embar- the first three sections will facilitate the use of the atlas as
rassing by the individual, and patchy hypopigmentation is they are related to signs easily recognized by any practi-
extremely visible and may be a serious psychosocial burden, tioner, thus allowing the practitioner to recognizing skin
whereas smooth pale skin is what most people strive for. lesions and evaluate their importance.
The text continues with the classic efflorescences such as I thank the editors and all authors for their tremendous
papules, nodules, plaques, tumors, blisters, pustules, and work in the field of dermatology in colored skin, which rep-
ulcers. Scaly and atrophic lesions, bleeding, erythema, tel- resents in fact the majority of the world population.
angiectasia, cysts, sinuses, and eschar complete this section.
Section 3 is devoted to regional dermatoses, ranging
from the scalp to the sole of the foot. All regions that have
so fundamentally different anatomical characteristics and
Eckart Haneke
diverse functions are covered so that each “regional special-
Freiburg, Germany and Bern, Switzerland
ist” will find their diagnoses well and critically discussed.

xiii
Preface

Why a new book? This was a question we too asked our- per the clinical classification referred above. This arrange-
selves before starting work upon this book. The answer ment is in sync with the tone of “differential diagnosis,”
lies in the experience of teaching and interaction with which is the purpose of this atlas. However, this would
many colleagues. Most of the textbooks and reference mean that the sequence of entities would bear relevance to
books follow an etiological approach (i.e., they describe morphology in priority rather than how common or rare
viral skin conditions together), and thus, readers learn they are. Obviously again, entities would have presence in
about varicella and common warts in the same chapter. more than one chapter, which is true to observe in this atlas.
This arrangement of entities does not help readers, espe- Each such repeated condition has been described in detail
cially the beginners, in developing their clinical acumen. in one chapter, whereas at other places, it bears a brief men-
Diagnostic skills depend on generating as well as exclud- tion only. In order to keep the atlas concise, very rare enti-
ing differentials for a given presentation based on mor- ties have been incorporated into tables and omitted from
phology (and other details), and thus it is understandable the discussion. Readers desirous of description of such enti-
that a book discussing dermatoses based on morphologi- ties would be advised to refer to comprehensive text books
cal similarities and differences will help readers improve of the field.
their clinical skills. Hence, we decided to work upon an The role of investigations and treatment protocols were
atlas of dermatology tailored to the interest and need of not within the scope of this book, so they have been delib-
young clinicians and trainees. erately omitted. Only the salient clinical points and impor-
The content of the book is divided into five sections. tant differentials have been incorporated in the abridged
The first section of the atlas focuses on the basic vocabu- description of disease entities.
lary required to understand the language of dermatology. A book covering these many aspects along with an
The different attributes of skin lesions have been elabo- enormous number of images would be a bulky textbook.
rated with emphasis on its morphology, distribution, pat- To retain its handy shape and size, the publishers and
tern, and arrangement. Section two is the backbone of editors converged upon a novel design of the book. The
the atlas as it incorporates the differential diagnosis of print and online versions of designated chapters in the
primary, secondary, and special skin lesions. As skin is same book have helped it achieve a suitable form and size.
not uniform all over and diseases tend to express differ- Of course, for convenience, the table of contents is com-
ently over the body parts, a need for a separate section mon for online as well as print portions. Editors antici-
(three) on regional dermatology was felt. The subse- pate that this form of book will offer an advantage to its
quent fourth section is to emphasize and cover the link users.
between skin manifestations and systemic (internal) dis- Dermatology being primarily a visual science, clinch-
eases. The remaining unclassified but important topics, ing diagnosis fairly depends upon the mental image
which could not be included in the preceding sections, that one forms out of innumerable visual impressions
have been incorporated in the concluding fifth section absorbed during clinical training, fortified by sound
of miscellaneous topics (neonatal dermatoses, geriatric theoretical learning and the knowledge of appropriate
dermatoses, pregnancy-related dermatoses, cutaneous investigative methods. Adequate memory image devel-
adverse drug reaction, and dermatoses with photosensi- opment demands repetitive exposure to clinical cases
tivity, etc.). and appreciation of minute variations in the presenta-
The introductory part of every chapter classifies various tion of lesions. Images in different forms such as print
entities according to their salient clinical features (number/ and digital supportive media further aid in acquiring
color/ distribution/ presence or absence of systemic features, visual skills. This atlas is enriched with numerous clini-
etc.). This clinical approach is followed by a brief description cal images, depicting the spectrum of morphological
of the entities. In the description part, the entities appear as changes in colored skin. We are sure the rich collection

xiv
 Preface xv

of images will appeal to every clinician dealing with der- Happy and fruitful learning!
matoses in colored skin.
This book is not intended to be an exhaustive text on Education is not the learning of facts but the training of
dermatology but to help clinicians hone clinical skills. We the mind to think. – Albert Einstein
hope that our book, titled Atlas of Clinical Dermatology in
the Skin of Color: A Morphological Approach, serves its pur- PC Das
pose and achieves popular acceptance amongst the trainees Piyush Kumar
and practitioners of dermatology for whom it is intended [email protected]
and dedicated to.
Acknowledgements

●● All the patients who put trust in us and allowed us to ●● Ms. Shivangi, Ms. Himani, and others from CRC team
use images for teaching purpose for timely guidance, motivation, and cooperation
●● Past and present residents of Department of throughout the project
Dermatology, Katihar Medical College and Hospital, ●● Many persons whose cooperation have been felt, but
Katihar especially Dr. Sushil S Savant, Dr. Rizwana whom we have missed mentioning
Barkat, Dr. Ghuncha Alam, Dr. Danish Akhtar, Dr.
Talat Fatima, Dr. Priya Rajbansh, Dr. Mamta Yadav, Dr. Editors
Rashid Shahid, and Dr. Rashmi Roy
●● Dr. Hiral Shah, Baroda Medical College, Vadodara for ●● All the teachers and colleagues from the Department of
generously allowing us to borrow clinical images from Dermatology, Medical College and Hospital, Kolkata – my
her collection and for valuable inputs on various aspects alma mater where I learned dermatology and where many
of the book clinical photographs used in this book were collected
●● Dr. Devrashetti Srinivas, Nizamabad, India for ●● All the teachers and colleagues from the Department
generously allowing us to borrow clinical images from of Dermatology, Katihar Medical College and Hospital,
his collection Katihar where I learned and thought dermatology and
●● Colleagues from “Urgent” group, where we discussed where many clinical photographs used in this book were
different topics for a better understanding collected
●● Ms. Snehlata (Jha) and Mr. Harsh Narayan Jha for their
invaluable help in arranging files for submission Piyush Kumar

xvi
Editors

Dr. PC Das is a senior practicing clinical dermatologist at Dr. Piyush Kumar finished his post-graduate work in der-
Purnea and Katihar districts of Bihar, India. He has gradu- matology from Medical College and Hospital, Kolkata in
ated and post-graduated from Patna Medical College, Patna 2011 and was judged the best outgoing university medical
University, India, in 2000. He has been practicing clini- graduate in dermatology. He received many scholarships
cal dermatology, lasers, and dermatosurgery for over two during and after his post-graduate work to attend and pres-
decades. He has participated and presented papers in inter- ent papers at national and international conferences. He
national, national, and regional dermatology conferences has been teaching Dermatology residents for more than a
and organized some of them. His fields of special interest decade and is currently working as Professor, Dermatology
are newer drugs in leprosy and clinical dermatology. at Madhubani Medical College, Bihar, India. He has pub-
lished more than 150 papers in indexed national and
international journals and works as a reviewer for various
national and international specialty journals. He has con-
tributed many chapters in various books and has co-edited
Nail Disorders: A Comprehensive Approach, Clinical Cases
in Disorders of Melanocytes, and Clinical Cases in Leprosy.
His areas of interest are clinical dermatology, dermatopa-
thology, nail disorders, and genital dermatoses.

xvii
Assistant editors

Dr. Santoshdev Rathod, MD, DNB Dr. Indrashis Podder, MD, DNB
Professor & Head of Unit, Dermatology Assistant Professor
SCL General Hospital, Smt. NHL Municipality Medical Department of Dermatology
College College of Medicine and Sagore Dutta Hospital
Ahmedabad, India Kolkata, India

Dr. Bhushan Madke, MD Dr. Dipali Rathod, DNB, DDVL, FIADVL

Professor & Head (Dermatopathology)
Department of Dermatology, Venereology & Leprosy, Assistant Professor, Dermatology
Jawaharlal Nehru Medical College, Datta Meghe Institute Seth GS Medical College and KEM Hospital
Higher Education and Research Mumbai, India
Wardha, India
Dr. Swetalina Pradhan, MD
Dr. Shekhar Neema, MD, FEBDV Associate Professor, Dermatology
Associate Professor All India Institute of Medical Sciences
Department of Dermatology Patna, India
Armed Forces Medical College
Pune, India Dr. Somodyuti Chandra, MBBS (Hons), MD (Gold
Medalist), DNB, MRCP-SCE (UK), FRGUHS
Dr. Niharika Ranjan Lal, MD Consultant Dermatologist
Assistant Professor Kolkata, India
Dermatology ESIPGIMSR and ESIC Medical College
Joka, Kolkata, India Dr. Komal Agarwal, MBBS (Hons.) (Gold Medalist), MD
(DVL), MRCP- SCE (UK)
Dr. Anup Kumar Tiwary, MD Senior Resident
Consultant Dermatologist Department of Dermatology
Yashoda Hospital and Research Center Calcutta National Medical College & Hospital
Ghaziabad, India Kolkata, India

xviii
Contributors

Komal Agarwal Kalgi Baxi

Senior Resident, Dermatology Assistant Professor, Dermatology
Calcutta National Medical College & Hospital Smt NHL Municipal Medical College
Kolkata, West Bengal, India Ahmedabad, Gujrat, India

Trupti Agarwal Riti Bhatia

Consultant Dermatologist Assistant Professor
Cloudnine Hospital Department of Dermatology and Venereology
Mumbai, Maharashtra, India All India Institute of Medical Sciences
Rishikesh, Uttarakhand, India
Danish Akhtar
Senior Resident, Dermatology Anirban Das
Katihar Medical College and Hospital RMO-cum-Clinical Tutor
Katihar, Bihar, India Murshidabad Medical College & Hospital
Berhampore, West Bengal, India
Amarkant Jha Amar
Former Head of Unit, Dermatology
Anupam Das
Patna Medical College and Hospital
Assistant Professor, Dermatology
Patna, Bihar, India
KPC Medical College and Hospital
Kolkata, West Bengal, India
Balachandar S Ankad
Professor and Head
Nilay Kanti Das
Department of Dermatology
Professor and Head
S Nijalingappa Medical College
Department of Dermatology
Bagalkot, Karnataka, India
College of Medicine and Sagore Dutta Hospital
Kamarhati, Kolkata, India
Amina Asfiya M I
Senior Resident
Gaurav Dash
Department of Dermatology
Assistant Professor
Yenepoya Medical College
Department of Dermatology
Deralakatte, Mangalore, Karnataka, India
SCB Medical College and Hospital
Cuttack, Odissa, India
Sasi Attili
Consultant Dermatologist Chirag Desai
Vizag, Andhra Pradesh, India Consultant Dermatologist
Mumbai, Maharashtra, India
Rizwana Barkat
Senior Resident Talat Fatima
Department of Dermatology, Venereology and Leprosy Senior Resident, Dermatology
Anugrah Narayan Magadh Medical College Nalanda Medical College and Hospital
Gaya, Bihar, India Patna, Bihar, India

xix
xx Contributors

Satyaki Ganguly Swetha Jain

Associate Professor, Dermatology Consultant Dermatologist
All India Institute of Medical Sciences Mumbai, Maharashtra, India
Raipur, Chhatisgarh, India
Abhishek Jha
Ramesh C Gharami Associate Professor, Dermatology
Professor Patna Medical College and Hospital
Department of Dermatology Patna, Bihar, India
Medical College and Hospital
Kolkata, West Bengal, India Prerna
Aparajita Ghosh Senior Resident
Associate Professor, Dermatology Dermatology Government Medical College and Hospital
KPC Medical College and Hospital Purnea, Bihar, India
Kolkata, West Bengal, India
Bhumesh Kumar Katakam
Vishal Golay Assistant Professor, Dermatology
Consultant Nephrologist Gandhi Medical College/Hospital
Remedy Clinic Hyderabad, Telangana, India
Siliguri, West Bengal, India
Manish Khandare
Ankan Gupta Head of Department, Dermatology
Associate Professor Indian Naval Hospital Ship (INHS) Sanjivani
Department of Dermatology Kochi, Kerala, India
Christian Medical College
Vellore, Tamilnadu, India Meenaz Khoja
Consultant Dermatologist
Sunil Kumar Gupta Pune, Maharastra, India
Dermatology
All India Institute of Medical Sciences Sri Harsha Kolla
Gorakhpur, Uttar Pradesh, India Assistant professor, Dermatology
Mamata Academy of Medical Sciences
Vishal Gupta Bachupally, Telangana, India
Assistant Professor
Department of Dermatology and Venereology Sunil Kothiwala
All India Institute of Medical Sciences Consultant Dermatologist
New Delhi, India Jaipur, Rajsthan, India

Navya Handa Ch Venkata Krishna

Consultant Dermatologist Consultant Dermatologist
New Delhi, India Kiranya Anjana Skin and Hair Clinic
Nalgonda, Telangana, India
Shahid Hassan
Professor, Dermatology Dhiraj Kumar
Madhubani Medical College and Hospital Consultant Dermatologist
Madhubani, Bihar, India Patna, Bihar, India

Ravi Hiremagalore Ghaliyah Aziz Kutty

Consultant Dermatologist Consultant Pediatrician
Bangalore, Karnataka, India Mathery Medical Mission Hospital
Alappuzha, Kerala, India
Sourabh Jain
Assistant Professor Niharika Ranjan Lal
Dermatology Assistant Professor, Dermatology
Atal Bihari Vajpayee Government Medical College ESIPGIMSR and ESIC Medical College
Vidisha, Madhya Pradesh, India Joka, Kolkata, West Bengal, India
 Contributors xxi

Rajesh Kumar Mandal Anuradha Priyadarshini

Associate Professor, Dermatology Assistant Professor
North Bengal Medical College and Hospital Department of Dermatology
Sushrutanagar, Siliguri, West Bengal, India Sri Ramachandra Medical College
Chennai, Tamilnadu, India
Madhulika Mhatre
Director, Skin Saga Centre for Dermatology Priya Rajbansh
Mumbai, India Senior Resident, Dermatology
Katihar Medical College and Hospital
Avijit Mondal Katihar, Bihar, India
Assistant Professor, Dermatology
College of Medicine and JNM Hospital Dipali Rathod
Kalyani, West Bengal, India Assistant Professor, Dermatology
Seth GS Medical College and KEM Hospital
Mumbai, India
Sabha Mushtaq
Lecturer
Santoshdev P Rathod
Department of Dermatology, Venereology and Leprology
Professor & Head of Unit, Dermatology
Government Medical College, Jammu
SCL General Hospital, Smt. NHL Municipality Medical
Jammu and Kashmir, India
College
Ahmedabad, Gujrat, India
Shekhar Neema
Associate Professor Arpita Nibedita Rout
Department of Dermatology Assistant Professor
Armed Forces Medical College Department of Dermatology and Venereology
Pune, Maharashtra, India All India Institute of Medical Sciences
Bhubaneswar, Odissa, India
Balakrishna Nikam
Assistant Professor, Dermatology Rashmi Roy
Krishna Institute of Medical Sciences Senior Resident, Dermatology
Karad, Maharashtra, India Lord Buddha Koshi Medical College & Hospital
Saharsa, Bihar, India
Pooja Nupur
Assistant Professor, Dermatology Dilip Kumar SA
Nalanda Medical College and Hospital Professor
Patna, Bihar, India Department of Dermatology
LSLAM Government Medical College
Saumya Panda Raigarh, Chhattisgarh, India
Consultant Dermatologist
Divya Sachdev
Belle Vue Clinic
Consultant Dermatologist
Kolkata, West Bengal, India
Raipur, Chhatisgarh, India

Col. Vikas Pathania Abanti Saha

Commanding Officer Associate Professor, Dermatology
180 Military Hospital Medical College and Hospital
Missamari, Assam, India Kolkata, West Bengal, India

Swetalina Pradhan Kananbala Sahu

Associate Professor, Dermatology Assistant professor, Dermatology
All India Institute of Medical Sciences Sri Jagannath Medical College and Hospital
Patna, Bihar, India Puri, Odissa, India

Sai Prasanth Neti (Sqn Ldr) Sunmeet Sandhu

Consultant Dermatologist Assistant Professor, Dermatology
G. K. Polyclinic 7 Air Force Hospital
Hyderabad, Telangana, India Kanpur, Uttar Pradesh, India
xxii Contributors

Satish S Savant Sudhir Singh

Director, The Humanitarian Clinic: Skin, Hair and Laser Centre Associate Professor
Dr. Savant’s Institute of Dermatosurgery, Cosmetology, Department of Dermatology
Trichology and Laser Medicine Datta Meghe Medical College
Mumbai, Maharashtra, India Nagpur, Maharashtra, India

Sushil S. Savant Anwita Sinha

Assistant Director, The Humanitarian Clinic: Skin, Hair and Assistant Professor, Dermatology
Laser Centre Base Hospital
Dr. Savant’s Institute of Dermatosurgery, Cosmetology, Barrackpore, India
Trichology and Laser Medicine
Mumbai, Maharashtra, India
Preema Sinha
Rashid Shahid Professor & Head of Unit
Senior Resident, Dermatology Department of Dermatology
All India Institute of Medical Sciences Base Hospital
Patna, Bihar, India Lucknow, Uttar Pradesh, India

Aseem Sharma Chandra Sekhar Sirka

Chief Dermatologist Additional Professor
Skin Saga Centre for Dermatology Department of Dermatology and Venereology
Mumbai, India All India Institute of Medical Sciences
Bhubaneswar, Odissa, India
Preeti Sharma
Consultant Dermatologist Dependra Kumar Timshina
Chandigarh, Punjab, India Consultant Dermatologist
Remedy Clinic
Rampal Sharma
Siliguri, West Bengal, India
Former Head of Unit, Dermatology
LRM Government Medical College
Meerut, Uttar Pradesh, India Anup Kumar Tiwary
Consultant Dermatologist
Shivani Sharma Yashoda Hospital and Research Centre
Specialist Medical Officer, Dermatology District Hospital Ghaziabad, Uttar Pradesh, India
Mathura, Uttar Pradesh, India
Kanya Rani Vashisht
Manjunath M. Shenoy Consultant Dermatologist
Professor & Head of Unit Panchkula, Haryana, India
Department of Dermatology
Yenepoya Medical College Resham Vasani
Deralakatte, Mangalore, Karnataka, India Consultant Dermatologist
Bhojani Clinic
B Shiny Shulamite
Matunga, Mumbai, Maharashtra, India
Consultant Dermatologist
Research Associate
Capstone Clinic
B J Wadia Hospital for Children
Hyderabad, Telangana, India
Parel, Mumbai, Maharashtra, India
Amrita Sil
Associate Professor Vignasree A
Department of Pharmacology Consultant Dermatologist
Rampurhat Government Medical College Hyderabad, Telangana, India
Rampurhat, West Bengal, India
Mamta Yadav
Gautam Kumar Singh Senior Resident, Dermatology
Professor & Classified Specialist, Dermatology Maharshi Devrahawa Baba Autonomous State (MDBAS)
Base Hospital Medical College
Delhi, India Deoria, Uttar Pradesh, India
 1
Section    

Introduction to clinical dermatology

DOI: 10.1201/9781351054225-1
 E1
Overview of skin lesions

CHANDRA SEKHAR SIRKA, SWETALINA PRADHAN, KANANBALA SAHU,

ARPITA NIBEDITA ROUT

ABSTRACT
Description of the skin lesions is necessary to reach a clinical diagnosis. A good description of primary, secondary and foot
print of the lesions can help in diagnosis. This chapter discusses various skin lesions, arrangements and distributions necessary
for a good dermatological description of lesions.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

2 DOI: 10.1201/9781351054225-2
 Section     2
Approach to clinical diagnosis

PIYUSH KUMAR, PC DAS

Dermatology is a visual speciality, and diagnosing a der- In our opinion, clinical diagnostic skills in dermatol-
matological disease is different from making a clinical ogy rest on generating a list of differential diagnosis and
diagnosis in other specialities. While some skin diseases narrowing the list clinically as far as possible. Though
can be diagnosed by a mere glance (such as acne vulgaris, many dermatologists prefer to take history before clini-
fixed drug eruption, herpes zoster, etc.), the diagnosis of cal examination of lesions, we prefer to do a quick clini-
most dermatoses requires careful consideration of clues cal examination first to get a list of differential diagnosis
gathered from history and clinical examination. In der- as it helps us in getting a meaningful and clinically use-
matology, the diagnostic process usually involves these ful history. This quick mucocutaneous examination
five steps: rests on documentation and analysis of the following
(acronym-MADE):
1. Brief history – Ascertain onset, duration, symp-
toms, etc. Determine whether a condition is con- ●● Morphology – Identify primary and special lesions,
genital or acquired, acute or chronic, symptomatic secondary changes (if any), and the number, color, and
or asymptomatic, etc. shape of lesions.
2. Quick mucocutaneous examination – The pur- ●● Arrangement (configuration) – The lesions in
pose is to document the morphology (primary, many dermatoses assume a characteristic
secondary, special), distribution, and arrange- distribution, and sometimes configuration alone
ment of the lesions. It helps us to arrive at a is diagnostic. For example, grouped vesicles on
provisional diagnosis or to generate a list of dif- an erythematous base, arranged in a dermatomal
ferential diagnosis. pattern, are diagnostic of herpes zoster and
3. Detailed history – A good history in dermatology zosteriform herpes simplex.
aims at collecting positive and negative pointers ●● Distribution – Skin and mucosa have limited
for the conditions included in the list of differential ways of expressing themselves (as primary and
diagnosis generated in the second step. secondary lesions), and a variety of etiologically
4. Thorough examination – This involves derma- different conditions may present in a similar
tological and systemic examination to document manner. The differential distribution of these
findings, to elicit supportive clinical signs, if any, etiologically different conditions allows clinical
and to assess the extent and severity of involve- diagnosis of many conditions. For example,
ment of skin and other systems. eczematous changes over convex parts of face and
5. Investigation and diagnosis – A confirma- other photo-exposed areas suggest the diagnosis
tory test is done to establish the diagnosis and of photoallergic contact dermatitis, while similar
exclude differentials that could not be excluded changes localized to lower legs may be seen in
clinically. stasis dermatitis.

DOI: 10.1201/9781351054225-3
4 Approach to clinical diagnosis

●● Evolution – Evolution refers to changes in the lesions important to note evolution of lesions to arrive at a
over time. For example, the initial papule of lupus diagnosis.
vulgaris expands over time to form a plaque and then
further extends by central scarring. Among blistering The main aim of this book is to help readers generate
disorders, bulla in subepidermal blistering disorders clinical differentials. The subsequent chapters will discuss
may heal with scarring and milia formation, while some common and uncommon differential diagnosis for a
intraepidermal bullae heal with pigmentary given presentation.
changes only. Hence, it is evident that it is very
 1
Hypopigmented and depigmented macules
and patches: Localized

PC DAS, PRIYA RAJBANSH

INTRODUCTION and their distribution, followed by all possible and needful

investigations are always required to avoid misdiagnosis.
Pigmentary changes constitute a major proportion of the Some clinical clues to the diagnosis of localized hypopig-
patients consulting a dermatologist or general physician. In mented macules and patches have been summarized in
skin of color, hypo/depigmented macules and patches are Tables 1.1 and 1.2. Further, common dermatologic condi-
more evident. Etiologically, pigmentary loss can be due to tions have been discussed individually. 1-12
apoptosis of melanoblasts during differentiation (piebald-
ism), reduced melanogenesis (seen in pityriasis versicolor and Pityriasis alba
steroid-induced hypopigmentation), autoimmune destruc- ●● This is a common self-limiting, idiopathic condition

tion (vitiligo), impaired dendritic transfer of melanosomes to affecting children and adolescents. It presents as
keratinocytes (pityriasis alba), post-inflammatory (as seen in ill-defined, faintly erythematous, or hypopigmented
incontinentia pigmenti, lichen striatus, or annular lichenoi- macules and patches of 1–4 cm in size with fine scaling.
des dermatitis of youth), or due to extreme local cutaneous The number usually ranges from 4 or 5 to 20 or more.1
vasoconstriction (nevus anemicus and Bier spots). ●● The lesions are asymptomatic or mildly itchy.
While approaching a patient presenting with localized ●● The face (cheeks in most cases) is the most commonly
hypo/depigmented disorders, one should first observe if the affected site (Figure 1.1). Upper arms, neck, or
lesions are hypopigmented (reduced pigmentation) or depig- shoulders (and rarely trunk or lower extremities) may
mented (complete loss of pigmentation). There are only a also be affected in generalized disease.
few clinical differentials for localized depigmented patches, ●● History of atopy may be present.
and hence this observation can be very useful in narrowing ●● Uncommon variants are the pigmenting type (central

the list of differentials. Other than that, size, shape, and dis- hyperpigmented zone with hypopigmented, slightly
tribution of lesions are important clinical clues. There can scaly halo) and generalized type.
be various morphologies of hypo/depigmented macules, ●● Differential diagnosis (DD): Post inflammatory

such as guttate in idiopathic guttate hypomelanosis; con- hypopigmentation (history of prior dermatoses),
fetti-like and petaloid in pityriasis versicolor; leaf-shaped seborrheic dermatitis (affects seborrheic area), and early
in phylloid hypomelanosis; and ash-leaf macules of tuber- vitiligo (no scales).
ous sclerosis – linear, large, and irregular. Additionally, a
lesion can be scaly, surrounded by erythematous or hyper-
pigmented border, or associated with skin sclerosis or atro- Nevus depigmentosus (nevus achromicus)
phy as seen in morphea. Decreased or loss of sensation in ●● It is a congenital pigmentary mosaic condition due to

the lesion(s) favors Hansen’s disease, warranting further altered clones of melanocytes that have a decreased
investigation. Distribution of such hypopigmented macules ability to synthesize melanin and transport it to
is another important clue to arrive at a diagnosis or rule out keratinocytes.
differentials. Some diseases may present with only localized ●● It usually presents at birth or in early childhood.2

hypopigmentation, such as hypomelanosis of Ito, nevus ●● It presents as a solitary, asymptomatic, hypopigmented

depigmentosus, or piebaldism. macule/patch with a well-defined serrated margin

Therefore, detailed history regarding evolution of the that does not cross the midline. Very often smaller
lesions, careful examination of hypopigmented macules macules appear around the lesion, giving it a “splashed

DOI: 10.1201/9781351054225-4 5
6  Hypopigmented and depigmented macules and patches: Localized

Table 1.1  Clinical clues to localized hypo/depigmented macules

Variable sized Linear/segmental

Solitary/Few macules Guttate macules macules hypopigmentation
Early childhood • Nevus depigmentosus • Confetti-like macules D • Ash-leaf macule in • Hypomelanosis of
• Nevus anemicus in tuberous sclerosis tuberous sclerosis Ito
• Piebaldism D (and and multiple endocrine • Phylloid • Incontinentia
Waardenburg syndrome) neoplasia type 1 hypomelanosis pigmenti ***
Late childhood • Pityriasis alba • Eruptive • Vitiligo vulgaris D • Lichen striatus †
• Focal vitiligo D hypomelanosis • Segmental vitiligo D
• Pityriasis lichenoides
chronica
• Vitiligo punctata D
Adults • Focal vitiligo D • Vitiligo punctata D • Vitiligo vulgaris D • Corticosteroid-
• Chemical leukoderma D • Post-inflammatory • Post-inflammatory induced
• Melanoma associated hypopigmentation hypopigmentation hypopigmentation
leukoderma • Pityriasis versicolor • Progressive
• Indeterminate leprosy • Idiopathic guttate macular
• Tuberculoid leprosy hypomelanosis D hypomelanosis
• Borderline tuberculoid • Leukoderma punctata D • Lepromatous
leprosy • Physiologic anemic leprosy
• Morphea macules (Bier spots) • Macular post
• Annular lichenoides • Lichen sclerosus et kala-azar dermal
dermatitis of youth (ALDY) atrophicus * leishmaniasis
• Post-inflammatory • Tumor of follicular
hypopigmentation infundibulum **
• Halo nevus D and
phenomenon
• Woronoff’s ring
Notes:
*It is actually an indurated plaque. It has been included here in the table for clinical similarity of vitiligo.
**The lesions in tumors of follicular infundibulum are actually tiny papules that may be misinterpreted as macules if the observer is not careful
***Atrophy is associated; hence, lesion is not macule/patch.
†The primary lesions are tiny papules. The lesions heal with post-inflammatory hypopigmentation, which may be only clinical findings
observed in cases presenting late.
D Depigmented patches

paint” appearance (Figure 1.2a,b). In contrast to nevus

anemicus, the lesional skin shows erythema on rubbing
(Figure 1.2c).
●● The common sites involved are trunk and proximal
extremities.
●● The lesion grows in size with the growth of the body and
becomes stable thereafter.

Table 1.2  Approach to depigmented macules and patches

Onset Features Diseases

Congenital/ Non-progressive • Associated with
childhood (shows proportionate hyperpigmented
onset growth as child macules
grows) – piebaldism
• Segmental vitiligo
Acquired Randomly distributed, Vitiligo and its clinical
progressive variants
History of injury Leukoderma
Older population, Idiopathic guttate Figure 1.1  Pityriasis alba presenting as Ill-defined
lesion does not grow hypomelanosis hypopigmented mildly scaly macules on cheeks in a
much in size child. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Hypopigmented and depigmented macules and patches: Localized  7

Figure 1.2  (a) Nevus depigmentosus with typical splash paint border on the posterior aspect of leg. (b) Nevus depigmento-
sus on the buttock. (c) The lesional skin (circled) as well as surrounding skin shows erythema on rubbing. (a–c – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

●● Three clinical patterns have been observed – localized, Nevus anemicus

segmental, and linear or whorled. ●● It is a congenital anomaly characterized by localized
●● The linear, or whorled, type can be extensive in hypersensitivity of blood vessels to catecholamines,
distribution and associated with extra-cutaneous resulting in vasoconstriction. It may present at birth
findings like seizures, mental retardation, or become noticeable in early childhood and persists
hemihypertrophy, and yellow hair. It has considerable unchanged throughout life.
overlap with hypomelanosis of Ito, and hence its ●● It presents as single or a few asymptomatic,
existence as a separate entity is debatable. circumscribed, rounded, oval, or linear pale macules or
●● Wood’s lamp examination demonstrates an off-white patches with irregular margins that may be surrounded
color. by satellite macules.3
●● DD: Nevus anemicus (the margin disappears on ●● The lesion does not show erythema on rubbing, while
diascopy), vitiligo (chalky-white color on Wood’s lamp). the surrounding skin does (Figure 1.3).
8  Hypopigmented and depigmented macules and patches: Localized

Piebaldism
●● Piebaldism is an autosomal dominant disorder

characterized by localized absence of melanocytes due

to mutations in the c-kit proto-oncogene. The skin and
hair changes are present from birth and do not progress.
The characteristic feature is a midline triangular or
diamond-shaped, depigmented patch with poliosis.4
●● Hyperpigmented macules or islands of pigmentation

within the patch and at its border provide an important

clinical clue for diagnosis.
●● The frontal area is the most commonly affected site

(white forelock). The face (particularly the chin), trunk,

and mid-extremities (sparing hands and feet) are other
Figure 1.3  Nevus anemicus with hypopigmented patch commonly affected areas (Figure 1.4a–c).
with irregular, ill-defined margin. (Courtesy: Dr. Piyush ●● It could present as an isolated anomaly or as a part of

Kumar, Katihar, India.) Waardenburg syndrome, along with heterochromia

of the irides, lateral displacement of inner canthi, and
●● It is most commonly noted on the chest, although it can deafness (Figure 1.4d).
be found anywhere on the body. ●● DD: Vitiligo.
●● The margin becomes less prominent on diascopy.
●● It is rarely associated with neurofibromatosis, tuberous Halo nevus
sclerosis, or phakomatosis pigmentovascularis. ●● Synonyms are Sutton’s nevus and leukoderma

●● DD: Nevus depigmentosus (which can be differentiated acquisitum centrifugum.

from nevus anemicus by appearance of erythema on ●● It refers to melanocytic nevus with a zone of

rubbing and no change of the margin of the macule on depigmentation around the melanocytic nevus due to
diascopy). immune-inflammatory response against melanocytes.5

Figure 1.4  (a) Piebaldism affecting the forehead. Note poliosis. (b) Piebaldism affecting the abdomen. Note hyperpig-
mented macules within the depigmented patches. (c) Piebaldism affecting the knee. Note pigmented macules within the
area of depigmentation. (d) Two sisters with Waardenburg syndrome. (a,c – Courtesy: Dr. Anil Patki, Pune, India;
b – Courtesy: Dr. Piyush Kumar, Katihar, India; d – Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India. )
 Hypopigmented and depigmented macules and patches: Localized  9

Figure 1.5  (a) A classical halo nevus on the hip. (b) Halo nevus on the shoulder region. (a,b – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

●● Teenagers are commonly affected, and the trunk is the

most common site of affliction.
●● The patient develops one or multiple circular,
depigmented macule(s) with a central tan or brown
nevus (Figure 1.5a,b). Both the central nevus and zone
of depigmentation may or may not involute with time.
●● At times it may be associated with vitiligo.
●● Over time, many other non-melanocytic and non-neoplastic
conditions have been known to develop halo phenomenon.
●● Conditions in which the Halo phenomenon can be seen
are summarized in Table 1.3.
●● DD: Melanoma.
Woronoff’s ring Figure 1.6  Hypopigmented Woronoff ring around the pso-
●● It is a hypopigmented halo of uniform width around a riasis lesion. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
psoriatic plaque.
●● This develops after phototherapy or topical treatment of ●● DD: Halo nevus, post inflammatory hypomelanosis,
psoriatic lesions (Figure 1.6). pityriasis rosea.
●● A prostaglandin E2 injection given 1cm away from
Vitiligo
the Woronoff’s ring causes the hypopigmented area to
●● Vitiligo results from autoimmune destruction
develop erythema, which suggests vasoconstriction as a
possible etiology. of melanocytes and may present as localized or
generalized disease.
●● Early focal vitiligo often presents with hypopigmented
Table 1.3  Dermatoses with halo phenomenon macules or patches with ill-defined margin with no
Non-melanocytic scales.6 With time, it becomes sharply defined and
Melanocytic conditions conditions depigmented. Sometimes there may be a zone of
hypopigmented skin between depigmented and normal-
• Blue nevus • Basal cell epithelioma appearing skin (trichrome vitiligo) (Figure 1.7a–d).
• Congenital Mongolian • Lichen planus ●● With treatment or spontaneously, lesions of vitiligo
spots • Molluscum get repigmented frequently, and this repigmentation is
• Congenital contagiosum either perifollicular or from the margin (Figure 1.7e).
nevomelanocytic lesion • Angioma ●● Koebnerization may be seen.
• Dysplastic melanocytic • Psoriasis ●● Segmental forms of vitiligo are frequently seen and are
nevi • Seborrheic keratosis common in children (Figure 1.7f,g).
• Primary cutaneous • Neurofibroma ●● DD: Pityriasis alba (scales), lichen sclerosus (atrophy),
malignant melanoma or • Sarcoidosis Leprosy (sensory diminution and/or dry, anhidrotic
metastasis • Warts skin), Vogt-Koyanagi-Harada syndrome, chemical
• Spitz nevus leukoderma, Melanoma-associated leukoderma.
10  Hypopigmented and depigmented macules and patches: Localized

Figure 1.7  (a) Localized depigmented patches of vitiligo on

both eyelids. (b) Localized vitiligo affecting areola and nip-
ple. (c) Bilateral vitiligo on dorsum of hands. (d) Trichrome
vitiligo – there is a zone of hypopigmented skin between
depigmented and normal-appearing skin. (e) During repig-
mentation, perifollicular pigmented macules appear within
the vitiligo lesion. (f) Segmental vitiligo affecting left mid-
face. (g) Segmental vitiligo in the left periorbital region.
(a,d,e – Courtesy: Dr. Piyush Kumar, Katihar, India.)
  
 Hypopigmented and depigmented macules and patches: Localized  11

Leprosy ●● TT Leprosy – Patients present with one to three

●● It is a chronic infectious disease caused by M. leprae, with hypopigmented patches with well-defined margins
prominent involvement of skin and peripheral nerves. all around, complete or near complete sensory
●● It affects all races and age groups as well as both men loss, loss of hairs, and anhidrosis.6 Other common
and women equally. presentations include erythematous plaque and
●● It has a wide spectrum of clinical findings. Leprosy annular plaque.
is classified based on cell-mediated immunity (CMI) ●● BT leprosy – Compared to TT leprosy, the lesions
into polar forms, with lepromatous leprosy (LL) at one in BT are more in number and larger in size, and
end and tuberculoid leprosy (TT) at the other end and they demonstrate less severe features of nerve
three types of borderline leprosy in between: borderline damage – sensory loss, loss of hairs, and anhidrosis.
lepromatous (BL), borderline borderline (BB) and The largest diameter of BT lesions may be several
borderline tuberculoid (BT). The term indeterminate centimeters, sometimes involving an extremity
leprosy (IL) is used to describe patients with early almost completely, and may assume annular
leprosy lesions that cannot be categorized into any pole. configuration. The margin of the lesion is well
●● Localied hypopigmented lesions may be seen in defined at most places but is characteristically ill
indeterminate, tuberculoid (TT) or borderline defined at others, merging with normal skin. A
tuberculoid (BT) leprosy (Figure 1.8a,b). satellite lesion is often present and is a valuable
●● Indeterminate leprosy – It is characterized by one or a clinical clue for the diagnosis of BT leprosy.
few hypopigmented patches with ill-defined margins ●● DD: Early vitiligo (sensation intact, accentuation
(Figure 1.8c). The features of nerve damage – sensory of lesions on Wood’s lamp examination), morphea
loss or loss of sweating – are not appreciable. The (indurated), lichen sclerosus (atrophy present),
lesions may persist for a couple of years and then may pityriasis alba (sensation intact, scaling), post-
resolve spontaneously or may develop into one of the inflammatory hypopigmentation, hypopigmented
determinate forms depending on immunity. mycosis fungoides.

Figure 1.8  (a) Well defined dry, hypopigmented macule on leg in BT Hansen patient. Note epidermal atrophy
appreciable as crinkles. (b) Hypopigmented macule on lower back along with stria distensae in BT leprosy patient.
(c) Hypopigmented patch of indeterminate leprosy on the forearm. Note ill-defined margins. (a–c – Courtesy:
Dr. Piyush Kumar, Katihar, India.)
12  Hypopigmented and depigmented macules and patches: Localized

Figure 1.9  (a) Post kala-azar dermal leishmaniasis seen as multiple hypopigmented macules and patches predominantly
seen over the muzzle area of the face. In addition, there are some erythematous juicy papules. (b) Hypopigmented
patches in post kala-azar dermal leishmaniasis. Lesions are most prominent in perioral area. (b – Courtesy: Dr. Piyush
Kumar, Katihar, India.)

Post kala-azar dermal leishmaniasis Ash-leaf macules

●● It develops after partially treated or untreated visceral ●● Ash-leaf macules (ALMs) are classically seen in

leishmaniasis (Kala azar). However, in some cases there tuberous sclerosis (TS) – an autosomal dominant
is no history of prior kala azar. disorder characterized by seizures, mental retardation,
●● Although in advanced cases hypopigmented macules and skin findings.7
and patches are widespread, in early stages, patients ●● The cutaneous findings in TS include hypomelanotic

present with hypopigmented macules and patches macules, facial angiofibromas, fibrous plaques of
localized over the face. forehead, periungual and gingival fibromas, Shagreen
●● The typically affected area of the face is “muzzle area”– patches, and café’-au-lait macules.
around the nose and mouth (Figure 1.9a,b). ●● The hypomelanotic macules in TS are usually present at

●● It characteristically presents as asymptomatic birth or appear within the first few months of life and
hypopigmented non scaly macules, with a tendency are the most common cutaneous findings.7
to coalesce to form larger patches. If left untreated, ●● They adopt various configurations – polygonal

patients develop juicy erythematous papules and hypopigmented macules, ash-leaf macules, and confetti
nodules over it.6 macules.
●● DD: See Table 1.4. ●● Polygonal hypopigmented macules are the most

common and can appear anywhere over the body.

●● ALMs are characteristic of TS and appear as a few

Table 1.4  Differential diagnosis of PKDL mm to 5-cm hypopigmented patch that is ash-leaf
shaped, oval at one end and pointed at the opposite
Differential diagnosis
end. It is most often found on the trunk and buttocks
Localized disease • BT leprosy (sensory
(Figure 1.10a,b).
loss) ●● Confetti macules are most specific and mostly seen on
• Early vitiligo
extremities.
Widespread disease • Lepromatous leprosy ●● Lesional leucotrichia and poliosis have also been
(h/o kala-azar) (earlobe infiltration) described.
• Guttate vitiligo ●● Subtle lesions are better visualized during Wood’s lamp
(accentuation on examination
Wood’s lamp) ●● Three or more ALMs are a feature of tuberous sclerosis.
• Pityriasis lichenoides ●● DD: Nevus depigmentosus (serrated margin),
chronica nevus anemicus (margin disappears on diascopy),
• Leucoderma piebaldism (depigmented rather than hypopigmented,
syphiliticum midline distribution), vitiligo (occasionally develops
 Hypopigmented and depigmented macules and patches: Localized  13

Figure 1.10  (a) Typical ash-leaf macule on lumbar area. (b) Ash-leaf macule on upper limb.

soon after birth, lesion is depigmented), multiple ●● It is commonly seen after puberty (when activity of
endocrine neoplasia type 1 (MEN 1) (multiple facial sebaceous glands is high) and in summer.
angiofibromas, collagenomas, gingival papules, ●● Common sites are the upper trunk and upper
confetti-like hypomelanotic macules and CALMs), extremities. Facial involvement is less common
post-inflammatory hypopigmentation (due to atopic or but may be more commonly seen in infants and
seborrheic dermatitis in infants). immunocompromised patients.8
●● It typically presents as asymptomatic or mildly
Phylloid hypomelanosis itchy perifollicular hypopigmented to
●● Phylloid hypomelanosis is a distinct pattern of hyperpigmented macules with fine non-adherent
hypomelanosis that occurs in patients with mosaic scales and mild erythema. The lesions have a
forms of trisomy 13. tendency to coalesce to form larger polycyclic
●● It is characterized by congenital hypochromic macules patches, but the periphery may still show
resembling a floral ornament with various elements such as perifollicular lesions (an important clinical clue)
round or oval patches, asymmetrical macules resembling (Figure 1.11a–d). The lesions become more
begonia leaves, and pear-shaped or oblong lesions. prominent when wet (during bathing or after
●● Additional cutaneous features include telangiectatic sweating) – another important clinical clue.
macules and hypertrichosis. ●● Inverse variant – Lesions predominantly affect the
●● Associated extra cutaneous anomalies include flexural regions, the face, or isolated areas of the
cerebral defects (especially absence of corpus extremities. It may be more commonly noted in
callosum), conductive hearing loss, choroidal and immunocompromised persons.
retinal colobomas, craniofacial anomalies, and digital ●● Pityrosporum folliculitis – Patients develop multiple,
malformations. asymptomatic, 2- to 3-mm, monomorphic, follicular,
●● DD: Post-inflammatory hypopigmentation, segmental erythematous papules and pustules on trunk.
vitiligo. ●● Atrophic variant – This rare form is clinically
characterized by atrophic, ivory-colored to
Pityriasis versicolor (Tinea versicolor) erythematous, oval to round lesions. The surface has a
●● It is a common, superficial cutaneous fungal infection wrinkled appearance, and the atrophy is limited to the
caused by the dimorphic, lipophilic fungus of the areas affected by tinea versicolor.
genus Malassezia (Malassezia furfur, M. globosa, ●● DD: Pityriasis alba, vitiligo (non-scaly, Wood’s lamp
M. sympodialis). accentuation), lepromatous leprosy (loss of sensation,
●● Risk factors are warm, humid environments; non-scaly, presence of earlobe infiltration, co-existent
oil application; topical or systemic steroid use; nodular lesions, peripheral nerve thickening),
immunosuppression; malnutrition; pregnancy; and seborrheic dermatitis (scalp and face involvement, more
Cushing disease. itching).
14  Hypopigmented and depigmented macules and patches: Localized

Figure 1.11  (a) Pityriasis versicolor presenting as scaly

coalescing hypopigmented macules on neck, chest, and
shoulder. Note perifollicular macules – an important clinical
clue. (b) Hyperpigmented lesions in pityriasis versicolor. (c)
Pityriasis versicolor affecting the abdomen. (d) Pityriasis
versicolor affecting central chest. (a–d – Courtesy: Dr. Piyush
Kumar, Katihar, India.)

Idiopathic guttate hypomelanosis (IGH) ●● IGH presents as randomly distributed, discrete, angular
●● It is an acquired leukoderma of unknown etiology. or circular, hypopigmented or depigmented macules of
●● It is most commonly seen in middle-aged to older usually 1–3 mm.6 However, lesions may be as large as 10
people and has no sex predilection. mm. The number of lesions increases gradually, but the size
●● Most commonly affected sites are shins and forearms. of individual lesions remains the same (Figure 1.12a,b).
But it may be seen anywhere on the body. ●● DD: Post inflammatory hypopigmentation, guttate
●● Lesions are generally asymptomatic but can be mildly vitiligo (Wood’s lamp accentuation), guttate lichen
pruritic. sclerosus (atrophic lesion), leukoderma punctuate.
 Hypopigmented and depigmented macules and patches: Localized  15

Figure 1.12  (a) Idiopathic guttate hypomelanosis as depigmented macule on the shin. (b) Idiopathic guttate hypomelano-
sis lesion on the chest.

Physiologic anemic macules (Bier spots) ●● DD: morphea (induration present), hypopigmented
●● Bier spots are pale macules seen on the extremities due mycosis fungoides.
to localized vasoconstriction.9
●● They are seen most often in young females between 20
Tumour of follicular infundibulum
and 40 years of age and have no racial preponderance.
●● It is a rare benign cutaneous adnexal neoplasm arising
However, most cases have been reported among the
Chinese population. from the infundibular part of hair follicle.
●● It is mostly seen in the older population, with a slight
●● The lesions become more prominent when the limbs

are placed in a dependent position for a long time and female preponderance.
●● It has two variants – solitary and multiple.
resolve when limbs are elevated.
●● The most common form is solitary, which presents as
●● They can sometimes be seen in association with

cryoglobulinemia and scleroderma renal crisis. asymptomatic papulonodular scaly lesion measuring
●● Bier spots with insomnia and tachycardia are called about 1–2 cm.
●● The eruptive or multiple form (infundibulomatosis)
“Marshall-White syndrome.”
●● DD: Punctate vitiligo, pityriasis versicolor, nevus presents with a sudden onset of multiple, bilaterally
anemicus. symmetric, hypopigmented macules and papules over
face, neck, and upper trunk. The surface may show
scaling.11
●● DD: Pityriasis versicolor, post kala-azar dermal
Annular lichenoides dermatitis of youth (ALDY) leishmaniasis.
●● ALDY is a recently described condition of unknown

etiology; most cases are from Mediterranean area. It has

been documented in other parts of the world – central Hypomelanosis of ITO (incontinentia pigmenti
Europe, Japan, and Korea. achromicans)
●● It is predominantly seen in adolescents and has a male ●● It is a condition of pigmentary mosaicism characterized

predilection. ALDY presents as asymptomatic, sharply by various patterns of unilateral or bilateral

demarcated annular erythematous macules and patches hypopigmentation following the lines of Blaschko.12
with central hypopigmentation. Lesions may or may The hypopigmentation is due to presence of a clone of
not show a bilateral and symmetrical distribution. The melanocytes with decreased ability to produce pigment.
central hypopigmentation may not be noticeable in ●● It usually manifests in the first year of life, with a female

some cases.10 predominance (F:M= 2.5:1).

●● Most commonly affected sites are flanks and groin. ●● Trunk and extremities are the most commonly affected

Other common sites include axilla and neck. sites.

16  Hypopigmented and depigmented macules and patches: Localized

   

Figure 1.13  (a) Multi-dermatomal hypopigmented bands of hypomelanosis of Ito. (b) Hypomelanosis of Ito affecting
the right shoulder region and right upper extremity. (c) Hypomelanosis of Ito in a young boy. (a,b – Courtesy: Dr. Piyush
Kumar, Katihar, India; c – Courtesy: Dr Hiral Shah, Baroda Medical College, Vadodara, India.)

●● It consists of asymptomatic, small hypopigmented Table 1.5  Neurological and musculoskeletal abnormalities
macules coalescing to form large linear and whorl- in hypomelanosis of Ito
like patches following the lines of Blaschko. It can be
unilateral or bilateral. The lesion covers more than one Musculoskeletal Neurological
dermatome (Figure 1.13a–c). abnormalities abnormalities
●● Neurological and musculoskeletal abnormalities • Cleft palate Mental retardation
may be associated and should be looked for • Hemihypertrophy Seizures
(Table 1.5). • Limb, hand, and/or Neural tumors
●● DD: Linear and whorled nevoid hypomelanosis. foot abnormalities
• Nail abnormalities
Corticosteroid induced hypopigmentation • Hypotonia
●● This occurs after topical or injectable corticosteroids • Teeth abnormalities
used for the treatment of certain dermatosis (e.g., lichen • Hair anomalies
planus, keloid) or orthopedic conditions such as tennis • Face and/or skull
elbow. anomalies
●● It usually develops after a few weeks of injection/

topical use and may resolve spontaneously in several ●● It may extend along the blood vessels and lymphatics,
months. resulting in hypopigmentation in linear and branch-like
●● Asymptomatic, non-scaly, hypopigmented macules and fashion, tailing venous, and lymphatic drainage.
patches with or without atrophy are present at the site of ●● DD: Linear vitiligo, post inflammatory
injection/application (Figure 1.14a,b). hypopigmentation.
 Hypopigmented and depigmented macules and patches: Localized  17

Table 1.6  Causes of post-inflammatory hypopigmentation

Inflammatory skin disease Infections

• Allergic contact dermatitis • Chickenpox
• Atopic dermatitis • Herpes zoster
• Discoid lupus erythematosus • Impetigo
• Insect bite reactions • Onchocerciasis
• Lichen planus • Pinta
• Lichen striatus • Pityriasis
• Pityriasis lichenoides chronica versicolor
• Psoriasis • Syphilis
• Sarcoidosis
• Scleroderma
• Stevens-Johnson syndrome
Procedure related Miscellaneous
• Chemical peels • Burns
• Cryotherapy
• Dermabrasion
• Laser

REFERENCES
1. Lee D, Kang JH, Kim SH, Seo JK, Sung HS, Hwang SW. A case of
extensive pityriasis alba. Ann Dermatol 2008;20(3):146–148.
2. Deb S, Sarkar R, Samanta AB. A brief review of nevus depigmento-
sus. Pigment Int 2014;1:56–58.
3. Sethi A, Kaur T, Puri K. Giant nevus anemicus: A rare case report.
Indian J Paediatr Dermatol 2013;14:39–40.
4. Nargis T, Pinto M, Shenoy MM. Piebaldism with complete poliosis:
A rare presentation. Indian J Paediatr Dermatol 2018;19:183–185.
5. Vasani R. Meyerson phenomenon. Indian J Paediatr Dermatol
2019;20:78–80.
6. Kumarasinghe P, Uprety S, Sarkar R. Hypopigmentary disorders in
Asian patients. Pigment Int 2017;4:13–20.
7. Cardis MA, De Klotz CMC. Cutaneous manifestations of tuber-
ous sclerosis complex and the paediatrician’s role. Arch Dis Child
2017;102:85863.
8. Ghosh SK, Dey SK, Saha I, Barbhuiya JN, Ghosh A, Roy AK.
Pityriasis versicolor: A clinicomycological and epidemio-
logical study from a tertiary care hospital. Indian J Dermatol
2008;53:182–185.
9. Mahajan VK, Khatri G, Singh R, Chauhan PS, Mehta KS. Bier spots:
An uncommon cause of mottled skin. Indian Dermatol Online J
2015;6(2):128–129.
10. Di Mercurio M, Gisondi P, Colato C, et al. Annular lichenoid derma-
Figure 1.14  (a) Steroid (used for lichen planus) induced titis of youth: Report of six new cases with review of the literature.
depigmentation on left ankle and lower leg. (b) Injectable Dermatology 2015;231:195–200.
steroid induced localized depigmented patch. (a – 11. Suchonwanit P, Ruangchainikom P, Apibal Y. Eruptive Tumors of
Courtesy: Dr. Piyush Kumar, Katihar, India.) the Follicular Infundibulum: An unexpected diagnosis of hypopig-
mented macules. Dermatol Ther 2015;5(3):207–211.
12. Chethan C J, Ashique K T, Sukumar D, Nanda Kishore B.
Hypomelanosis of ITO. Indian J Dermatol 2006;51:65–66.
Post-inflammatory hypopigmentation
●● This clinically presents as hypopigmented macules or

patches with or without erythema; lesions can be rough SUGGESTED READING

and scaly. 1. Hypopigmentation. Edited by Electra Nicolaidou, Clio Dessinioti,
●● History of dermatoses, infection, trauma, or burn is Andreas Katsambas. 1st edition. 2020. CRC Press.
suggestive. 2. The Pigmentary System: Physiology and Pathophysiology. Edited
●● Causes of post-inflammatory hypopigmentation are by James J. Nordlund Raymond E. Boissy Vincent J. Hearing
Richard A. King William S. Oetting Jean-Paul Ortonne. 2nd edition.
summarized in Table 1.6.
2006. Blackwell Publishing Ltd.
 E2
Hypopigmented and depigmented macules
and patches: Generalized

PC DAS, MAMTA YADAV

ABSTRACT
Hypopigmentation refers to lack of melanin pigment. Hypopigmentary disorders may be congenital or acquired, diffuse, or
localized, and may occur in isolation or may be associated with a wide range of congenital or acquired disorders. This chapter
reviews the common causes and salient features of hypopigmentary disorders presenting in a generalized manner.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

18 DOI: 10.1201/9781351054225-5
 2
Hyperpigmented macules
and patches: Localized

PC DAS, DANISH AKHTAR

INTRODUCTION others are acquired during childhood (freckles, Becker’s

nevus). A review of medications and exposure to plants and
Increased skin pigmentation results from increased number ultraviolet radiation can help determine prior drug reac-
and/or activity of melanocytes. Other pathomechanisms, tion or phototoxic reaction resulting in post-inflammatory
such as increased thickness of stratum corneum (as in acan- hyperpigmentation. Noting the distribution of lesions is
thosis nigricans), too might be responsible. The list of clini- also important. Melasma, actinic lichen planus, nevus of
cal differentials for localized cutaneous hyperpigmentation Ota, etc. typically occur on sun-exposed parts such as the
is long and includes many congenital as well as acquired face; Schamberg’s disease is mostly seen on the lower limbs
conditions. A purposeful history and physical examination in symmetrical fashion. This chapter discusses the clinical
offer valuable clues to the underlying cause. Some conditions approach to conditions presenting with localized pigmen-
are congenital (e.g., congenital melanocytic nevus), while tation outlined in Table 2.1 and their salient features are

Table 2.1  Clinical approach to localized hyperpigmented macules and patches

Onset Color Diseases

Congenital Blue-gray • Face – nevus of Ota
• Shoulder region – nevus of Ito
• Lower back – Mongolian spot
Brown-tan • Uniformly pigmented – café-au-lait macule
• Speckled – nevus spilus
Brown-black Congenital melanocytic nevus
Acquired Blue-gray Lichen planus pigmentosus, melasma
Brown-black Macules – freckles, lentigines
Patches
• Face (see Chapter 3) – melasma, lichen planus pigmentosus, solar lentigo, erythema
dyschromicum perstans, actinic lichen planus, post-chikungunya pigmentation (nose – chik
sign), periorbital hyperpigmentation, nevus of Hori, tanning
• Neck – atopic dirty neck, acanthosis nigricans
• Trunk – pityriasis versicolor (scaly), Becker’s nevus (hypertrichosis), notalgia paresthetica,
progressive cribriform and zosteriform hyperpigmentation, macular amyloidosis (upper
back), lifa disease (chest)
• Extensor extremities – macular amyloidosis
• Legs – Schamberg’s disease, diabetic dermopathy, melanoma (acral lentiginous type)
• Anywhere – post-inflammatory hyperpigmentation, morphea (indurated skin), terra
firma-forme dermatosis, frictional melanosis
Patterned – linea nigra, progressive cribriform and zosteriform hyperpigmentation, linear and
whorled nevoid hypermelanosis

DOI: 10.1201/9781351054225-6 19
20  Hyperpigmented macules and patches: Localized

Table 2.2  Diagnostic pitfalls Mongolian spot (congenital dermal

melanocytosis)
• Acanthosis nigricans – thick, velvety plaques
●● It is a developmental anomaly resulting from failure
involving flexures
• Morphea – indurated plaque, atrophy of migration of melanocytes from neural crest to
• Atrophoderma of Pierini and Pasini – slightly epidermis.
●● It manifests at birth or in the first few weeks of life and
depressed areas with well-defined “cliff-drop”
borders and no obvious induration usually disappears in childhood; rarely, it may persist
• Terra firma-forme dermatosis – brown-gray, velvety, lifelong.
●● The most commonly affected site is the lumbosacral
pigmented patches or plaques
area. Buttocks, flanks, and shoulders may be affected in
extensive lesions.
●● It presents as solitary or multiple, asymptomatic, blue-

discussed below. Certain diagnostic pitfalls, which need a gray or dark blue, round or oval or irregularly shaped
consideration while evaluating for localized pigmentation, patches with ill-defined margins (Figure 2.2a–c). The
are enlisted in Table 2.2.1–9. blue color is because of the Tyndall effect, whereby
the shorter wavelength light is reflected more via
Nevus of Ota scattering.
●● Variants include generalized (entire trunk and
●● This is also known as oculomucodermal
extremities), speckled, or persistent types.
melanocytosis or nevus fusco-ceruleus
●● Extra cutaneous signs – Few cases of extensive
ophthalmo-maxillaris.
●● It is a hamartoma of dermal melanocytes, seen in the
Mongolian spots have been reported with inborn
errors of metabolism (e.g., GM1 gangliosidosis,
distribution of the ophthalmic and maxillary branches
Hurler syndrome, Niemann-Pick disease, and Hunter
of the trigeminal nerve and thought to result from
syndrome).
failure of migration of melanocytes from neural crest to
●● DD: bruise, blue nevus.
epidermis.
●● The lesion is asymptomatic, with an onset at birth or

puberty and a predilection for females.

●● It presents as unilateral, persistent, speckled, slate

brown or bluish gray, coalescing macules and patches

over the forehead, malar area, periorbital area, temple,
nose, and oral mucosa (Figure 2.1a,b). Rarely, bilateral
lesions may be seen.
●● Pigmentation of the ipsilateral sclera is common and is

a useful diagnostic clue (Figure 2.1c–e). Glaucoma may

develop in the affected eye.
●● The lesion remains unchanged in adulthood.

Malignant transformation (malignant melanoma) is

very rare and has been reported mostly with ocular
lesions.
●● DD: Pigmentary demarcation line (PDL), melanocytic

nevus, melasma.

Nevus of Ito
●● Nevus of Ito is a dermal melanocytic hamartoma

affecting the shoulder area in the distribution of the

posterior supraclavicular and lateral cutaneous brachial
nerves.
●● This asymptomatic condition is noted at birth or soon

thereafter and is seen frequently in patients with nevus

of Ota.
●● It presents as slate-brown or bluish-gray

hyperpigmentation in the shoulder region.

●● DD: PDL, melanocytic nevus, extrafacial

melasma, bruise, post-inf lammatory Figure 2.1  (a) Blue-gray speckled pigmentation on a blu-
hyperpigmentation. ish background in nevus of Ota. (Continued)
 Hyperpigmented macules and patches: Localized  21

Figure 2.1 (Continued)  (b) A young lady with nevus of Ota on right maxillary region. (c) Ocular involvement in nevus
of Ota. (d) A young boy with ocular and cutaneous lesions of nevus of Ota. (e) More prominent ocular lesions.
(c,d – Courtesy: Dr. Piyush Kumar, Katihar, India.)
22  Hyperpigmented macules and patches: Localized

Figure 2.2  (a) Mongolian spots seen as multiple, ill-

­ defined bluish patches on lumbosacral region. (b) An
infant with ichthyosis vulgaris and Mongolian spots.
(c) Mongolian spots seen on lumbosacral region.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; c – Courtesy:
Dr Kamlesh Kumar Agrawal, Pediatric Medicine, SMS
Medical College, Jaipur, India.)

Café-au-lait macules (CALMs)

●● CALMs are light-brown to dark-brown macules and

patches commonly seen in association with various

genetic conditions (Table 2.3). Also, isolated CALM
without any underlying condition may be seen.
●● They may be seen at birth or during infancy and

may affect any site of the body. A solitary CALM is

commonly noted on buttocks.
●● They may be single or multiple, well circumscribed,

discrete, pale-brown to dark-brown, oval macules and

patches varying in size from 0.5–20 cm (Figure 2.3a,b).
●● Six or more CALMs of a size more than 5

mm in pre-pubertal individuals and 15 mm

Table 2.3  Conditions associated with CALMs

1. Neurofibromatosis type 1 and, less commonly, type 2

2. McCune Albright syndrome
3. Legius syndrome
4. Tuberous sclerosis
5. LEOPARD syndrome
6. Cowden disease
7. Bloom syndrome
8. Fanconi syndrome
 Hyperpigmented macules and patches: Localized  23

●● The background hyperpigmentation is usually oval

in shape of 3–6 cm size. But the lesion may be linear
or zosteriform (Figure 2.4d) or may be as large as
60 cm.
●● The macules and papules may be pathologically
lentigines or junctional, compound, or dermal nevi.
Less commonly, Spitz nevus and blue nevus may be
seen.

Figure 2.3  (a) Uniformly pigmented, tan brown patch

of café-au-lait macule (CALM). Many smaller lesions of
CALM are appreciable. (b) Giant lesion of CALM on the
chest with plexiform neurofibroma involving the left thigh.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

in post-pubertal individuals are suggestive of

neurofibromatosis type 1 (NF1).
●● Smaller CALMs affecting axilla (axillary freckling,
Crowe sign) and inguinal regions (inguinal freckling)
are common in NF1.
●● DD: Lentigines, nevus spilus, melanocytic nevi.

Nevus spilus (speckled lentiginous nevus)

●● Nevus spilus is a localized hyperpigmented lesion

arising due to focal proliferation of melanocytes along

the basal layer of the epidermis.
●● This asymptomatic condition presents at birth or during

infancy and may appear anywhere on the body, the

trunk being the most commonly affected site.
●● Nevus spilus presents as a well-demarcated, light brown Figure 2.4  (a) Nevus spilus seen as well-defined tan
or tan patch, speckled with black, brown, or red-brown brown patch with a few brown black macules and papules.
macules or papules of 2–3 mm (Figure 2.4a–c). (b) Nevus spilus on the face. (Continued)
24  Hyperpigmented macules and patches: Localized

●● Extracutaneous sign – Nevus spilus may be

associated with phacomatosis pigmentovascularis;
phacomatosis pigmentokeratotica; and facial features,
anorexia, cachexia, eye, and skin lesions (FACES)
syndrome.
●● DD: Partial unilateral lentiginosis, café-au-lait
macules.

Congenital melanocytic nevus (CMN)

●● Congenital melanocytic nevus is a benign proliferation

of melanocytes in nests (nevus cells) in epidermis and

dermis.
●● This asymptomatic condition usually presents at

birth but may appear later on in the first two

years of life (sometimes referred to as CMN
tardive).
●● These present as single or multiple, round or oval, well-

demarcated pigmented patches and plaques. They may

have increased hair growth (hypertrichosis), and the
surface may be papular, rough, warty, or cerebriform
with time.
●● Though many more classification systems exist, they

are usually classified as small (<1.5 cm in diameter),

medium (1.5–19.9 cm), and large or giant nevi (20 cm or
greater) (Figure 2.5a,b).
●● The large or giant nevi is surrounded by satellite

naevi.
●● Over time, CMN may darken or lighten and may

develop a more heterogeneous or homogeneous

pigmentation. There is an increase in hair growth,
and the surface becomes irregular and rough
(Figure 2.5c,d). Usually the lesion becomes thicker
and develops nodularity. Rarely the lesion may
develop a halo phenomenon and may regress
spontaneously.
●● Giant CMN – It usually affects the trunk and

extremities. It may affect more than one body segment

and may be present in “bathing trunk,” “stole,”
or “coat sleeve” distribution. Satellite lesions are
frequent. When a limb is affected, the limb may have
a reduced girth. Risk of developing melanoma in
giant CMN is high, and such melanoma carries a poor
prognosis.
●● Neurocutaneous melanosis (NCM) is characterized

by the presence of benign or malignant melanocytic

proliferations in the leptomeninges associated
with the occurrence of congenital melanocytic
Figure 2.4 (Continued)  (c) Nevus spilus on the trunk. lesions (one giant CMN, or three or more CMNs).
(d) Zosteriform nevus spilus. (a–d – Courtesy: Dr. Piyush Central nervous system symptoms result from
Kumar, Katihar, India.) increased intracranial pressure and include
headache, vomiting, irritability, increased head
●● Speckled lentiginous nevus syndrome refers to a circumference, seizures, papilledema, and
neurocutaneous condition in which ipsilateral neurological sometimes cranial nerve palsies.
abnormalities are noted in association with SLN. ●● DD: Becker’s nevus, nevus of Ota/Ito.
 Hyperpigmented macules and patches: Localized  25

  

  

Figure 2.5  (a) Well-defined, black patch with lesional hypertrichosis in congenital melanocytic nevus (CMN). (b) Giant con-
genital melanocytic nevus (CMN) on the back of an infant. (c) Lesional hypertrichosis in CMN. (d) Giant CMN with hypertri-
chosis. (c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
26  Hyperpigmented macules and patches: Localized

Lichen planus pigmentosus (LPP) ●● DD: Erythema dyschromicum perstans (raised

●● This is a variant of lichen planus with unknown etiology. erythematous border and lesions in sun-protected
●● It most commonly occurs in young to middle-aged adults, areas), fixed drug eruption, actinic lichen planus (fine
with an increased predilection in darker-skinned people. scaling over violaceous lesions).
●● Triggering factors are sunlight, hepatitis C infection,

exposure to dye, and application of mustard oil or Freckles (ephelides)

amla oil. ●● Freckles are an acquired pigmentary condition.
●● The disease usually starts on the face and neck, ●● The condition typically affects sun-exposed areas.

spreading later to the upper extremities and trunk. ●● Both genetic and environmental factors have role in its
●● The initial lesions are small, brown, oval macules with development.
diffuse borders. Later, they merge to form pigmented ●● Both the sexes are affected.

areas that are gray or brown (Figure 2.6a–c). ●● It may occur in any age group and race, with
●● The pigmentation may be diffuse, reticulate, blotchy, predominance among skin types I and II.
or perifollicular. The patches are usually symmetrical ●● It is characterized by brown macules of irregular shape

in distribution but may be found in a segmental, and < 0.5 cm of size (Figure 2.7).
zosteriform, or Blaschkoid pattern. ●● It is typically seen on sun-exposed areas such as face,
●● The rare sites of involvement are axilla, inframammary area, neck, shoulder, and dorsum of hand.
and groin; palm, soles, oral mucosa, and nails are spared. ●● Summer aggravation is also characteristic, with fading
●● Lesions are asymptomatic, but mild pruritus and of lesion during winter.
burning can be seen in some patients. ●● DD: Lentigines.

Figure 2.6  (a) Blue-gray pigmented patches of lichen planus

pigmentosus on the dorsum of hand. (b) Blue-gray pigmented
patches of lichen planus pigmentosus on the face and ear.
(c) Multiple, well-defined, brown patches on the flexor
aspect of the forearms. (a–c – Courtesy: Dr. Piyush Kumar,
Katihar, India.)
 Hyperpigmented macules and patches: Localized  27

●● The condition is typically seen in fair-skinned

individuals with red hair.
●● It is most commonly seen on the face, shoulder, upper
chest, and dorsum of the hand.
●● It presents as hyperpigmented, brown to black,
round to oval macules in sun-exposed areas
(Figure 2.8a,b).
●● The macules gradually increase in size and number.
●● In long-standing cases, many lesions coalesce to from
larger lesions.
●● DD: Lichenoid keratosis, seborrhoeic keratosis,
junctional melanocytic nevus.

Lentigo simplex
Figure 2.7  Multiple brown macules on the sun-exposed ●● This is the most common type of lentigo.
parts of the face. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
●● The exact etiology of the condition is unknown, but

both environmental and genetic factors are found to

have a role in its development.
Solar lentigo ●● It is seen in childhood but can appear at birth also.
●● This is an acquired benign pigmentary skin condition ●● It may occur in both sexes and any age group, but

due to chronic sun exposure. genetically determined lesions are common among
●● Synonyms are sun spots, liver spots, or senile lentigo. children.
●● It is most commonly seen in the 30 to 50 year old age ●● It is not associated with sun exposure or systemic

group but can occur in any age group having chronic disease.
exposure to sun. ●● It is characterized by asymptomatic sharply defined,
●● It is not associated with any systemic condition. round to oval, dark-brown to black (evenly distributed

Figure 2.8  (a) Well-defined, brown-black, smooth-surfaced patch of solar lentigo. (b) Brown smooth-surfaced patch
(arrow) of solar lentigo. Also, seborrheic keratosis are appreciable as pigmented papules. (a,b – Courtesy: Dr. Piyush
Kumar, Katihar, India.)
28  Hyperpigmented macules and patches: Localized

  

Figure 2.9  (a) Well-defined, brown-black macules of lentigines on the face. (b) Extensive lentigines in Carney complex.
(Courtesy: Dr. Piyush Kumar, Katihar, India.)

pigmentation) macules that are 3–15 mm in diameter

(Figure 2.9a).
●● Lesions may occur anywhere in skin and mucosa and
are not associated with sun exposure.
●● Genetic syndromes associated with lentiginosis are Carney
complex (Figure 2.9b), LEOPARD syndrome, Peutz-
Jeghers syndrome, Bannayan-Riley-Ruvalcaba syndrome,
progressive cribriform and zosteriform hyperpigmentation,
phakomatosis pigmentovascularis, transient pustular
melanosis, and multiple Mongolian spots.
●● DD: Freckles, multiple lentigines syndrome.

Segmental lentigines
●● Lentigines are benign proliferation of melanocytes

(without forming nests).

●● Segmental lentigines have their onset in early childhood

and do not aggravate on sun-exposure.

●● The patient presents with numerous, grouped, brown to

black lentigines within an area of normal skin, along one

or more dermatomes, and the lesion respects the midline
(Figure 2.10). The lesions may be unilateral or bilateral or
present in a checkerboard pattern or in midline clusters.

Figure 2.10  Lentigines in segmental distribution. The

intervening skin between the macules appears normally
pigmented. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Hyperpigmented macules and patches: Localized  29

●● DD: Nevus spilus (background skin is tan to light- 2. Malar pattern involving the cheeks and nose
brown patch). (Figure 2.11c).
3. Mandibular pattern involving the ramus of the
Melasma mandible.
●● It typically affects women of reproductive age with

Fitzpatrick skin types IV–VI, though the condition can

occur in men also.
●● Genetic predisposition; ultraviolet (UV) radiation

exposure; hormonal factors such as female sex

hormones, thyroid disease, pregnancy, taking
contraceptive pills; and drugs like phenytoin are
known risk factors.
●● Pigmentation usually fades after delivery or on stopping

the pill, but it may persist.

●● It manifests as symmetric hyperpigmented

macules, and patches on the face, involving

the forehead, cheeks, and moustache area and
occasionally V-area of the neck. The lesions darken
after sun exposure.
●● According to the distribution of lesions, three clinical

patterns of melasma are recognized:

1. Centrofacial pattern is the most common pattern
and involves the forehead, cheeks, upper lip, nose,
and chin (Figure 2.11a,b).

Figure 2.11  (a) Melasma as brown-pigmented patches on the cheek, forehead, nose, and upper lip area. (b) Melasma in a
man. (c) Melasma affecting the nasal tip. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
30  Hyperpigmented macules and patches: Localized

●● Using the Wood’s light examination, melasma can be

classified into four major histological types depending
upon the depth of pigment deposition:
1. Epidermal type: Pigmentation is intensified under
Wood’s light and is the most common type.
Melanin is increased in all epidermal layers.
2. Dermal type: There is no accentuation of pigmen-
tation under Wood’s light.
3. Mixed type: Pigmentation becomes more apparent
in some areas, while in others there is no change.
4. Indeterminate type: Pigment is apparent in the
Wood’s light in individuals with skin type VI.
●● DD: Freckles, solar lentigo, Riehl’s melanosis, post-
inflammatory hyperpigmentation, frictional melanosis,
ochronosis (endogenous and exogenous), acanthosis
nigricans.

Erythema dyschromicum perstans (EDP)

●● It is present in the second to third decade of life but can

occur even in younger patients.

●● Individuals with skin phototypes III and IV are more

frequently affected.
●● It presents as symmetrical distribution of slate-gray

to brown-blue asymptomatic macules and patches of

variable shapes and sizes (0.5–3 cm).
●● It is mostly distributed over the face, neck, trunk, and

proximal upper extremities. The palms, soles, and scalp

are rare sites of involvement.
●● Mucosae are spared. Figure 2.12  Well-defined gray-black patch of actinic
●● Mild pruritus can be noted. lichen planus with perilesional halo.
●● Early lesions present with a thin, raised, erythematous

border that tends to resolve over months.

●● Peripheral hypopigmentation can be seen in older ●● Lichenoid papules and plaques may occur
lesions. simultaneously with annular lesions or may occur
●● DD: Lichen planus pigmentosus (dark brown macules, independently as a sole manifestation of actinic LP.
sun-exposed areas), lichenoid drug eruptions (PIH and ●● DD: Fixed drug eruption, actinic prurigo, actinic
alopecia), generalized fixed drug eruptions (sharply keratosis, lichenoid drug eruption, granuloma annulare.
demarcated, dusky, violaceous hue).
Post-Chikungunya pigmentation (PCP)10
Actinic lichen planus (LP) ●● Chikungunya fever is an acute, usually self-limiting,
●● Actinic LP is a variant of LP seen mostly in dark- febrile illness caused by the Chikungunya virus.
skinned people. Unlike classical LP, Koebner ●● In the acute stage, the patient complains of fever,

phenomenon is not seen in actinic LP. intense asthenia, arthralgia, myalgia, headache, sore
●● The condition is mostly noted in young adults, and throat, conjunctivitis, and maculopapular or other skin
onset is frequently in spring and summer. eruptions.
●● Actinic LP typically affects the sun-exposed areas. ●● Pigmentation develops usually two weeks after the

The lateral aspects of the forehead, the dorsa of hands, rash, when febrile symptoms have already subsided;
the extensor parts of upper extremities, the lower hence, the pigmentation is called post-Chikungunya
lips, the cheeks, and the neck are the most commonly pigmentation.
involved sites. Sun-covered areas may be involved rarely ●● Pigmentation manifests as hyperpigmented macules and

(Figure 2.12). patches over the nose (chik sign) and cheeks without any
●● Oral mucosa and nails are spared. The most common preceding erythema during the febrile illness (Figure 2.13).
clinical presentation is single or multiple annular However, the pigmentation may also involve the trunk,
plaques with thready rolled border and hyperpigmented flexures, palmar creases, and mucosa.
center. The lesions are frequently surrounded by a ●● Other patterns of pigmentation are discrete confetti-like

hypopigmented halo. macules, freckle-like, diffuse, flagellate, Addisonian

 Hyperpigmented macules and patches: Localized  31

Figure 2.13  Post-chikungunya nasal tip pigmenta-

tion (chik sign). (Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India.)

type of palmar pigmentation, periorbital melanosis, and

pigmentation of pre-existing acne lesions.
●● Patients may have persistent asthenia and joint pain in
the convalescence period.
●● DD: Melasma, freckles, and lentigines (PCP has an
acute onset and follows febrile illness).
Nevus of Hori11
Figure 2.14  Speckled, slaty gray macules on side of the
●● This is also known as acquired bilateral nevus of Ota-like
face in nevus of Hori in a woman. (Courtesy: Dr. Zubin
macules (ABNOM) and nevus fuscocaeruleus zygomaticus. Mandlewala, Mumbai, India.)
●● It is mostly seen in Asian women after the third decade of life.

●● It presents as asymptomatic, multiple, speckled brown or

slate-gray macules occurring bilaterally on malar regions

Atopic dirty neck
without ocular or mucosal pigmentation (Figure 2.14).
●● Atopic dirty neck is acquired hyperpigmentation,
●● Forehead, upper eyelids, cheeks, and nose are less

commonly affected. characteristically seen in patients with atopic dermatitis.

●● Pigmentation has a rippled pattern; it occurs due to
●● The color of lesions varies with the maturity of the lesions.

Initially, these macules are usually brown and discrete and the combined effect of both frictional melanosis and
become bluish-gray and diffuse over the time. post-inflammatory hyperpigmentation in patients with
●● This may coexist with other facial pigmentary disorders atopic dermatitis.
●● It is aggravated by itching, heat and sweat.
like melasma, freckles, solar lentigines, and nevus of Ota.
●● It usually affects the anterolateral aspect of neck, axilla,
●● DD: Nevus of Ota (mucosal and conjunctival

involvement), melasma, Riehl’s melanosis. and flexures.

●● DD: Acanthosis nigricans.
Periorbital hyperpigmentation
●● Dark circles or periorbital hyperpigmentation is a Poikiloderma of Civatte
common complaint affecting individuals of both ●● This condition is also known as cervical idiopathic

genders of all ages and races. poikiloderma.

●● The condition is characterized by bilateral homogeneous ●● It is characterized by irregular pigmentary alterations,

hyperchromic areas in the periorbital region. with hypo- and hyperpigmentation, superficial
●● Lesions may vary in intensity according to fatigue or atrophies and telangiectasias (Figure 2.15).
lack of sleep and may worsen with aging due to sagging, ●● It is usually asymptomatic; burning or pruritus may be

thinning of skin, and loss of subcutaneous fat. present rarely.

32  Hyperpigmented macules and patches: Localized

Figure 2.15  Reddish-brown, reticulate hyperpigmentation

on neck in poikiloderma of Civatte. (Courtesy: Dr. Hiral
Shah, Baroda Medical College, Vadodara, India.)

●● Pigmentation is reticulate, reddish to brown, irregular

and symmetrically distributed.
●● It affects the face, neck, and upper third of chest; it
spares the shaded chin area.
●● DD: Acanthosis nigricans, atopic dirty neck, pigmented
contact dermatitis.

Schamberg’s disease
●● This is an acquired condition characterized by

extravasation of erythrocytes in the skin with marked

hemosiderin deposition.
●● Risk factors are hypertension, diabetes mellitus, venous

stasis, strenuous exercise, and occupations involving

prolonged standing.
●● The legs are affected in a bilateral symmetrical

manner. Rarely, other sites such as the trunk and upper

extremities may be involved (Figure 2.16). Figure 2.16  Localized brown macules and patches on
●● The initial clinical presentation is characteristic orange-
the lower leg. Some erythematous macules are still
brown, speckled macules and papules (cayenne pepper– appreciable.
like spots).
●● In most cases, lesions rarely progress proximal to the

knee. ●● Individual lesions persist for months together (average

●● DD: Venous stasis, post-inflammatory
18–24 months), and then either the pigmentation fades
hyperpigmentation, diabetic dermopathy. or the lesion clears completely.
●● DD: healed lesions of prurigo nodularis and lichen
Diabetic dermopathy planus, pretibial myxedema.
●● Diabetic dermopathy is characterized by light brown or

reddish, oval or round, slightly indented scaly patches Acral lentiginous melanoma (ALM)
most often appearing on the shins. (see Chapter E27)
●● Although these lesions may appear in anyone, ●● ALM presents as a slowly expanding, solitary,

particularly after an injury or trauma to the area, they pigmented patch on the palm or sole. The lesion
are one of the most common skin problems found in typically shows color variation (brown, blue-gray,
patients with diabetes mellitus. black) and is flat and smooth-surfaced to begin with.
●● Established lesions are characterized by asymptomatic, Gradually the lesion becomes thicker and the surface
round or elongated, brown, atrophic patches of 1 cm to shows hyperkeratosis, ulceration, and bleeding
2.5 cm size. (Figure 2.17a,b).
 Hyperpigmented macules and patches: Localized  33

Figure 2.17  (a) solitary hyperpigmented patch on sole

in acral lentiginous melanoma with surface ulceration
and hyperkeratosis. (b) Acral lentiginous melanoma.
(a – Courtesy: Dr. Piyush Kumar, Katihar, India; b –
Courtesy: Dr. Sasi Kiran Attili, Visakha Institute of Skin and
Allergy, Visakhapatnam, India.)

●● Clinical pointers of ALM (ABCDE)

●● Asymmetry of lesion
●● Border – irregular
●● Color – variegated
Figure 2.18  Pityriasis versicolor seen as hyperpigmented
●● Diameter – > 6 mm patches with fine scaling. (Courtesy: Dr. Piyush Kumar,
●● Evolution – increasing size and thickness; Katihar, India.)
development of ulceration

Pityriasis versicolor (tinea versicolor)

●● Though hypopigmented lesions are more ●● Wood’s lamp examination – yellow green fluorescence.
common, some patients may develop hyperpigmented ●● DD: Post-inflammatory hyperpigmentation (for
lesions too. Sometimes, both types of lesions hyperpigmented lesions of PV).
co-exist.
●● The lesions may be asymptomatic or mildly itchy. Acquired brachial cutaneous dyschromatosis
●● A history of hyperhidrosis is often present. (ABCD)12
●● It commonly affects the trunk, neck, and arms. ●● ABCD is an acquired pigmentary disorder of unknown
●● Patients present with hyperpigmented macules etiology. Chronic sun-exposure is thought to play a role.
and patches with bran-like (furfuraceous) scales ●● This condition is mostly reported in older women.

(Figure 2.18). ●● ABCD presents as asymptomatic, reticulated, gray-

●● Lesions start at perifollicular locations and coalesce to brown patches with an irregular geographical border,
form bigger patches. interspersed with hypopigmented macules, present
●● Stretching the skin of hyperpigmented scaly macules bilaterally on the extensor surface of the forearms.
and patches reveals fine scaling (Besnier sign). ●● DD: Extrafacial melasma, macular amyloidosis.
34  Hyperpigmented macules and patches: Localized

Becker’s nevus hairs develop in and around the lesions several months
●● Becker’s nevus is an acquired disorder of pigmentation after appearance of pigmentation. The central area in
of unknown etiology. The lesional skin shows increased the patch thickens over time and develops acneiform
androgen receptors. lesions. Rarely, bilateral lesions may be observed.
●● This asymptomatic condition has its onset in the ●● Becker’s nevus syndrome occurs when Becker’s nevus is
peripubertal period, with a male preponderance. found in association with unilateral breast hypoplasia
●● The most commonly affected areas are the shoulder and muscle, skin, and/or skeletal abnormalities.
region, upper chest, and back. ●● DD: Congenital melanocytic nevus, nevus of Ito, Linear
●● Patients present with asymptomatic, unilateral, and whorled nevoid hypermelanosis.
irregular tan or brown patches. New pigmented macules Notalgia paresthetica (NP)
and patches develop at the periphery and coalesce with
●● Notalgia paresthetica is a localized neuropathy
the larger patch (Figure 2.19a,b). Frequently, thick
and dysesthesia syndrome classically affecting the
infrascapular area unilaterally. Many patients have
associated cervical disease and/or brachioradial
pruritus.
●● The condition is primarily noted in adults and older

populations and may have a female preponderance.

●● Patient complains of localized burning, pain,

tenderness, hyperalgesia, or dysesthesias. Infrascapular

area is typically affected, but upper back, neck, scalp,
and shoulder areas too may be affected in some cases.
●● The skin finding is a unilateral, ill-defined, tan,

or hyperpigmented non-indurated patch on the

infrascapular area (mid back). The size of lesion usually
ranges from 3–10 cm (Figure 2.20).
●● Skin changes secondary to itching and chronic

rubbing include excoriations, secondary infection,

lichenification, lichen amyloid, eczematous changes,
and xerosis.
●● The disease runs a chronic course, with a natural

tendency to wax and wane.

●● DD: Macular amyloidosis, café-au-lait macule.

Figure 2.20  Localized pigmentation on the scapular

Figure 2.19  (a) Brownish patch with lesional hypertrichosis region in notalgia paresthetica. Bottom part of lesion
in Becker’s nevus. (b) Becker’s nevus involving right chest shows slight lichenification. (Courtesy: Dr. Deverashetti
and arm. (a – Courtesy: Dr. Piyush Kumar, Katihar, India.) Srinivas, Nizamabad, India.)
 Hyperpigmented macules and patches: Localized  35

Figure 2.22  Localized pigmentation of the skin over

clavicles in lifa disease. (Courtesy: Dr. Piyush Kumar,
Katihar, India.)
Figure 2.21  (a) Dirty-looking patch of pigmentation on
the upper back in macular amyloidosis. (b) Pigmented
macules in the rippled pattern in macular amyloidosis. ●● The epidermis, especially the basal layer, gets squeezed
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.) between the washing agent and the underlying bone,
which leads to pigmentary incontinence.
●● DD: macular amyloidosis, post-inflammatory
Macular amyloidosis (MA) hyperpigmentation.
●● Macular amyloidosis (MA) is the most subtle form

of cutaneous amyloidosis, characterized by brownish Post-inflammatory hyperpigmentation (PIH)14

●● Post-inflammatory hyperpigmentation (PIH) is
macules in a rippled pattern, distributed predominantly
over the trunk and extremities. a common, acquired pigmentary disorder caused
●● Clinically, MA presents as poorly delineated by various cutaneous endogenous inflammatory
hyperpigmented patches of grayish-brown macules with conditions, external injury, or cutaneous procedures
a rippled pattern, associated with deposition of amyloid (Table 2.4) and is common in dark-skinned
material in the papillary dermis (Figure 2.21a,b). individuals.
●● The sites most commonly involved are the interscapular

area and extremities (shins and forearms), although Table 2.4  Common causes of post-inflammatory
involvement of the clavicles, breast, face, neck, and hyperpigmentation
axilla have also been reported. Inflammatory Acne/acneiform eruption, eczema,
●● DD: Tinea versicolor, post-inflammatory
dermatoses psoriasis, lichen planus,
hyperpigmentation, notalgia paresthetica. dermatomyositis, lupus erythematosus,
vasculitis, pemphigs vulgaris, bullous
Lifa disease13
pemphigoid
●● Lifa disease (frictional dermal melanosis) is a common
Infections Impetigo, viral exanthem, chickenpox,
pigmentary disfiguring problem, especially among herpes zoster, dermatophytosis
females, and is usually seen in the people in the Middle
Cutaneous Phototoxic dermatitis, erythema
East and other parts of Asia.
adverse drug multiforme, fixed drug eruption,
●● It most probably appears as a result of using rough
reactions Stevens-Johnson syndrome/toxic
washing agents during bathing, such as lifa, scrub pads,
epidermal necrolysis
or nylon towels.
Dermatological Chemical peel, dermabrasion, laser
●● The disease mainly affects slim subjects and is
procedures treatment
characterized by pigmentation that occurs bilaterally and
Trauma Mechanical trauma, friction
symmetrically over bony prominences such as clavicular
areas, upper back, upper arms, and shins (Figure 2.22). Miscellaneous Neurotic excoriation, sunburn
36  Hyperpigmented macules and patches: Localized

●● There is a history of inflammatory dermatoses, trauma, Linea nigra

or cutaneous procedures, and PIH lesions are preceded ●● Linea nigra is a physiological hyperpigmentation
by erythema. occurring during pregnancy.
●● Two clinical forms are recognized – epidermal and ●● It is clinically characterized by linear
dermal. Some patients may have mixed patterns. hyperpigmentation that appears over the central
●● Epidermal PIH presents as well circumscribed, light abdomen during pregnancy; it is mainly seen in the
to dark brown macules and patches at the site of prior darker-skinned population (skin types IV and V).
dermatoses/trauma, etc. (Figure 2.23a,b). ●● It develops due to the combined effect of
●● Dermal PIH presents as blue-gray patches with ill- placental hormone, metabolic factors, and
defined margins. immunological factors; increased melanin
●● Epidermal PIH may disappear spontaneously within production is associated with hyperestrogenic
months or years, whereas dermal PIH usually persists. state of pregnancy.
●● PIH due to fixed drug eruption (FDE) is usually round ●● DD: Post-inflammatory hyperpigmentation.
and brown black or gray-blue black in color. The
appearance of PIH is so characteristic that it allows Progressive cribriform and zosteriform
recognition of prior FDE. History of intermittent hyperpigmentation (PCZH)15
erythema, edema, and burning on exposure to certain ●● This condition appears usually after the second decade

drug confirms the diagnosis of FDE (Figure 2.23c). of life.

Common offending drugs are analgesics, antibiotics, ●● It presents as hyperpigmented, progressive, and

muscle relaxants, and anticonvulsants. asymptomatic macules and patches that are uniformly
●● Of note, pseudoephedrine, piroxicam, cotrimoxazole, tan with zosteriform distribution with cribriform
sorafenib, and tadalafil may be associated with non- configuration.
pigmenting FDE, and no PIH is seen. ●● It is arranged in whorls and streaks and follows the lines
●● DD: Freckles, lentigines, melasma. of Blaschko.

   

Figure 2.23  (a) Post-inflammatory hyperpigmentation following resolution of lichen planus. (b) Post-inflammatory hyper-
pigmentation after traumatic injury to skin. (c) Round-shaped, post-inflammatory hyperpigmentation following fixed drug
eruption. Some erythema is appreciable as there is reactivation of lesions.
 Hyperpigmented macules and patches: Localized  37

Table 2.6  Pigmentary mosaicism – the unifying concept17,18

• The term “pigmentary mosaicism” is proposed to

encompass all pigment anomalies caused by
chromosomal mosaicism. It refers to patterned
hypo- or hyperpigmentation resulting from a clone of
cells with altered ability to produce melanin.
• Three major clinical patterns are established:
• Streaks and swirls following the Blaschko lines: It
includes previously described entities like
hypomelanosis of Ito, linear and whorled nevoid
hypermelanosis, progressive cribriform and
zosteriform hyperpigmentation, etc.
• Segmental or “checkerboard” distribution: It
includes segmental nevus depigmentosus,
segmental pigmentation disorder, etc.
• Phylloid (leaf-like) pattern: It includes leaf-like, pear-
shaped, and oblong lesions of pigmentary alteration
as seen in mosaic abnormalities of chromosome 13.
• Individuals showing pigmentary mosaicism may have
Figure 2.24  Hyperpigmented macules in a whorled pat-
multiple congenital abnormalities, developmental
tern along Blaschko’s lines in linear and whorled nevoid
delays, and mental retardation.
hypermelanosis. (Courtesy: Dr. Shekhar Neema, Armed
Force Medical College, Pune, India.) • Sometimes both hypopigmented and
hyperpigmented patches may be seen (cutis tricolor).
●● The trunk is mainly involved, and it may extend into
pubic areas. deafness, and brachydactyly have been mentioned in the
●● DD: Linear and whorled nevoid hypermelanosis literature in association with LWNH.
(childhood), linear lichen planus (violaceous color), ●● DD: Incontinentia pigmenti, hypomelanosis of Ito,
third stage of incontinentia pigmenti (congenital). epidermal nevus, PCZH (Tables 2.5 and 2.6).
Linear and whorled nevoid hypermelanosis (LWNH)16
●● Linear and whorled nevoid hypermelanosis (LWNH) is REFERENCES
a rare disorder of pigmentation. 1. Stulberg DL, Clark N, Tovey D. Common hyperpigmentation dis-
●● It is characterized by hyperpigmented macules in a orders in adults: Part I. Diagnostic approach, café au lait macules,
streaky configuration along lines of Blaschko without diffuse hyperpigmentation, sun exposure, and phototoxic reac-
preceding inflammation or atrophy (Figure 2.24). tions. Am Fam Physician 2003;68(10):1955–1960.
●● It is distributed mainly on the trunk and extremities,
2. Stulberg DL, Clark N, Tovey D. Common hyperpigmentation
disorders in adults: Part II. Melanoma, seborrheic keratoses, acan-
sparing palms, soles, and mucosae. thosis nigricans, melasma, diabetic dermopathy, tinea versicolor,
●● The onset of hyperpigmentation usually occurs within and post-inflammatory hyperpigmentation. Am Fam Physician
the first few weeks of life and continues to progress for a 2003;68(10):1963–1968.
year or two before stabilization. 3. Vashi N, Kundu R. Congenital and inherited hyperpigmenta-
●● Various skeletal anomalies, central nervous system
tion disorders. In: Stratman E, Corona R, editors. UpToDate.
Available from https://www.uptodate.com/contents/
diseases (microcephaly, arhinencephaly and congenital-and-inherited-hyperpigmentation-disorders
epilepsy), congenital heart diseases (ventricular septal 4. Taıeb A, Ezzedine K, Morice-Picard F. Diagnosis of some com-
defect and tetralogy of Fallot), psychomotor delay, mon and uncommon hyperpigmentation disorders in children.
Dermatologica Sinica 2014;32:211–216.
5. Sori T, Jaisankar T J, Thappa DM, Nath AK. Hyperpigmentary dis-
Table 2.5  Difference between linear and whorled nevoid
orders in children: A hospital-based study in a tertiary care center.
hypermelanosis and progressive cribriform and Indian Dermatol Online J 2013;4:148–152.
zosteriform hyperpigmentation 6. Plensdorf S, Livieratos M, Dada N. Pigmentation disorders: Diagnosis
and management. Am Fam Physician 2017;96(12):797–804.
LWNH PCZH 7. Rendon MI. Hyperpigmentation disorders in Hispanic population in
the United States. J Drugs Dermatol 2019;18(3):s112–s114.
Appear early in life, progress in Usually appear after
8. Lipsker D, Lenormand C. Hyperpigmentations [Hyperpigmentation].
first two years and then second decade of life Ann Dermatol Venereol 2019;146(10):666–682.
eventually stabilize 9. Nieuweboer-Krobotova L. Hyperpigmentation: Types, diagnostics
Diffuse lesions appearing in Localized and targeted treatment options. J Eur Acad Dermatol Venereol
streaks and swirls pigmentation 2013;27 Suppl 1:2–4.
10. Ghosh S, Das A, Kumar P. Chikungunya and pigmentation: A tale of
Associated with other anomalies No such association two cases. Pigment Int 2018;5:59.
38  Hyperpigmented macules and patches: Localized

11. Park JM, Tsao H, Tsao S. Acquired bilateral nevus of Ota-like mac- 17. Thapa R. Pigmentary mosaicism: An update. Indian J Dermatol
ules (Hori nevus): Etiologic and therapeutic considerations. J Am 2008; 53(2):96–97.
Acad Dermatol 2009;61(1):88–93. 18. Kromann AB, Ousager LB, Ali IKM, Aydemir N, Bygum A.
12. Rongioletti F, Rebora A. Acquired brachial cutaneous dyschroma- Pigmentary mosaicism: a review of original literature and
tosis: A common pigmentary disorder of the arm in middle-aged recommendations for future handling. Orphanet J Rare Dis.
women. J Am Acad Dermatol 2000;42(4):680–684. 2018;13(1):39.
13. Sharquie KE, Al-Dorky MK. Frictional dermal melanosis (lifa dis-
ease) over bony prominences. J Dermatol 2001;28(1):12–15.
14. Kaufman BP, Aman T, Alexis AF. Postinflammatory hyperpigmenta- SUGGESTED READING
tion: Epidemiology, clinical presentation, pathogenesis and treat-
1. Dimitris Rigopoulos, Alexander C. Katoulis (Eds).
ment. Am J Clin Dermatol 2018;19(4):489–503.
Hyperpigmentation. 1st edition. 2017. CRC Press.
15. Das A, Bandyopadhyay D, Mishra V, Gharami RC. Progressive
2. James J. Nordlund, Raymond E. Boissy, Vincent J. Hearing,
cribriform and zosteriform hyperpigmentation. Indian J Dermatol
Richard A. King, William S. Oetting, Jean-Paul Ortonne (Eds). The
Venereol Leprol 2015;81:321–323.
Pigmentary System: Physiology and Pathophysiology. 2nd edition.
16. Sinha P, Chatterjee M, Singh KK, Sood A. Linear and whorled
2006. Blackwell Publishing Ltd.
nevoid hypermelanosis with depigmentation. Indian Dermatol
Online J 2017;8:131–133.
 3
Acquired facial melanosis

PREETI SHARMA, ANUPAM DAS

INTRODUCTION Patients frequently develop more than one pattern of

involvement.
Acquired facial melanosis is a common presentation
in the dermatology out-patient department (OPD). It ●● Extrafacial lesions are uncommon but may affect the
adversely affects the psycho-social life of the patient neck, sternum, and forearms.
and creates a great diagnostic dilemma for the treating ●● DD: Ephelids, lentigines, acanthosis nigricans, pigmen-
dermatologist, especially because of overlapping clini- tary demarcation lines, Hori’s nevus, exogenous ochro-
cal presentations. This chapter discusses the clinical nosis, Riehl’s melanosis, lichen planus pigmentosus.
approach to common disorders causing facial melanosis,
as outlined in Figure 3.1 and Table 3.1.1–5 Face may be Lichen planus pigmentosus (LPP)
involved in many congenital or acquired pigmentary dis- ●● LPP, a variant of lichen planus, occurs more predomi-
orders causing widespread involvement of the body; they nantly in Fitzpatrick skin types IV–VI.
are not discussed here. ●● Various photosensitizers, such as cosmetic fragrances,

hair dyes, and mustard oil, have also been implicated.

Melasma ●● Patient presents with symmetrical, diffuse or patchy,

●● Melasma is common in darker individuals, more com- gray-brown to dark-brown macules and patches over
mon in women than in men (9:1), and seen mostly in sun-exposed areas and sun-protected flexural skin over
reproductive-age groups. inguinal and axillary areas (Figure 3.3a–d).
●● Over time, lesions coalesce to form bigger patches and
●● Various risk factors implicated are

1. Ultraviolet radiation new lesions appear.

●● The morphological variants that have been described
2. Elevated levels of estrogens and progesterone(like
in pregnancy) are diffuse, reticular, blotchy, perifollicular, and
3. Thyroid disorders (Thyroid-stimulating hormone annular.
●● If it predominantly affects the trunk and flexural areas,
[TSH] and adrenocorticotropic hormone [ACTH]
resemble α-melanocyte-stimulating hormone it is known as LPP inversus, which is a variant of LPP.
[α-MSH]) Sometimes, linear or variants overlapping with lichen
4. Genetic factors planus can be present.
●● DD: Erythema dyschromicum perstans (EDP),
5. Drugs like phenytoin, griseofulvin, NSAIDs, etc.
●● Melasma presents as bilateral, symmetrical, asymptom- melasma, post-inflammatory hyperpigmentation, phy-
atic, hyperpigmented macules over sun-exposed areas tophotodermatitis (Figure 3.3e).
of the face (malar areas, nose, forehead, mandibular
areas) (Figure 3.2a–c). Erythema dyschromicum perstans (EDP)
●● The lesions tend to coalesce to form larger patches. ●● EDP is also known as erythema chronicum figuratum

●● The lesions may follow various patterns, such as blotchy, melanodermicum, and ashy dermatosis of Ramirez.
polycyclic, arcuate, and irregular. ●● The exact etiology is not known; various factors, such as

●● Facial melasma is clinically divided into the following ammonium nitrite, cobalt, HIV, radiocontrast media,
three types: drugs (such as ethambutol, penicillin, and BZDs), and
1. Centrofacial whipworm infestation, are implicated.
2. Malar ●● EDP commonly affects the trunk and is distributed

3. Mandibular symmetrically. Face involvement may be seen.

DOI: 10.1201/9781351054225-7 39
40  Acquired facial melanosis

A. Diffuse facial melanosis

Sun-exposed areas Others

• Addison’s disease • Lateral face – Exogenous

• Nutritional – pellagra, ochronosis
vitamin B12 deficiency • Random – Lichen planus
• Phytophotodermatitis pigmentosus (LPP), erythema
dyschromicum perstans (EDP)
• Drug-induced melanosis
(psoralens, minocycline,
cyclophosphamide,
clofazimine)
• Tanning

Figure 3.1  Approach to diffuse facial hyperpigmentation.

Table 3.1  Approach to acquired focal facial melanosis

●● Early lesions start as erythematous macules with ery-
thematous raised borders; they later develop brown or
Site Diseases gray macules.
Periorbital • Brown/brown-black – Pigmentary
●● DD: LPP (Table 3.2), Riehl’s melanosis, Lichenoid drug
demarcation lines type F and G, reaction.
periocular melanosis Maturational pigmentation6
• Slate-gray – Post-inflammatory hyperpig- ●● This presents as darkening of sun-exposed skin in the
mentation due to fixed drug eruptions fourth to fifth decade.
• Blue-gray – nevus of Ota, nevus of Hori
Perioral • Peribuccal pigmentation of Brocq
• Pigmentary demarcation line type H
Nose • Nasal tip – melasma, post-Chikungunya
pigmentation (chik sign)
• Alar groove – seborrheic melanosis
• Nasal salute (transverse nasal line –
atopic dermatitis)
Malar area • Predominantly discrete macules –
freckles, lentigines
• Macules and patches
• Brown-black – melasma
• Blue-gray – nevus of Ota, nevus of Hori
• Scattered depigmented macules, caviar
like papules – exogenous ochronosis
Cheeks/ • Extending from infraorbital area –
lateral pigmentary demarcation lines
face • Associated tiny papules –
erythromelanosis follicularis faciei et colli
• Pebbly appearance, textural changes –
acanthosis nigricans
• Others – Riehl’s melanosis, maturational
hyperpigmentation
• Lichen planus pigmentosus – face and
flexures
Forehead • Along hairline – Riehl’s melanosis
• Convex areas – macular amyloidosis,
frictional melanosis
Figure 3.2  (a) Melasma as symmetric, brown patches on
• Concave areas – facial acanthosis nigricans
the face, mainly affecting forehead, cheeks, and nose.
• Sun exposed – melasma
(Continued)
 Acquired facial melanosis  41

  

Figure 3.2 (Continued)  (b) Melasma on lateral face in a middle-aged male. (c) Extensive melasma involving forehead,
malar areas, and nose. Lentigines and freckles too are noted. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● It appears especially in skin types V and VI. ●● The dorsum of hands and feet may also be affected.
●● It affects the lateral aspects of face (zygomatic areas) and ●● The separate existence of this entity is debatable, and
merges with surrounding skin with blurred, ill-defined some authors consider it as early facial acanthosis nigri-
margins (Figure 3.4a,b). cans or frictional melanosis.

Figure 3.3  (a) Sometimes lichen planus pigmentosus may be extensive and may cause extensive involvement of the face
and neck. (b) Diffuse lesions of lichen planus pigmentosus in a young male. (Continued)
42  Acquired facial melanosis

    

Figure 3.3 (Continued)  (c) Bilateral periorbital lichen pla-

nus pigmentosus in a child. (d) Reticular pattern of facial
pigmentation in lichen planus pigmentosus. (e) Diffuse
facial pigmentation following resolution of airborne
contact dermatitis due to parthenium. (a–e – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

Table 3.2  Differences between lichen planus pigmentosus

(LPP) and erythema dyschromicum perstans (EDP)

LPP EDP
Sex More common in Equal in both males
females and females
Color of Slate-gray to Ash-colored
lesion brownish-black
Site Mainly on sun- Mainly involving the
exposed areas trunk
Borders Discrete macules Polycyclic macules
with diffuse/ with elevated,
ill-defined borders erythematous
border like a piece
of string

Periorbital melanosis7 ●● Increased melanogenesis

●● This is also known as idiopathic cutaneous hyperchro- ●● Genetics
mia of the orbital region or dark circles. ●● Loose subcutaneous tissue
●● Periorbital melanosis is not an entity but rather a pre- ●● Increased vascularity
sentation of different etiologies including ●● Prominent tear-trough
●● Post-inf lammatory hyperpigmentation ●● Deep-brown hyperpigmentation is present around peri-
secondary to atopic dermatitis, seborrheic orbital skin and sometimes extends over to the upper
dermatitis nose and glabella (Figure 3.5a,b).
 Acquired facial melanosis  43

Figure 3.4  (a) A middle aged lady with maturational pigmentation – central face is involved and skin appears thickened.
(b) Maturational pigmentation in a middle-aged obese man. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 3.5  (a) Periorbital melanosis in a young female. (b) Periorbital melanosis in a lady. (a – Courtesy: Dr. Piyush Kumar,
Katihar, India; b – Courtesy: Dr. Vishwas Patel, Viksha skin clinic, Ahmedabad, India.)
44  Acquired facial melanosis

Figure 3.6  (a) Facial acanthosis nigricans – skin appears thickened and pebbly. (b) Facial acanthosis nigricans with thick-
ened, pigmented, pebbly skin. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● On stretching the skin, if pigmentation decreases it is ●● Stage II: progressive hyperpigmentation, pigmented
because of the vascular component; if it remains the colloid milium (caviar-like lesions), and atrophy
same it is because of increased melanogenesis. In many (Figure 3.7b)
cases, both components are present. ●● Stage III: papulonodular (sarcoid-like) lesions

Acanthosis nigricans Riehl’s melanosis

●● Facial acanthosis nigricans (AN) mainly involves the ●● It is also known as pigmented contact dermatitis.

forehead, temporal region, zygomatic region, and some- ●● It is a non-eczematous variant of contact dermatitis

times periocular and perioral regions (Figure 3.6a,b). that is clinically characterized by hyperpigmenta-
●● Facial AN is associated with AN of other parts of the tion with little or no signs of dermatitis. It is a type
body, especially neck and axilla, and is a good pointer four hypersensitivity reaction to low concentration
toward diagnosis. of allergens, not enough to cause spongiosis but can
●● Facial AN is associated with metabolic syndrome cause basement membrane damage and melanin
involving higher BMI, hyperinsulinemia, hypertension, incontinence.
and dyslipidemia. ●● Common chemicals implicated in pigmented contact

dermatitis include:
Exogenous ochronosis (EO) ●● Fragrances – benzyl salicylate, sandalwood oil,
●● The etiology is homogentisic acid accumulation due eugenol, cinnamic acid derivatives, and balsam of
to prolonged use of hydroquinone, resorcinol, phenol, Peru
picric acid, oral antimalarials, and/or mercury. ●● Pigments – aniline dyes and kumkum (a red powder
●● EO presents as asymptomatic, bilaterally symmetrical, commonly used by Hindu women)
speckled blue-black macules with caviar-like papules. The ●● Optical whiteners used in washing powder contain-
affected area is remarkable for interspersed depigmented ing Tinopal or CH3566
macules. Telangiectasias may be present (Figure 3.7a). ●● Coal tar derivatives, which increase photosensitivity
●● Three clinical stages are described by ●● Bactericidals – carbanilides such as trichlorocarba-
●● Stage I: erythema and mild hyperpigmentation nilide and Irgasan CF3
 Acquired facial melanosis  45

Figure 3.7  (a) Exogenous ochronosis with scattered

hypopigmented macules in a background of diffuse pig-
mentation. (b) Exogenous ochronosis with hyperpigmen-
tation and caviar like papules. (a – Courtesy: Dr. Piyush
Kumar, Katihar, India; b– Courtesy: Dr. Hiral Shah, Baroda
Medical College, Vadodara, India.)

Figure 3.8  (a) Riehl’s melanosis due to hair dye, present-

●● It presents as reddish-brown to slate-gray pigmentation ing as pigmented macules and patches on the upper
in a reticulate pattern (Figure 3.8a,b). face and ears. (b) Cosmetic-induced Riehl’s melanosis.
●● There is no active or preceding clinical dermatitis or (a – Courtesy: Dr. Zubin Mandlewala, Mumbai, India;
pruritus, thus making the clinical diagnosis difficult in b – Courtesy: Dr. Vishwas Patel, Viksha skin clinic,
many cases. Ahmedabad, India.)
46  Acquired facial melanosis

Figure 3.9  (a) Nevus of Ota as unilateral bluish-pigmented

macules and patches on the mid face. Note involvement
of ipsilateral sclera. (b) Localized nevus of Ota affect-
ing the right orbital area and eyelids. (c) Bilateral nevus
of Ota. (a – Courtesy: Dr. Hiral Shah, Baroda Medical
College, Vadodara, India; b – Courtesy: Dr. Dependra
Kumar Timshina, Remedy Clinics, Siliguri, India; c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● The face is the most common site affected; however,

lips, axillary borders, and thighs may be affected due to
lipstick color, shirt dye, and trouser dyes respectively.

Nevus of OTA (nevus fusco caeruleus

ophthalmo-maxillaris)
●● Nevus of Ota is nine times more common in women

than in men and presents more commonly around the

perinatal period (50%) and puberty (30%).
●● It is characterized by speckled blue-gray pigmentation of

the area supplied by ophthalmic and maxillary divisions

of trigeminal nerve, which may also involve oral mucosa
and conjunctiva, sclera, retrobulbar fat, and cornea and
retina in the eye. In the eye, conjunctiva is stained brown
and the sclera is stained blue (Figure 3.9a,b).
●● It is unilateral in 90% of cases but may be bilateral too

(Figure 3.9c).
●● Based on the extent, it is classified into

●● Type I (mild):
– IA: Mild orbital type: on upper and lower eye-
lids, periocular and temple region
 Acquired facial melanosis  47

Figure 3.10  (a) Hori nevus as bilateral brown-blue macules and patches near the lateral canthus and on adjacent skin of
temple region. (b) Hori nevus seen as brown-blue macules and patches. (a – Courtesy: Dr. Zubin Mandlewala, Mumbai,
India; b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

– IB: Mild zygomatic type: on the infra palpebral Pigmentary demarcation lines (PDLs)8
fold, nasolabial fold and zygomatic region ●● PDLs, also known as Futcher’s or Voight’s lines, are
– IC: Mild forehead type: on forehead only physiological, abrupt transitions from deeper pigmented
– ID: on ala nasi only skin to lighter pigmented skin. It becomes more promi-
●● Type II (moderate): on upper and lower eyelids, nent after puberty, and that is the reason most patients
periocular, zygomatic, cheek, and temple with PDL presents after puberty. There is a familial
regions tendency for the development of PDL.
●● Type III (severe): on scalp, forehead, eyebrow, and ●● PDLs, labeled A-H, have been described. Briefly, they
nose appear as follows:
●● Type IV (bilateral type): Bilateral ●● PDL type A – On the lateral aspect of the upper arm
extending over the pectoral area
Hori nevus/acquired bilateral nevus of Ota-like ●● PDL type B – On the posteromedial portion of the
macules (ABNOM) lower limb
●● It is an acquired variant. ●● PDL type C – On the medio-sternal line, a vertical
●● It is characterized by bilaterally symmetrical speckled hypopigmented line in the pre and parasternal area
or confluent brownish-blue pigmentation over malar ●● PDL type D – On the posteromedial area of the spine
region, temples, root of nose, ala nasi, eyelids, and fore- ●● PDL type E – As bilateral hypopigmented streaks,
head (Figure 3.10a,b). bands, or lanceolate areas over the chest in the
●● It spares the mucosa and presents in late adulthood. zone between the mid third of the clavicle and the
periareolar skin
Freckles (ephelides) and lentigines ●● PDL type F – As V-shaped hyperpigmented lines
●● Freckles and lentigines present as pigmented macules between the malar prominence and the temple
(Table 3.3) (Figure 3.11a,b). (Figure 3.12a,b)
48  Acquired facial melanosis

Table 3.3  Freckles and lentigines

Differentiating
features Freckles Lentigines
Color Light brown Dark-brown to
black
Borders Less defined, Well-defined,
Irregular regular
Genetic MC1R gene Not familial*
mutation
Syndrome Not present Centrofacial
association lentiginosis,
Carvajal
syndrome, and
Naxos syndrome
are associated
Sites Present over Present over
sun-exposed sun-exposed as
areas well as sun-
protected areas.
Mucosa Usually not Frequently affected
affected
Sun exposure Increases with Increases with sun
sun exposure exposure but
and disappears doesn’t
when sun disappear when
exposure is there is no sun
removed exposure.
Seasonal Increases in No seasonal
variation summer season variation
Histopathology Increase in Linear increase in
melanosome melanocyte
concentration, number present.
melanocyte
number
remains the
same.
* There are syndromes (e.g., LEOPARD syndrome) associated with
lentigines, which may be familial.

●● PDL type G – As W-shaped hyperpigmented lines Figure 3.11  (a) Light-brown, ill-defined macules of freck-
between the malar prominence and the temple les. (b) Dark-brown to black, well-demarcated macules
●● PDL type H –As linear bands of hyperpigmentation in lentigines. Note involvement of lip. (a – Courtesy: Dr.
from the angle of the mouth to the lateral aspects of Divya Sachdev, Raipur, India; b – Courtesy: Dr. Piyush
the chin (Figure 3.12c) Kumar, Katihar, India.)

Erythromelanosis follicularis faciei et colli (EFF)

●● EFF is seen in young/middle-aged men. distinguished by the extension onto the neck and the
● It is an unusual condition characterized by the presence of pigmentation.
triad of hyperpigmentation, follicular plug-
ging, and well-defined erythema of the face and Erythrose péribuccale pigmentaire de Brocq
neck (Figure 3.13). (peribuccal pigmentation of Brocq)
●● It has been considered as a variant of keratosis pilaris ●● This predominantly affects middle-aged women. It is

– in view of the follicular papules, blotchy erythema, a type of pigmented contact dermatitis affecting the
and associated keratosis pilaris, although EFF is perioral area.
 Acquired facial melanosis  49

   

Figure 3.12  (a) Bilateral pigmentary demarcation line

type F on the face. (b) Well-demarcated, brownish,
V-shaped patch of pigmentary demarcation line type F.
(c) Pigmentary demarcation line type H. (a – Courtesy:
Dr. Pooja Manwar, Mahatma Gandhi Institute of Medical
Sciences, Sevagram, India; b – Courtesy: Dr. PC Das,
Katihar, India; c – Courtesy: Dr. Vishwas Patel, Viksha skin
clinic, Ahmedabad, India.)

●● The etiology is that it is due to photodynamic substance

in cosmetics.
●● Diffuse brownish-red pigmentation with ery-
thema develops symmetrically around the mouth
(Figure 3.14a).
●● It spares a narrow perioral rim.
●● DD: Post-inflammatory hyperpigmentation due to lip
licking dermatitis (Figure 3.14b).

Seborrheic melanosis9
●● This term is commonly used among Indian dermatolo-

gists for a particular pattern of pigmentation affecting

certain seborrheic areas of the face – the alar grooves,
angles of the mouth and labio-mental crease.
●● Such a pattern of pigmentation is known among

darker people from Asia and Africa and in Hispanic

populations.
50  Acquired facial melanosis

Figure 3.13  Erythromelanosis follicularis faciei pre-

senting as erythema, pigmentation, and tiny pap-
ules on the lateral face. (Courtesy: Dr. Piyush Kumar,
Katihar, India.)

●● Patients may have a prior history of erythema, which

frequently goes unnoticed in skin of color. Patients
usually present with hyperpigmentation of the alar
grooves, angles of the mouth, and labio-mental crease.
It may be accompanied by scaling and seborrhea
(Figure 3.15a–c).
●● The nosology of this entity is debatable and many
authors have stated that acanthosis nigricans and post-
inflammatory hyperpigmentation are responsible for
Figure 3.14  (a) Peribuccal pigmentation of Brocq.
the clinical presentation. (b) Perioral pigmentation due to lip licking dermati-
tis. (a – Courtesy: Dr. Vishwas Patel, Viksha skin clinic,
Poikiloderma of Civatte
Ahmedabad, India; b – Courtesy: Dr. Piyush Kumar,
●● Long-term exposure to UV light, genetic predisposition,
Katihar, India.)
certain cosmetic ingredients, hormonal influences, and
age contribute to its causation.
●● Reddish-brown reticulate pigmentation with atrophy ●● Patients present with hyperpigmented macules mainly on
and telangiectasia are present in symmetrical fashion the nose (chik sign). It can also appear similar to melasma
over the sides of cheeks and neck (Figure 3.16). or periorbital melanosis or with flagellate patterns on the
●● Submental and submandibular areas that are sun pro- trunk, extremities, abdomen, and palms (Figure 3.17).
tected are characteristically spared.
Addison’s disease
Post-Chikungunya pigmentation ●● Tuberculosis is the predominant cause of Addison’s
●● This is seen in patients with Chikungunya infection disease in developing countries; however, nowadays
from Aedes mosquitoes. autoimmune pathology is gaining attention.
 Acquired facial melanosis  51

Figure 3.16  Poikiloderma of Civatte affecting the neck

in a middle-aged lady. (Courtesy: Dr. Ganesh Avhad,
Mumbai, India.)

Figure 3.17  Grayish macular pigmentation of the nose

and cheeks following Chikungunya. (Courtesy: Dr. Sushil S
Savant, Consultant Dermatologist, Mumbai, India.)

●● Increased ACTH or B-lipotropin simulates MSH, which

results in melanocyte production.
●● Cutaneous features of Addison’s disease appear before
other clinical features appear.
●● There is generalized hyperpigmentation of sun-exposed
and trauma-prone areas, along with oral mucosal pig-
mentation (Figure 3.18a,b).
●● There is hyperpigmentation of palmar creases, genitals,
nipples, and existing scars.
●● There is development of new pigmented nevi, and older
nevi darken.
●● Other clinical features are fatigue, abdominal pain, hypo-
tension, nausea, vomiting, diarrhea, and craving for salt.
Vitamin B12 deficiency10
●● The pigmentation in vitamin B deficiency mimics that
12

Figure 3.15  (a, b) Hyperpigmentation in alar groove and of Addison’s disease.

●● Inadequate intake, low consumption of animal-
angle of mouth in seborrheic melanosis. (c) Seborrheic
melanosis in a young lady. (a–c – Courtesy: Dr. Piyush source foods and pernicious anemia due to deficient
Kumar, Katihar, India.) intrinsic factor are major causes in younger adults.
52  Acquired facial melanosis

Malabsorption, in part due to gastric atrophy, is a major

contributing factor in elderly persons.
●● Mucocutaneous features include diffuse and patchy
hyperpigmentation, graying of hairs, and mucosal
changes (mucosal pigmentation, atrophic glossitis and
angular stomatitis).
●● Hyper pigmentation of friction- and pressure-prone
areas, such as the dorsum of the hands and feet, with
accentuation over the interphalangeal joints and ter-
minal phalanges is a classical feature of vitamin B12
deficiency (Figure 3.19a,b).
●● Systemic features include fatigue, weight loss, and
anorexia.

Figure 3.18  (a) Diffuse brownish pigmentation of the

face in Addison’s disease. (b) Same patient with pig- Figure 3.19  (a) Facial pigmentation in vitamin B12 defi-
mented palmar creases. (a,b – Courtesy: Dr. Piyush ciency. (b) Same patient with pigmentation of both palms.
Kumar, Katihar, India.) (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Acquired facial melanosis  53

Figure 3.20  Clofazimine induced patchy pigmenta-

tion of the face and ear. (Courtesy: Dr. Piyush Kumar,
Katihar, India.)

Figure 3.21  Dark-brown pigmentation over the forehead

in frictional melanosis. Also note seborrheic melanosis in
Drug induced pigmentation the alar groove and mento labial crease. (Courtesy:
Dr. Piyush Kumar, Katihar, India.)
●● Various patho-mechanisms involved are accumulation

of melanin, accumulation of the drug itself, nonspecific

inflammation, deposits of iron following trauma to ves- ●● The pigmentation typically affects the skin over bony
sels, aggravation by sun exposure, formation of com- prominences, sparing the mid forehead and nose
plexes between melanin and causative drug, and drug (Figure 3.21).
particles engulfed by dermal macrophage. ●● The pigmentation is usually symmetric, uniform, deep
●● Drugs implicated are nonsteroidal anti-inflammatory
dark brown. The affected skin usually does not show
drugs, antimalarials, amiodarone, tetracyclines, textural changes, but that may be present in longstand-
psychotropic drugs, clofazimine (Figure 3.20), heavy ing cases.
metals, etc. ●● Stopping the acts of friction may hasten the resolution
●● Drug-induced pigmentation can be over photo-exposed
of the condition.
areas, mucosae, nails, or even Addisonian-like. ●● DD: melasma, facial macular amyloidosis, facial
acanthosis nigricans, lichen planus pigmentosus,
Macular amyloidosis11 pigmented contact dermatitis, post-inflammatory
●● It commonly affects the upper back and interscapular
hyperpigmentation.
area but may occasionally affect the forehead.
●● It presents as small dusky-brown to gray pigmented Post-inflammatory hyperpigmentation (PIH)
macules associated with pruritus. It may be associated ●● It is a common sequelae of inflammatory derma-
with severe itching. These macules may later coalesce tosis (such as acne vulgaris, impetigo and atopic
into patches. dermatitis). It may be epidermal, dermal, or mixed,
●● DD: frictional melanosis, post-inflammatory hyperpig- depending on the trauma or insult to skin. When
mentation, lichen planus pigmentosus. inflammation causes damage to the basement mem-
brane, it results in melanin incontinence and PIH,
Frictional melanosis which is dermal.
●● In a tropical country like India, people sweat a lot and ●● It tends to affect Fitzpatrick skin types IV–VI.

use handkerchiefs or other material to wipe away the ●● It presents as tan, brown, or dark-brown macules on

sweat. The repeated acts of friction result in pigmenta- any part of the face along with a history of a preceding
tion of the face. dermatosis in the same region (Figure 3.22a–c).
54  Acquired facial melanosis

  

Figure 3.22  (a) Post-inflammatory hyperpigmentation fol-

lowing acne vulgaris. (b) Linear post-inflammatory hyper-
pigmentation following trauma. (c) Post-inflammatory
hyperpigmentation over lips and around eyes following fixed
drug eruption. (a – Courtesy: Dr. Piyush Kumar, Katihar, India;
b – Courtesy: Dr. PC Das, Katihar, India; c – Courtesy:
Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)

Authors’ note
1. Various entities have been described purely on
the basis of clinical and epidemiological data.
Pathological and ultrastructural studies are needed
to confirm or refute the independent existence of
such diseases. We have not tried to clarify or settle
this issue but have included them in this chapter.
2. In our practice, very often a patient with facial
pigmentation have been found to have more than
one co-existing pigmentary disorder. For exam-
ple, a patient of melasma may have concomitant
post-inflammatory hyperpigmentation from
the topical medications they have used in past
and from excessive cleaning (frictional). At the
same time, they may have metabolic syndrome
and facial acanthosis nigricans. It is common for
patients having PDL to develop melasma, and
while melasma improves with treatment, PDL
generally does not, which may be considered as
 Acquired facial melanosis  55

treatment failure. Hence, we encourage our read- 6. Sonthalia S, Sarkar R, Neema S. Maturational
ers to examine the complete face thoroughly and hyperpigmentation: Clinico-dermoscopic and histo-
make multiple diagnoses, if needed, as one diag- pathological profile of a new cutaneous marker of
nosis does not exclude the possibility of other metabolic syndrome. Pigment Int 2018;5:54–56.
co-existing conditions. 7. Sarkar R, Das A. Periorbital hyperpigmentation:
What lies beneath? Indian Dermatol Online J
REFERENCES 2018;9:229–230.
8. Singh N, Thappa DM. Pigmentary demarcation lines.
1. Vashi NA, Kundu RV. Facial hyperpigmenta- Pigment Int 2014;1:13–16.
tion: Causes and treatment. Br J Dermatol 9. Verma SB, Vasani RJ, Chandrashekar L, Thomas M.
2013;169(3):41–56. Seborrheic melanosis: An entity worthy of men-
2. Khanna N, Rasool S. Facial melanoses: Indian tion in dermatological literature. Indian J Dermatol
perspective. Indian J Dermatol Venereol Leprol Venereol Leprol 2017;83:285–289.
2011;77:552–564. 10. Sachdev D, Kumar P, Debbarman P. A Young Man
3. Nicolaidou E, Antoniou C, Katsambas AD. Origin, with Generalized Pigmentation. In: Kothiwala S,
clinical presentation, and diagnosis of facial hyper- Tiwary AK, Kumar P, editors. Clinical Cases in
melanoses. Dermatol Clin 2007;25(3):321–326. Disorders of Melanocytes. 1st edition. Cham:
4. Vashi NA, Wirya SA, Inyang M, Kundu RV. Facial Springer; 2020. P. 103–110.
hyperpigmentation in skin of color: Special consid- 11. Bandhlish A, Aggarwal A, Koranne RV. A
erations and treatment. Am J Clin Dermatol 2017 clinico-epidemiological study of macular amy-
Apr;18(2):215–230. loidosis from north India. Indian J Dermatol
5. Pérez-Bernal A, Muñoz-Pérez MA, Camacho F. 2012;57(4):269–274.
Management of facial hyperpigmentation. Am J Clin
Dermatol 2000;1(5):261–268.
 E3
Hyperpigmented macules and patches:
Generalized

PC DAS, TALAT FATIMA, NIHARIKA RANJAN LAL

ABSTRACT
Widespread hyperpigmentation is usually due to increased melanin production or deposition of drugs or heavy metals within
the dermis. Generalized hyperpigmentation is seen in a variety of conditions, many of which are associated with other sys-
temic disorders. This chapter discusses a morphological approach that, in conjunction with the clinical history and necessary
laboratory tests, allows a definitive diagnosis to be reached.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

56 DOI: 10.1201/9781351054225-8
 E4
Diffuse hyperpigmentation

RAMPAL SHARMA

ABSTRACT
Increased pigmentation may present in a patchy manner or may cause extensive involvement of a part of or the whole body.
A variety of causes, including genetic, metabolic, or inflammatory factors; ultraviolet radiation; drugs; and malignancy, may
cause diffuse pigmentation of the skin. This chapter discusses conditions presenting with diffuse pigmentation.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-9 57
 4
Reticulate and mottled pigmentation

SAUMYA PANDA, RASHID SHAHID

INTRODUCTION ●● The disease starts occasionally with erosion at birth,

followed by prominent traumatic or spontaneous
There are many congenital and acquired pigmentary disor- blistering over hands and feet during infancy, which
ders that may present with hyperpigmented macules and/or heal without milia formation or scarring. During
hypopigmented macules on normal or diffusely hyperpig- childhood, the skin fragility and photosensitivity
mented skin with various patterns of distribution. Broadly, improves significantly; however, reticulated
these have been categorized into two groups: reticulate and erythematous hypopigmentation within mottled
mottled. Their most widely accepted definitions are given hyperpigmentation and telangiectasia develops in sun-
in Box 4.1. The clinical diagnosis rests on correct identi- exposed areas and later spreads to non-exposed areas
fication of the pattern of pigmentation. The clinical differ- such as chest, abdomen extensor aspects of arms and
entials of reticulate and mottled pigmentation are listed in thighs, and occasionally flexors in adulthood.
Table 4.1. Detailed clinical, personal, and family histories, ●● Diffuse poikiloderma appears gradually, becomes more
including any exposure to drugs or chemicals, along with prominent later in life, and persists throughout life.
comprehensive dermatological and general physical exami- Tissue-paper–like atrophy is present in sun-exposed
nations remain crucial diagnostic tools. Clinical clues such areas, particularly on the dorsum of hands, feet, knees,
as age of onset – early (infantile and/or childhood) and late and elbows (Figure 4.3a,b).
(adolescence and/or adulthood) – distribution of pigmenta- ●● Other features include erosive gingivitis, dental caries,
tion (face, acral, flexures and trunk/generalized) and associ- ectropion, intraoral and corneal scarring, esophageal
ated cutaneous as well as systemic findings help in making stricture, rectal and urethral bleeding, palmoplantar
a clinical diagnosis. The clinical approach to the diagnosis hyperkeratosis, and mild webbing of digits.
of reticulate and mottled pigmentary disorders is summa- ●● There is an increased risk of squamous cell carcinoma
rized in Figures 4.1a,b and 4.2a,b, and salient features of (SCC) of lip, oral mucosa, and acral skin.
different entities are discussed below.1–8 This chapter focuses ●● DD: Rothmud-Thompson syndrome (poikiloderma
on diseases that typically present with reticulate or mottled associated with sparse hair, short stature, cataract,
pigmentation. Some other pigmentary disorders, such as hypogonadism), Bloom syndrome (telangiectasia,
lichen planus pigmentosus, may sometimes present with photosensitivity, erythema of the face and sun
reticular pigmentation and are discussed elsewhere. exposed areas but not true poikiloderma, xeroderma
pigmentosum (sparing of acral areas).  
RETICULATE HYPERPIGMENTATION
X-linked reticulate pigmentary disorder
Kindler syndrome ●● This is an extremely rare X-lined recessive

●● Synonyms are Kindler–Weary syndrome, bullous genodermatosis caused by mutation in POLA1.

acrokeratotic poikiloderma of Kindler and Weary, ●● Female carriers have clinical manifestations limited to

congenital bullous poikiloderma, and hereditary skin.

acrokeratotic poikiloderma. ●● At birth or within a few weeks of life, female infants

●● It is a rare autosomal recessive disorder, resulting from develop reticulate hyperpigmentation along the
mutations in the FERMT1 gene. lines of Blaschko. Mild onychodystrophy may be
●● The syndrome is characterized by congenital acral skin reported sometimes but only in correspondence
blistering, skin fragility, photosensitivity, progressive to pigmentary lesions. During adulthood,
poikiloderma, and cutaneous atrophy. hypomelanosis may fade away.

58 DOI: 10.1201/9781351054225-10
 Reticulate and mottled pigmentation  59

BOX 4.1: Definitions of terminology used

●● Mottled pigmentation: may have one of the following two presentations

●● Mixture of small hyperpigmented and hypopigmented macules of variable sizes and shapes but with distinct

border, on normal looking skin. It is also called “dyschromia.”

●● Mottled pigmentation with guttate hypopigmented macules superimposed on generalized/diffuse

hyperpigmentation.
●● Reticulate hyperpigmentation: Presence of variably pigmented freckle-like hyperpigmented macules with indistinct
border on normal-looking skin.

●● Between fourth and fifth years of age, male patients photophobia, visual impairment, gastroesophageal
may develop asymptomatic, generalized, reticulate reflux and intermittent constipation, and kidney stones
hyperpigmentation, with initial involvement of inner associated with hypercalciuria.
thighs, buttocks, and cheeks. ●● Additional features include dental anomalies,
●● During the first few months of life, systemic hypohidrosis, coarse hair, arched eyebrows, dysmorphic
manifestations include recurrent pneumonia and face with upswept frontal hairline, xerosis, delayed bone
chronic obstructive pulmonary disease. Other systemic age, short metacarpals.
manifestations include low birth weight, neonatal ●● DD: In males – dyskeratosis congenita. In females –
colitis, developmental delay, seizures, hemiplegia, severe stage III incontinentia pigmenti.

Table 4.1  Causes of reticulate and mottled pigmentations

B1. Mottled pigmentation B2. Mottled pigmentation with

A. Hyperpigmented macules on normal with both hypo-and Hypopigmented macules superimposed
skin in reticular fashion hyperpigmented macules on generalized/diffuse hyperpigmentation
• Kindler syndrome • Dyschromatosis • Epidermolysis bullosa simplex with
• Incontinentia pigmenti (3rd stage) symmetrica hereditaria mottled pigmentation
• Reticulate acropigmentation of Kitamura (DSH) / (Reticulate • Dyskeratosis congenita and its variants:
(RAPK) acropigmentation of Dohi • Revesz syndrome
• Dermatopathia pigmentosa reticularis • Dyschromatosis • Hoyeraal-Hreidarsson (HH) syndrome
• Naegeli–Franceschetti–Jadassohn universalis hereditaria • Rothmund Thomson syndrome (RTS)
syndrome (NFJS) (DUH) • Bloom syndrome
• Fanconi anemia • Xeroderma pigmentosum • Chediak-Higashi syndrome (CHS)
a. Progeria/acrogeria syndromes (XP) • Griscelli syndrome
Hutchinson-Gilford syndrome (classic • Amyloidosis cutis • Ziprowski-Margolis syndrome
progeria) dyschromica • Wende-Baukus (Pegum) syndrome
b. Werner syndrome (adult progeria) • Porphyria cutanea tarda • Berlin syndrome
c. Nestor-Guillermo progeria syndrome • Progeria and acrogeria
d. Acrogeria • Anonychia with bizarre flexural
• Prurigo pigmentosa pigmentation
• Erythema ab igne • Chronic arsenicism
• Riehl’s melanosis • Mycosis fungoides
• Lichen planus pigmentosus (LPP) • Systemic sclerosis
• Erythema dyschromicum perstans (EDP) • Secondary syphilis with necklace of
• Atopic dirty neck Venus
• Pigmentation reticularis faciei et colli • Pinta
• Confluent and reticulated papillomatosis • Photoleukomelanodermatitis of Kobori
of Gougerot and Carteaud (CRP) (induced by thiazide, afloqualone,
• Dowling Degos disease (DDD) tetracycline)
• Galli-Galli disease • Acromelanosis albo-punctata
• Haber’s syndrome • Acquired brachial cutaneous
• Post-inflammatory hyperpigmentation dyschromatosis (ABCD)
• Drug induced • Photoaging
• X-linked reticulate pigmentary disorder • Vagabond’s leukomelanoderma
60  Reticulate and mottled pigmentation

Naegeli-Franceschetti-Jadassohn syndrome (NFJ) ●● Other features include hypohidrosis, lifelong poor heat
●● This is a rare, autosomal dominant form of ectodermal intolerance, absent or hypoplastic dermatoglyphics,
dysplasia resulting from heterozygous mutations in the non-cicatricial alopecia of scalp, eyebrows, diffuse
keratin 14 gene. punctate palmoplantar keratoderma, and dental
●● Brown or gray-brown reticulated pigmentation anomalies.
develops by the age of two years, without ●● There may be nail changes including onychodystrophy,
preceding inf lammatory changes, and often fade in onycholysis, subungual hyperkeratosis, and congenital
puberty. malalignment of the great toenails.
●● Sites involved are abdomen, periocular and perioral ●● DD: Dermatopathia pigmentosa reticularis (lifelong
regions, with variable involvement of neck, extremities, persistence of hyperpigmentation, partial alopecia,
trunk, axilla, and groin. absence of dental anomalies), dyskeratosis congenita
●● Occasionally some patients develop blistering on their (X-linked recessive, other features such as alopecia,
palms and soles. mucosal leukoplakia, poikiloderma and blood

Reticulate and mottled pigmentation

disorders with childhood onset

Hyperpigmented macules on Mottled pigmentation (hypo- and

normal skin in reticular hyperpigmented macules, or
fashion hypopigmented macules on
generalized hyperpigmentation)

Kindler syndrome Epidermolysis bullosa simplex

with mottled pigmentation
Incontinenti pigmenti (3rd
stage) Dyschromatosis symmetrica
hereditaria (Reticulate
Reticulate acropigmentation acropigmentation of Dohi)
of Kitamura Early onset (during
infancy and childhood) Dyschromatosis universalis
Naegeli–Franceschetti– hereditaria
Jadassohn syndrome
Xeroderma pigmentosum
Dermatopathia
pigmentosareticularis Porphyria cutanea tarda

Fanconi anemia Rothmund Thomson syndrome

Haber’s syndrome Bloom syndrome

X-linked reticulate Chediak Higashi syndrome

pigmentary disorder
Griscelli syndrome
Progeria/acrogeria
syndromes Amyloidosis cutis dyschromica

a. Hutchinson-Gilford Anonychia with bizarre flexural

progeria syndrome mottled pigmentation
b. Nester-Guillermo progeria
syndrome Acromelanosisalbo-punctata
c. Acrogeria
Berlin syndrome

Progeria

Dyskeratosiscongenita and its

variants:

Revesz syndrome

Hoyeraal-Hreidarsson
(HH) syndrome
(a)

Figure 4.1  (a) Disorders causing reticulate and mottled pigmentation- early onset. (Continued)
 Reticulate and mottled pigmentation  61

Reticulate and mottled pigmentation

disorders of late onset

Mottled pigmentation (hypo- and

Hyperpigmented macules on
hyperpigmented macules, or
normal skin in reticular
hypopigmented macules on
fashion
generalized hyperpigmentation)

Prurigo pigmentosa Secondary syphilis with

necklace of venus
Atopic dirty neck
Pinta
Erythema abigne
Systemic sclerosis
Riehl’s melanosis
Late onset Photoleukomelanodermatitis
Lichen planus pigmentosus of Kobori (induced by
(adolescent or
adult) thiazide, afloquanone,
Erythema dyschromicum
tetracycline)
perstans
Acquired brachial cutaneous
Confluent and reticulate
dyschromatosis
papillomatosis of Gougerot and
Carteaud Chronic arsenicism
Werner syndrome Werner syndrome
Dowling Degos disease Mycosis fungoides
Galli-Galli disease Photoaging
Pigmentation reticularis faciei Vagabond’s
et colli
leukomelanoderma
Postinflammatory Acquired brachial
hyperpigmentation cutaneous dyschromatosis
Drug induced (b)

Figure 4.1 (Continued)  (b) Disorders causing reticulate and mottled pigmentation- late onset.

dyscrasias), epidermolysis bullosa simplex (blistering ●● There is normal sweating and teeth. However, oral
is more prominent), X-linked reticulate pigmentary leukoplakia may be present.
disorder, Kindler syndrome (poikiloderma present).  ●● Palmoplantar hyperkeratosis may accompany the
disease and usually gets worse with age.
Hidrotic ectodermal dysplasia ●● Hyperpigmentation present over bony prominences,
●● This is also called Clouston syndrome. joints, axillae, areola, and pubic area. Papules coalescing
●● It is an autosomal dominant condition caused by to form a cobblestone-like pattern may extend from
mutation in the GjB6 gene. palms and soles onto the dorsal surface of the digits
●● Nail abnormalities are the most consistent feature and distally.
frequently manifest at birth or in early infancy. Nails ●● DD: Pachyonychia congenita (hypertrophic nail
are typically milky white and smaller, with gradual dystrophy, painful palmoplantar keratoderma
thickening throughout childhood. and blistering, palmoplantar hyperhidrosis, and
●● Slow growth of nail plates, thickening, and spontaneous follicular keratoses on the trunk and extremities, oral
separation of the nail plate are seen in adults. leukokeratosis, pilosebaceous cysts), hypotrichosis-
●● Scalp hairs are slow growing, wiry, pale, fine, and deafness syndrome.
brittle, with patchy alopecia and reduced hair
strength. Eyebrows and eyelashes are frequently Dermatopathia pigmentosa reticularis (DPR)
sparse and axillary, pubic, and body hairs too may be ●● This is an autosomal dominant disorder due to mutation

affected. in the keratin 14 gene.

●● Sparse eyelashes may predispose to development of ●● It usually manifests by the age of two years.

conjunctivitis, blepharitis, cataract, photophobia, and ●● Reticulated confetti-like hyperpigmented macules

strabismus. develop initially at birth or during infancy before two

62  Reticulate and mottled pigmentation

years of age, involving trunk and proximal extremities, ●● Nail changes include onychodystrophy, loss of nail, and
and persist throughout life. pterygium formation.
●● Further widespread reticulated pigmentation begins in ●● Other variable features include hypo- or hyperhidrosis,
childhood, along with non-scarring alopecia of scalp, punctate palmoplantar keratoderma, and
eyebrows and axillae, and onychodystrophy. adermatoglyphia.
●● Sparse hair of scalp, eyebrows, and eyelashes with ●● DD: Naegeli-Franceschetti-Jadassohn syndrome (lacks
progressive and diffuse alopecia develops. alopecia, less persistent hyperpigmentation – which fades

FLEXURAL INVOLVEMENT FACIAL INVOLVEMENT

Lichen planus pigmentosus Lichen planus pigmentosus

Confluent and reticulate papillomatosis of Riehl’s melanosis

Gougerot and Carteaud
Postinflammatory
Dowling Degos disease hyperpigmentation

Galli-Galli disease Pigmentation reticularis faciei et

colli
Haber’s syndrome
Naegeli–Franceschetti–
Erythema dyschromicum perstans Jadassohn syndrome

Atopic dirty neck Progeria

Naegeli-Franceschetti-Jadassohn syndrome Drug induced

X-linked reticulate pigmentary disorder

ACRAL INVOLVEMENT TRUNCAL/LIMB INVOLVEMENT

Acroreticulate pigmentation of Kindler syndrome

Kitamura
Prurigo pigmentosa
Kindler syndrome
Naegeli-Franceschetti-Jadassohn syndrome
Progeria
Dermatopathia pigmentosa reticularis
Acrogeria
Haber syndrome

Erythema dyschromicum perstans

Fanconi’s anemia

Incontinenti pigmenti (3rd stage)

Erythema ab igne

(a) Progeria

Figure 4.2  (a) Regional distribution of disorders with reticulate pigmentation. (Continued)
 Reticulate and mottled pigmentation  63

FACIAL INVOLVEMENT

FLEXURAL INVOLVEMENT Porphyria cutanea tarda

Chronic arsenicism Xeroderma pigmentosum

Anonychia with bizarre flexural pigmentation Rothmund Thomson syndrome

Wende-Bauckus (Pegum) syndrome Dyskeratosis congenita

Berlin syndrome Hoyeraal-Hreidarsson (HH) syndrome

Systemic sclerosis

Progeria

Photoleukomelanodermatitis of Kobori

Photoaging

TRUNCAL INVOLVEMENT

Mycosis fungoides
ACRAL/ EXTREMITIES INVOLVEMENT
Systemic sclerosis
Porphyria cutanea tarda
Amyloidosis cutis dyschromica
Dyschromatosis symmetrica hereditaria
Progeria
Progeria and acrogeria
Epidermolysis bullosa simplex with mottled
Acromelanosis albo-punctata
pigmentation
Acquired brachial cutaneous dyschromatosis Dyschromatosis universalis hereditaria
(extensor forearms)
Dyskeratosis congenita (including Revesz
syndrome and Hoyeraal-Hreidarsson
syndrome)

Secondary syphilis with necklace of venus

Pinta

Chronic arsenism

Epidermolysis bullosa simplex with mottled

pigmentation

(b) Vagabond’s leukomelanoderma

Figure 4.2 (Continued)  (b) Regional distribution of disorders presenting with mottled pigmentation.

after puberty – and dental anomalies), dyskeratosis ●● It is caused by DKC1 gene mutation, and the male-to-
congenita (X-linked recessive, alopecia, mucosal female ratio is 3:1.
leukoplakia, poikiloderma, and blood dyscrasias ●● The condition is characterized by progressive bone
present). marrow failure and clinically by triad of reticulated
hyperpigmentation, nail dystrophy, and leukoplakia.
Dyskeratosis congenita (DKC) ●● During the first decade of life, a lacy reticulated
●● DKC is also known as Zinsser-Engman-Cole syndrome. pattern of hyperpigmentation develops, primarily on
●● The most common pattern of inheritance is X-linked the neck, upper arms, and upper chest. Occasionally
recessive. However, a few patients may have autosomal hypopigmented macules are also present.
dominant and autosomal recessive forms, which have ●● Nail changes – such as longitudinal ridging, splitting
fewer abnormalities and later onset of symptoms. followed by pterygium formation, flaking or poor
64  Reticulate and mottled pigmentation

●● Teeth malformation, extensive caries, decreased root/

crown ratio, periodontal disease, and missing teeth are
also seen.
●● Epiphora due to atresia of lacrimal ductal is common.
Blepharitis, ectropion, entropion, and exudative
retinopathy may be found.
●● Poikiloderma is seen in some patients. Additional
features include premature gray hair or hair loss,
palmoplantar hyperhidrosis and hyperkeratosis,
adermatoglyphia, short stature, acrocyanosis, frictional
bullae, wrinkling of extremities, and small genitalia.
●● Bone marrow failure is present in most patients during
the third or fourth decade, presenting with anemia,
thrombocytopenia, or pancytopenia. Pancytopenia
may result in pallor, bruising, petechiae, and frequent
infections.
●● Risk of malignancy, such as squamous cell carcinoma
of mouth, anus, vagina, cervix, esophagus, and skin,
increases in the fourth or fifth decade. Additionally,
there is increased risk of acute myeloid leukemia,
myelodysplastic syndrome, and gastrointestinal
carcinomas.
●● Other systemic manifestations include pulmonary
fibrosis, esophageal stenosis, liver cirrhosis, enteropathy,
atrial or ventricular septal defect, fibrosis, dilated
cardiomyopathy, urethral stenosis, cryptorchidism,
male hypogonadism, osteoporosis, avascular necrosis
of the hips and shoulder, short stature, developmental
delay, microcephaly, and immunodeficiency.
●● DD: Fanconi anemia (pigmentation is more generalized,
less often reticulated), Naegeli–Franceschetti–Jadassohn
(NFJ) syndrome, and dermatopathia pigmentosa
reticularis (DPR) (both NFJ and DPR lack leukoplakia
and bone marrow involvement).

Incontinentia pigmenti
●● It is X-linked dominant genodermatosis and is

embryonically lethal for males.

●● Within the first few weeks of life stages 1 and 2 are

characterized by erythema, blisters, and verrucous

lesions along Blaschko lines.
●● Linear reticulate hyperpigmentation (stage 3) develops

during infancy, persists during childhood, and fades

during adolescence.
●● Atrophic hypopigmentation (stage 4) develops during

adolescence and persists indefinitely.

●● Associated findings include scarring alopecia, nail
Figure 4.3  (a) Poikiloderma on the face and neck in a changes, ophthalmological abnormalities, dental
girl with Kindler syndrome. (b) Atrophic scarring on the abnormalities, and neurological abnormalities.
acral areas in the same patient. (a,b – Courtesy: Dr. Piyush
Kumar, Katihar, India.) Fanconi anemia
●● Primary clinical features include physical features,

growth, and occasionally complete loss of nails – appear progressive bone marrow failure, and cancer
during early childhood. susceptibility. However, some individuals have neither
●● In the majority of patients, premalignant physical abnormalities nor bone marrow failure.
leukoplakia develops mostly on the lateral portion ●● Prenatal and/or postnatal short stature is a striking

of the tongue. finding.

 Reticulate and mottled pigmentation  65

●● It involves abnormal skin pigmentation (40%), stature, progressive microcephaly with loss of cognitive
generalized hyperpigmentation, café-au-lait macules, skills, premature ovarian failure in females, recurrent
and hypopigmentation. sinopulmonary infections, and an increased risk for
●● There may be skeletal malformations (e.g., hypoplastic cancer, particularly lymphoma).
thumb, hypoplastic radius), microcephaly, ophthalmic
anomalies (e.g., microphthalmia, cataracts, Reticulated acropigmentation of Kitamura (RAK)
astigmatism, strabismus, epicanthal folds, hypotelorism, ●● It is a rare autosomal dominant pattern of inheritance

hypertelorism, ptosis), genitourinary tract anomalies, (caused by heterozygous mutation in the ADAM10
endocrine irregularities (hypothyroidism, glucose/ gene) with high penetrance.
insulin abnormalities), developmental delay, and/or ●● It is characterized by atrophic, reticulated,

intellectual disability. slightly depressed, well-demarcated, lentigo-like

●● Incidence of acute myeloid leukemia is 13% by age hyperpigmented macules.
50 years. There are solid tumors affecting the head and ●● It is seen commonly on the dorsum of hands and feet

neck, skin, gastrointestinal tract, and genitourinary during the first decade of life.
tract. ●● During adulthood, these macules slowly darken over time

●● DD: Neurofibromatosis 1 (because of café-au-lait and spread proximally to the extensor aspect of hand, neck,
macules), Nijmegen breakage syndrome (NBS) (short and face but rarely involve palms and soles (Figure 4.4a–c).

Figure 4.4  (a) Pigmented macules in a reticular fashion on the dorsum of feet in a patient with reticulated acropigmenta-
tion of Kitamura. (b) Discrete pigmented macules on the neck of a young man. (c) Coalescing, pigmented macules on the
face and neck of a young lady. (d) Palmar pits – an important clinical clue to reticulated acropigmentation of Kitamura.
(a–d – Courtesy: Dr. Piyush Kumar, Katihar, India.)
66  Reticulate and mottled pigmentation

●● Flexors may occasionally be involved. ●● Isolated genital involvement presents in a few patients.
●● Sunlight may aggravate the condition. ●● Mucous membranes are spared.
●● Other features include pits on palms (Figure 4.4d), ●● Common associated findings include pitted follicular
soles, and dorsal phalangeal surface; dermatoglyphic perioral and facial scars, comedones like follicular
disruption; partial non-scarring alopecia; and papules on the back or neck, epidermoid cysts,
occasionally plantar keratoderma. hidradenitis suppurativa, seborrheic keratosis, pilonidal
●● DD: Solar lentigines (hyperpigmented macules are cysts, SCC, and keratoacanthoma.
slightly atrophic and develops early in RAK). ●● Known variants are Haber’s syndrome, Galli Galli
disease, and pigmentatio reticularis faciei et colli.
Dowling-Degos disease (DDD) ●● DD: Acanthosis nigricans (velvety plaques, no follicular
●● This is a rare autosomal dominant disorder caused by involvement), neurofibromatosis type 1 (freckles in
mutations in the KRT5, POFUT1, or POGLUT1 gene. axillae and groin), reticulated acropigmentation of
●● There is no gender predilection. Kitamura (RAK) (atrophic hyperpigmented macules on
●● Onset is typically during the third or fourth decade of the dorsum of hands and freckles on face appear during
life. childhood, acral involvement).
●● It is characterized by acquired reticular

hyperpigmentation in the intertriginous areas. Galli-Galli disease

Reticulated hyperpigmentation with lentigo-like ●● This is a rare acantholytic variant of Dowling-Degos

brown macules and small brown papules, sometimes disease, linked to mutations in the KRT5 gene.
pruritic, begins in flexors such as axillae and groin ●● It is an autosomal dominant with variable penetrance,

(Figure 4.5a–c). As the disease progresses, it also but it can occur sporadically.
involves trunk, neck, intergluteal, inframammary areas, ●● It is a benign but very pruritic and unaesthetic

and inner aspects of arms and thighs. genodermatosis.

   

Figure 4.5  (a) Pigmented macules on the neck in Dowling Degos disease. (b) Axilla showing numerous coalescing pig-
mented macules in Dowling Degos disease. (c) Reticulate pigmented macules affecting neck and axilla. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India; c – Courtesy: Dr. Dipali Rathod, Mumbai, India.)
 Reticulate and mottled pigmentation  67

●● It is clinically characterized by reticulated appear in the intermammary area and less frequently in
hyperpigmentation, predominantly affecting the the interscapular and epigastric area.
flexures along with pruritic, erythematous, scaly ●● Lesions can also involve neck, upper trunk, antecubital
papules, similar to the DDD. fossa, and popliteal fossae (Figure 4.6a).
●● The papules coalesce to form confluent centrally and
Confluent and reticulated papillomatosis (CRP) reticulated peripherally (Figure 4.6b).
●● This is also known as confluent and ●● It is largely asymptomatic but rarely mild pruritus may
reticulated papillomatosis of Gougerot and be noted.
Carteaud. ●● CRP is limited only to the skin, with no systemic
●● CRP primarily is caused by a bacterium, Dietzia involvement. The mucous membranes, palms, and soles
papillomatosis, an aerobic gram-positive coccus/ are spared.
rod actinomycete. It may also be caused by ●● DD: Acanthosis nigricans (presence of peripheral
endocrinopathies, especially insulin resistance, obesity, reticulation and the absence of mucosal and nail
pituitary, and thyroid disorders. Others postulated involvement in CRP; in AN there are thicker, more
causes are ultraviolet (UV) light, keratinization, and velvety plaques, no scales, lack of reticulation;
Malassezia furfur. acanthosis nigricans appears prominently in the
●● Occurrence is mostly sporadic, but familial cases are axillae and groin area, while CRP presents on
also reported. the central back and chest), terra firma-forme
●● Young women are commonly affected. dermatosis (TFFD) (lesions can easily be removed
●● Initial lesions are 1–2 mm papules that enlarge rapidly with alcohol swab), Darier disease (involves
to 4–5 mm and become brown, hyperkeratotic, seborrheic area, palmoplantar pits, and nail
verrucous papules, patches, or thin plaques. Lesions first changes, such as blue-red striations and distal

Figure 4.6  (a) Confluent and reticulated papillomatosis with reticulated, scaly papules and plaques on the neck and
upper back. (b) Confluent and reticulated papillomatosis with reticulated, scaly papules and plaques on the central
chest. (a – Courtesy: Dr. Neethu Mary George, Sri Siddhartha Medical College, Tumkur, India; b – Courtesy: Dr. Anil
Patki, Pune, India.)
68  Reticulate and mottled pigmentation

V-shaped nicking, may be present), Pityriasis

versicolor (brown scales but no reticulation and
papillomatosis).

Prurigo pigmentosa
●● This is a rare inflammatory skin disease of unknown

pathogenesis.
●● Various etiological factors include friction with clothes,

sun exposure, physical trauma, contact allergens, H.

pylori infection, Borrelia spirochetes, atopic disease,
mechanical stimuli, and drugs such as bismuth
subsalicylate containing antacid.
●● Females are more commonly affected in third decade of

life.
●● It is characterized by a sudden onset of symmetrical

distribution of pruritic and erythematous macules,

urticarial papules, and plaques that may coalesce,
then involute within a week to form crusted and
scaly red papules leaving a macular reticulated
hyperpigmentation.
●● There is a predilection for back, neck, and chest.

Recurrence occurs at the same site.

●● Rarely, pustular and bullous variants present. Often,

lesions of different stages are seen.

●● DD: Confluent and reticulated papillomatosis (CRP)

(central confluence and peripheral reticulation, neck

and axilla spared, asymptomatic, no pruritus).       

Erythema ab igne Figure 4.7  Reticulate, persistent hyperpigmentation

●● This is also known as toasted skin syndrome or fire of erythema ab igne. (Courtesy: Dr. Hiral Shah, Baroda
Medical College, Vadodara, India.)
stains.
●● It is a localized skin condition caused by chronic and

repeated exposure to infrared radiation in the form of

heat insufficient to cause a direct burn. MOTTLED PIGMENTATION
●● Risk factors include heat applied to relieve pain,

working near heat exposure, decreased sensation, Epidermolysis bullosa simplex with mottled
habitually warming at fire places. pigmentation
●● Heat sources include hot water bottles, heating pads, ●● It is a rare autosomal dominant disorder resulting from
prolonged hot bathing, infrared lamps, laptops, open mutations in either the KRT5 or KRT14 gene. Males and
fires, etc. females are affected equally.
●● A lesion starts as transient macular erythematous ●● Clinically, it is characterized by mechanically induced
and blanchable; with repeated exposure to heat, the hemorrhagic blisters that usually heal without scarring
lesion becomes hyperpigmented and non-blanchable or milia formation. Scarring, when noted, is always
(Figure 4.7). atrophic. Sites affected are feet, knees, and hands.
●● Epidermal atrophy may overlie the reticulated Mucous membrane, teeth, and hair are generally spared.
pigmentation. Mottled pigmentation appears usually during infancy
●● These lesions are usually asymptomatic but can be and persists throughout life. It affects trunk, neck,
associated with pain, burning, and itching. axilla, and extremities (Figure 4.8a,b).
●● The bullous form of erythema ab igne is a rare variant. ●● DD: Autoimmune blistering diseases, staphylococcal
The long-term risk of cutaneous malignancies include scalded skin syndrome.
squamous cell carcinoma and Merkel cell carcinoma.
●● DD: Livedo reticularis (erythema is not persistent, Porphyria cutanea tarda
no hyperpigmentation), poikiloderma atrophicans ●● Porphyrias are photosensitivity disorders due to defects

vasculare (no distinct vascular pattern, more atrophic, in biosynthesis of heme. Porphyria cutanea tarda
telangiectatic), Majocchi disease (superficial and is the most common porphyria, which results from
annular). decreased activity of uroporphyrinogen decarboxylase,
 Reticulate and mottled pigmentation  69

Figure 4.8  (a) Epidermolysis bullosa simplex with mottled pigmentation in a child. Note the eroded blister on palm
(marked with black arrow). (b) Posterior side of same patient. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

the fifth enzyme in heme biosynthesis. Type 1 PCT ●● It generally manifests during infancy or
is an acquired or sporadic variant; type 2 PCT is an early childhood, with hyperpigmented and
autosomal dominant familial variant. hypopigmented macules of 3–5 mm that often
●● It presents with skin fragility, blister formation, erosion, increases in size and number until adolescence. Sites
crust, milia formation, thickening of skin and scar in involved are distal extremities, usually the dorsa of
sun-exposed areas. Vesicles and bullae may also develop the hands and feet and sometimes forearms and legs.
in sun-exposed areas. Palms, soles, nails, hair, and mucous membranes are
●● Mottled pigmentation may also be seen. Pigmentary spared (Figure 4.9).
changes are more frequently present in females on the ●● DD: Xeroderma pigmentosum (hyper- and
face (especially periocular). hypopigmented macules are photo-distributed
●● Other cutaneous findings include scarring alopecia, involving face and truck also), Dowling Degos
hypertrichosis, onycholysis, morpheaform, and disease (involvement of flexures, dark comedone-like
sclerodermoid plaque. lesions, pitted perioral acneiform scars), reticulate
●● DD: Pseudoporphyria, hepatoerythropoietic acropigmentation of Kitamura (no hypopigmentation),
porphyria, scleroderma, morphea, bullous pemphigoid, vitiligo (no hyperpigmentation), dyschromatosis
polymorphous light eruption.            universalis hereditaria (lesions present at birth with
truncal involvement). 
Dyschromatosis symmetrica hereditaria (DSH)
●● This is also called acroreticulate pigmentation of Dohi. Dyschromatosis Universalis Hereditaria (DUH)
●● It is a rare skin condition with an autosomal dominant ●● It is a rare disorder of dyschromatosis having both

pattern of inheritance, rarely autosomal recessive. autosomal dominant and recessive patterns of
Sporadic cases are also known. inheritance. Sporadic cases are also seen.
70  Reticulate and mottled pigmentation

SCC, and malignant melanoma of sun-exposed part of

eyes.
●● Neurological deficits include severe progressive
neurological degeneration, microcephaly, delayed
growth and sexual development, sensorineural
deafness, intellectual impairment, peripheral
neuropathy, dementia, and ataxia.
●● Pigmented xerodermoid is a variant of xeroderma
pigmentosum and presents at a later age, with onset in
the third or fourth decade of life. The clinical features
are similar to those of XP, but the risk of malignancy
is much less (compared to XP), and neurological
manifestations are rare (Figure 4.10d).
●● DD: Rothmund-Thomson syndrome (no
photosensitivity, absence of pigmentary changes),
trichothiodystrophy (absence of ichthyosis, brittle
hair and micrognathia, no increase in malignancies),
Figure 4.9  Dyschromatosis symmetrica hereditaria cerebro-oculo-facial syndrome (absence of xerosis,
with mottled pigmentation of the dorsa of both hands. poikiloderma, telangiectasia, and atrophy), porphyrias,
(Courtesy: Dr. Anil Patki, Pune, India.) basal cell nevus syndrome, UV sensitive syndrome
(solar lentigines, photosensitivity, otherwise normal).          

●● DUH usually develops dyschromia by the age of Rothmund-Thomson syndrome

6 years in most of the patients and at birth in few. ●● It is a rare genetic disorder with autosomal recessive

Hyperpigmented and hypopigmented macules of inheritance caused by mutations in RECQL4.

various sizes usually start on the hands then progress to Consanguinity is reported in few patients.
involve head, neck, trunk. Mottled pigmentation of the ●● Onset of the acute phase is in early infancy, with

tongue and oral mucosa may be seen occasionally. erythema, edema, vesicles on the cheeks and face,
●● Thin, hyperpigmented dystrophic nails with pterygium followed by the chronic and persistent phase, which
formation are rarely present. develops gradually over a period of months to
●● Extracutaneous abnormalities include short stature, years with development of poikiloderma (atrophy,
high tone deafness, neurosensory hearing defects, hyperpigmentation and hypopigmentation and
photosensitivity, glaucoma, cataracts, and seizures. telangiectasia) affecting the dorsal aspect of hands and
●● DD: Xeroderma pigmentosum (involves photo-exposed buttocks.
areas whereas DUH involves unexposed areas also), ●● Other features include sparse eyebrows, eyelashes, and

acropigmentation of Dohi (localized form involving scalp hair; hypoplastic or poorly formed nails; dental
only the acral areas).                  anomalies (microdontia, caries, hypoplastic teeth; short
stature; mental retardation; speech developmental delay;
Xeroderma pigmentosum (XP) hyperkeratosis of palms and soles; bilateral juvenile
●● It is an autosomal dominant disease caused by faulty cataracts; chronic vomiting; diarrhea; sensorineural
DNA repair mechanisms. deafness; and pituitary hypogonadism.
●● The skin is normal at birth, and cutaneous ●● There is increased risk for osteogenic sarcoma,

manifestations appear in early childhood, starting with myelodysplastic syndrome, and BCC and SCC.
marked photosensitivity and photophobia. Minimal ●● DD: Bloom syndrome (no true poikiloderma, recurrent

sun exposure can lead to sunburn, manifesting as otic and pulmonary infections), Werner syndrome
erythema, blistering, edema, and vesicles. After two (late onset, initial signs are hoarseness followed by
years of age, the patient develops solar lentigines and bilateral cataract, type 2 diabetes mellitus), xeroderma
freckles on sun-exposed areas such as face and limbs. pigmentosum (marked photosensitivity, lentigines and
Repeated sun exposure results in xerosis, hyper- and freckles on minimal sun exposure, xerosis, keratitis),
hypopigmentation, telangiectasia, atrophy, and scarring Kindler syndrome (marked photosensitivity; acral
of skin (Figure 4.10a–c). bullae at birth or after minor trauma; keratotic papules
●● Actinic keratosis, squamous cell carcinoma (SCC), of hands, feet, knees, elbows; atrophy of eyelid skin).
basal cell carcinoma (BCC), and melanoma are other
complications that can develop with age. Chronic arsenicism
●● Ocular abnormalities include photophobia, ●● Arsenic is a metal that can be ingested through water

conjunctivitis, keratitis, corneal opacification and or medicines or inhaled in occupational exposures.

ulceration, vascularization, ectropion, loss of eyelashes, Acute toxicity can be lethal. Chronic arsenic exposure
 Reticulate and mottled pigmentation  71

Figure 4.10  (a) Mottled pigmentation on the upper extremities in a case of xeroderma pigmentosum. (b) Xeroderma
pigmentosum with multiple facial malignancies and mottled pigmentation on the trunk. (c) Back of the same patient
(Figure 4.10b). (d) Pigmented xerodermoid with mottled pigmentation on the trunk. (a – Courtesy: Dr. Deverashetti
Srinivas, Nizamabad, India; b–d – Courtesy: Dr. Piyush Kumar, Katihar, India.)

is characterized by hyperpigmentation of palms, primary cutaneous lymphoma. Males are more

soles, groin, axilla, nipple, pressure point area with commonly affected than females.
superimposed raindrop-like guttate hypopigmented ●● MF has three cutaneous stages. In the patch stage
macules. Arsenical keratosis develops on hands and feet lesions are usually flat, non-palpable, erythematous
(Figure 4.11a–d). with fine scales and mild pruritus. There is skin
●● Other features include peripheral neuropathy, nasal atrophy of varying degrees with mottled hyper-
septal perforation, hepatomegaly, liver fibrosis, diabetes and hypopigmentation with telangiectasia present
mellitus, and diarrhea. Increased risk of malignancies in poikiloderma vasculare atrophicans, a variant
including Bowen’s disease, basal cell carcinoma, of patch phase. Lesions tend to occur initially on
squamous cell carcinoma, urinary bladder, liver and covered body areas such as buttocks, trunk, and
lung malignancies is noted. limb. Unusual shapes, such as annular, polycyclic,
●● DD: Dyschromatosis universalis hereditaria. or horseshoe, are common. In the plaque stage
there is erythematous plaque, which may ulcerate
Mycosis fungoides (MF) and become secondarily infected. Plaque may be
●● MF is the most common type of cutaneous T-cell hyperpigmented in darkly pigmented skin. The
lymphoma, accounting for around 50% of the all tumor stage may consist of patch, plaque, and
72  Reticulate and mottled pigmentation

  

Figure 4.11  (a) Increased pigmentation and depigmented macules in chronic arsenicism. (b) Back of the same patient
(Figure 4.11a). Few pigmented, scaly plaques may be appreciated. (c) Another patient with multiple hyperkeratotic scaly
plaques on the back in chronic arsenicism. (d) Palmar hyperkeratotic papules in chronic arsenicism. (a–d – Courtesy: Dr.
Piyush Kumar, Katihar, India.)

tumors. Tumors may arise from pre-existing plaque. ●● The etiology is unknown, but genetic, environmental
Tumor stage tends to ulcerate. and immunological factors have been considered.
●● DD: Patch stage – vitiligo, pityriasis alba, pityriasis ●● It is characterized by prepubertal onset of mottled
versicolor, idiopathic guttate hypomelanosis, leprosy, pigmentation (mixture of hyper- and hypopigmented
post-inflammatory hypopigmentation, drug reaction, and/or depigmented macules of various sizes) primarily
eczema, large plaque psoriasis. Plaque stage – large plaque affecting sun-exposed areas (Figure 4.12a–d).
psoriasis, lymphomatoid drug reaction, lymphomatoid ●● DD: Xeroderma pigmentosum (marked photosensitivity,
contact dermatitis. Tumor stage – cutaneous T-cell lentigines and freckles on minimal sun exposure,
lymphoma with diffuse pleomorphic infiltrates. xerosis, keratitis, telangiectasia, atrophy, and scarring
of skin, cutaneous malignancies), dyschromatosis
Amyloidosis cutis dyschromica universalis hereditaria (lesions present at birth with
●● It is a very rare and distinct variant of primary truncal involvement), reticulate acropigmentation
cutaneous amyloidosis. It may have an autosomal of Dohi (lesions appears during infancy or early
dominant inheritance pattern. childhood, only distal extremities are affected),
 Reticulate and mottled pigmentation  73

Figure 4.12  (a) Amyloidosis cutis dyschromica with both hyperpigmented and hypopigmented macules on the back. (b) Close-up
of lesions on the thigh. (c) Mottled pigmentation on the back in amyloidosis cutis dyschromica. (d) Close of lesions on the leg.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; c,d – Courtesy: Dr Hiral Shah, Baroda Medical College, Vadodara, India.)
74  Reticulate and mottled pigmentation

dyskeratosis congenita (progressive bone marrow failure to mutation of the AR/BANF1 gene. Males and
and clinically by triad of reticulated hyperpigmentation, females are equally affected.
nail dystrophy, leukoplakia). ●● Disease onset is usually in the third year
of life. It is characterized by reticulate
Progeria hyperpigmentation, skin atrophy, generalized
●● Hutchinson-Gilford syndrome loss of subcutaneous fat, prominent scalp veins,
●● It is a rare genetic disease caused due to mutation in thin nose and lip, micrognathia, prominent eyes,
LMNA gene characterized by accelerated aging that and short stature.
begins during infancy. The patient is usually normal ●● Other findings include nail dystrophy, progressive
at birth. alopecia, severe osteolysis (notably on mandible,
●● In the first year, reticulate hyperpigmentation clavicles, ribs, distal phalanges, and long bones),
develops on sun-exposed areas, with osteoporosis, joint stiffness (mainly of fingers,
sclerodermatous skin changes on abdomen, hands, knees, and elbows), dental anomalies,
upper thigh, and buttocks. Skin is thin, loose, and delayed closure of anterior fontanelle, absence
wrinkled on hands and feet, with sparse body hair, of cardiovascular, atherosclerotic and metabolic
alopecia of scalp, eyebrows, and eyelashes. complications.
●● Growth failure with rapid infantile fat loss results in ●● Survival is into adulthood.
prominent superficial veins, perioral cyanosis, small ●● DD: MDPL syndrome, Hutchinson Gilford
face, beaked nose, protruding eyes and ears, large syndrome, Penttinen syndrome (disease onset is in
cranium, frontal bossing and micrognathia giving infancy to childhood with normal stature).
appearance of “plucked bird.”
●● Other findings include dental anomalies, nail Photoaging
dystrophy, high-pitched voice, hearing loss, ●● It is premature aging of skin caused by repeated

corneal dryness, insulin resistance, premature exposure to ultraviolet radiation (UV) mainly from
atherosclerosis, myocardial fibrosis. sun and from other artificial sources as well as
●● Average survival is 12 years. environmental pollution.
●● DD: Werner syndrome, Nestor-Guillermo progeria ●● Photoaging usually appears after 50 years of age.

syndrome, Penttinen syndrome. ●● Pigmentary changes include mottled pigmentation,

●● Werner syndrome solar lentigines, freckles, diffuse hyperpigmentation,

●● It is also called adult progeria or pangeria. seborrheic keratoses, and guttate hypopigmentation.
●● It is a rare progeroid syndrome with an autosomal It affects mostly sun-exposed areas such as face, neck,
recessive pattern of inheritance caused by mutations back of hands, lips and upper chest and, in women,
in the RECQL2 (WRN) gene. The patient is normal between knee and ankle.
until the beginning of the second decade, when ●● Other findings include marked wrinkling around the

cessation of growth occurs near puberty. eyes and mouth, deep creases, forehead frown lines
●● Other features of syndrome develop during second that are visible when not frowning, dry and rough skin,
or third decade of life. Characteristic features include atrophy, inelastic or leathery skin, and telangiectasias.
short stature, beaked nose, prominent eyes, premature Dark skin tends to have much less pronounced and
graying of hair, alopecia, bird-like facies, high-pitched delayed onset of photoaging.
voice, teeth anomalies, and micrognathia. ●● Cutis rhomboidalis nuchae (sailor’s or farmer’s neck)

●● The cutaneous changes include reticulate occurs due to chronic sun-exposure. Skin becomes
hyperpigmentation, skin atrophy, sclerodermatous thick, leathery, tough, lichenified on the back of the
skin changes, nail dystrophy or hypoplasia, plantar neck. Favre-Racouchot syndrome presents with nodular
hyperkeratosis, and trophic ulcers of legs. elastosis, cysts, and comedones on malar and inferior
●● Associated systemic findings include juvenile periorbital, forearm, and helix of ear.
cataract, hypogonadism, hyperlipidemia, ●● DD: chronological aging (pigmentary changes are pale,

osteoporosis, type 2 diabetes mellitus, and white, hypopigmentation, fine wrinkles, skin is dry and
metastatic calcification. flaky).
●● There is an increased risk of melanoma,
osteosarcoma, and soft tissue sarcoma. Vagabond’s leukomelanoderma
●● DD: Hutchinson Gilford syndrome, Nestor- ●● It is a skin condition characteristically occurring in

Guillermo syndrome, Rothmund syndrome, elderly persons with dietary deficiency in association
mandibular hypoplasia deafness progeroid features with poor hygiene and heavy Pediculus humanus
and or lipodystrophy (MDPL) syndrome. corporis infestation.
●● Nestor-Guillermo syndrome ●● It presents as hypopigmented macules superimposed on

●● It is a rare genetic progeroid syndrome with a hyperpigmented background associated with severe
autosomal recessive pattern of inheritance due itching.
 Reticulate and mottled pigmentation  75

●● The lesions can be seen on any site but most commonly over extremities, genitalia, areola, and trunk.
affects axilla, groin, wrist, medial side of thigh, and Repigmentation occurs on treatment.
posterior neck.
●● DD: Chronic arsenicosis, leucoderma syphiliticum. Acquired brachial cutaneous dyschromatosis
●● This condition results from sun exposure over a
Onchocerciasis prolonged period of time.
●● Onchocerciasis, or river blindness, is caused by the ●● It presents with bilateral, asymptomatic, brown

nematode Onchocerca volvulus. patches with geographic borders, interspersed with

●● It predominantly affects skin and eyes. hypopigmented, slightly atrophic macules on the
●● Cutaneous manifestations include subcutaneous extensor aspect of forearms.
nodules containing adult worms, papular dermatitis, ●● It may be associated with poikiloderma of Civatte.

lichenification, and leukoderma. ●● DD: Lentigines.

●● Leukoderma is a finding seen in the late stage and is

accompanied by sparing of perifollicular skin. This is

described as leopard skin and is most often evident on REFERENCES
the shins. 1. Sardana K, Goel K, Chugh S. Reticulate pigmentary disorders.
●● DD: Chronic eczema with post-inflammatory Indian J Dermatol Venereol Leprol 2013;79:17–29.
pigmentary changes, leukoderma of scleroderma. 2. Sinha S, Kulhari A. Reticulate pigmentary disorders: A review.
Pigment Int 2019;6:67–76.
Treponematoses 3. Vachiramon V. Approach to reticulate hyperpigmentation. Clin Exp
Dermatol 2011;36(5):459–466.
●● Pinta: Hypomelanotic macules occur during late phase
4. Zhang J, Li M, Yao Z. Updated review of genetic reticulate pigmen-
of the disease and present as irregular vitiligo-like tary disorders. Br J Dermatol 2017;177(4):945–959.
depigmented areas surrounded by hyperpigmentation 5. Baselga E, Esterly NB. Genetic Epidermal Syndromes: Disorders
over bony prominences. Atrophy, xerosis, and alopecia Characterized by Reticulated Hyperpigmentation. In: Nordlund JJ,
Boissy RE, Hearing VJ, King RA, Oetting WS, Ortonne JP, editors.
may also be seen.
The Pigmentary System: Physiology and Pathophysiology. 2nd edi-
●● Yaws: Symmetric hypomelanotic macules may
tion. Oxford: Blackwell Publishing; 2006. P. 780–808.
develop in untreated tertiary yaws over the dorsal 6. Westerhof W, Dingemans KP. Generalized mottled pigmentation
aspect of wrists, hands, and metacarpophalangeal with postnatal skin blistering in three generations. J Am Acad
and interphalangeal joints. These macules enlarge and Dermatol 2004;50(5 Suppl):S65–S69.
7. Vachiramon V, Thadanipon K, Chanprapaph K. Infancy-
coalesce to involve the entire area.
and childhood-onset dyschromatoses. Clin Exp Dermatol
●● Bejel (Endemic syphilis): Well-defined
2011;36(8):833–838.
hypomelanotic macules may occur in late stages 8. Vachiramon V, Thadanipon K, Rattanakaemakorn P. Adult-onset
of bejel. They are distributed symmetrically dyschromatoses. Clin Exp Dermatol 2012;37(2):97–103.
 5
Papules: Localized

NIHARIKA RANJAN LAL

INTRODUCTION ●● DD: Acquired melanocytic nevus, Dermatofibroma,

skin appendageal tumor.
A papule is defined as an elevated, solid lesion of less than
1 cm in diameter. Papules may be localized or distributed
in a generalized fashion. They exhibit various morphologi- Fibrous papule of nose
cal characteristics in terms of color (violaceous in lichen ●● Also known as fibrous papule of the face and sporadic

planus and lichenoid diseases, red in vascular diseases angiofibroma, this is a benign angiofibroma affecting
such as cherry angioma and granuloma pyogenicum, yel- the nose most commonly.
low in lipid disorders), surface changes (rough in warts ●● It is mostly seen in the middle-aged patients

and angiokeratoma, scaly in psoriasis, shiny in molluscum presenting as a solitary, asymptomatic, flesh-colored,
and xanthelasma), and arrangement (coral-bead arrange- dome-shaped, firm papule seen predominantly on the
ment in multicentric reticulohistiocytosis) are unique to nose (Figure 5.2).
certain diseases. Regional distribution of localized papules
may further help in making a correct diagnosis; for exam-
Psoriasis
ple, syringoma and trichoepithelioma are mostly seen on
●● These are well-circumscribed, pruritic, circular, red
the face, angiolymphoid hyperplasia and milia en plaque
behind the ears. Some papules, such as Gottron papules papules or plaques with silvery-white, dry scales
and xanthomatous papules, may be indicators of systemic (Figure 5.3).2
●● Removal of scales reveals tiny bleeding spots: Auspitz
illnesses. Table 5.1 summarizes the common causes of
localized papules and their salient diagnostic points are sign.
●● They are mainly distributed on extensor surfaces such
discussed. (Facial papules and follicular papules have not
been discussed here.) as elbows, knees, buttocks, trunk.
●● They may develop on sites of trauma: Koebner’s

phenomenon.
Basal cell carcinoma ●● DD: Lichen planus, tinea corporis, lichen
●● This is the most commonly occurring cancer in the simplex.
world, a slow-growing tumor for which metastasis is
rare.
●● Clinical features are flesh-colored or pink pearly Lichen planus
papules; these appear on the head or neck in 85% of ●● Shiny, red/purple-colored, flat-topped papules are

cases.1 Patients usually present late when the lesion present (Figure 5.4a).
grows in size and becomes plaque (Figure 5.1). ●● Thin, adherent, transparent scale is present.

●● Ulceration or telangiectatic vessels are also commonly ●● Wickham’s striae – fine whitish points/lacy lines –

seen. may be seen on the surface of well-developed

●● The majority occur on areas of skin that are regularly papules. 2
exposed to sunlight or other ultraviolet radiation. ●● A common site is the flexor surfaces of extremities
●● They can be highly destructive and disfiguring to local (Figure 5.4b).
tissues when presentation is delayed or treatment is ●● It exhibits Koebner phenomenon.

inadequate. ●● DD: Psoriasis, lichen simplex chronicus.

76 DOI: 10.1201/9781351054225-11
 Papules: Localized  77

Table 5.1  Morphologic characteristics of localized papules

A. Single • Basal cell carcinoma 5. Yellow papules • Xanthelasma palpebrarum

• Granuloma pyogenicum • Juvenile xanthogranuloma
• Fibrous papule of nose • Benign cephalic histiocytosis
• Steatocystoma simplex • Milia like idiopathic calcinosis cutis
• Acquired melanocytic nevus 6. White papules • Milia
B. Multiple • Molluscum contagiosum
1. Erythematous a. Scaly • Calcinosis cutis (milia like)
• Psoriasis 7. Skin-colored • Syringoma
• Lichen planus papules • Trichoepithelioma
• Subacute cutaneous lupus • Eccrine hidrocystoma
erythematosus • Pearly penile papules
• Discoid lupus erythematosus • Lichen nitidus
b. Non-scaly • Lichen scrofulosorum
• Papular sarcoidosis • Skin tags
• Lupus vulgaris • Acne keloidalis nuchae
• Granuloma annulare • Piezogenic pedal papules
• Lupus miliaris disseminatus • Steatocystoma multiplex
faciei (LMDF) • Neurofibroma
• Jessner’s lymphocytic • Localized papular mucinosis
infiltrate 8. Follicular • Lichen spinulosus
• Granuloma faciale papules • Keratosis pilaris
• Multicentric • Pityriasis rubra pilaris
reticulohistiocytosis • Follicular lichen planus
2. Papules mixed • Rosacea (with pustules) 9. Linear papules • Linear psoriasis
with vesicles or • Acne vulgaris (with pustules) • Linear lichen planus
pustules • Gianotti-Crosti syndrome (with • Linear verrucous epidermal
vesicles) nevus
3. Hyperpigmented • Granular parakeratosis • Inflammatory linear verrucous
• Darier disease epidermal nevus
• Dermatosis papulosa nigra • Lichen striatus
• Reactive perforating • Linear porokeratosis
collagenosis • Nevus comedonicus
• Lichen amyloidosis 10. Vascular • Lobular capillary hemangioma
• Adenoma sebaceum papules • Angiokeratomas
• Bowenoid papulosis • Bacillary angiomatosis
• Prurigo nodularis • Cherry angioma
• Acquired melanocytic nevi • Tufted angioma
• Eruptive vellus hair cyst • Targetoid hemosiderotic
• Gottron papules (violaceous) hemangioma
4. Verrucous • Verruca vulgaris • Angiolymphoid hyperplasia with
surface • Acrokeratosis verruciformis eosinophilia
• Seborrheic keratosis 11. Papules with • Papulonecrotic tuberculid
• Nevus sebaceous necrotic center • Lymphomatoid papulosis

Subacute cutaneous lupus erythematosus, ●● Lesions are not indurated, and heal without scarring;
papulosquamous variety vitiligo-like hypopigmentation may occur.
●● This presents as erythematous, scaly papules and ●● DD: Psoriasis, nummular eczema, pemphigus foliaceus.
plaques (Figure 5.5).2
●● It occurs in sun-exposed areas, including the upper Discoid lupus erythematosus (DLE)
thorax (V distribution), upper back, and the extensor ●● These are the most common lesions of chronic

surfaces of arms and forearms. cutaneous lupus erythematosus (CCLE).

78  Papules: Localized

Figure 5.1  Hyperpigmented annuar plaque on face in basal

cell carcinoma. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
Figure 5.2  Fibrous papule of nose. Solitary, firm, skin-
colored papule. (Courtesy: Dr. Swetalina Pradhan, AIIMS
Patna, India.)

●● Clinical features are well-demarcated, scaly,

erythematous macules or papules.
●● It gradually develops into an indurated discoid (coin-
shaped) plaque with an adherent scale that is painful to
remove (Figure 5.6).3
●● There may be extension into the hair follicle, result in
scarring alopecia.
●● Through time, lesions typically become atrophic, with
hyperpigmentation peripherally and depigmentation
centrally.
●● Localized DLE commonly involves the head and neck,
in particular scalp and ears.
●● DD: Psoriasis, lichen planus.

Papular sarcoidosis
●● This presents as asymptomatic, red-brown/purple

papules < 1 cm in diameter4 (Figure 5.7).

●● It often affects the eyelids, periorbital area, and

nasolabial folds.
●● It may herald the onset of systemic disease.

●● It may heal spontaneously with/without scarring.

●● DD: Acne, rosacea, lupus miliaris disseminatus faciei

(LMDF), lupus vulgaris.

Lupus vulgaris
●● This presents as soft reddish-brown papules with apple-

Figure 5.3  Erythematous scaly papules of psoriasis. jelly nodules on diascopy (Figure 5.8a,b).
 Papules: Localized  79

Figure 5.4  (a) Violaceous, flat-topped papules in lichen planus. (b) Lichen planus, flexor distribution. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

●● A lesion starts as a papule, but patients usually present

late when the lesion reaches plaque stage.
●● DD: Sarcoidosis, Jessner’s lymphocytic infiltration.

Granuloma annulare
●● This is a common, benign, chronic inflammatory

disorder of unknown cause.

●● It is characterized by grouped papules in an enlarging

annular shape (Figure 5.9).1

Figure 5.6  Erythematous papules with adherent scales on

the back in DLE. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 5.5  Psoriasiform papules in subacute lupus erythema- Figure 5.7  Papular sarcoidosis. (Courtesy: Dr. Piyush
tosus on back. (Courtesy: Dr. Piyush Kumar, Katihar, India.) Kumar, Katihar, India.)
80  Papules: Localized

Lupus miliaris disseminatus faciei (LMDF)

●● Granulomatous eruption characterized by

monomorphic, reddish-brown papules and nodules

(Figure 5.10a,b).5
●● It is predominantly localized on the face (chin, forehead,

cheeks, eyelids).

Figure 5.8  (a) Papular lesion of lupus vulgaris. (b) Grouped,

coalescing erythematous papules on the knee in Lupus
vulgaris. (a – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● The most common presentation is plaque; uncommon

forms are subcutaneous, perforating, patch, linear, and
rarely acute painful papules.
●● A small percentage of cases are associated with
autoimmune diseases, systemic diseases, infections, and
malignancies.
●● D/D: Lupus vulgaris, sarcoidosis, Hansen’s disease.

Figure 5.10  (a) Erythematous, monomorphic papules

distributed symmetrically on face in lupus miliaris dis-
Figure 5.9  Erythematous shiny papules in granuloma seminatus faciei. (b) Lupus miliaris disseminatus faciei. The
annulare. Annular plaque is also seen on forearm. scaling seen on the face is due to retinoid application.
(Courtesy: Dr. Piyush Kumar, Katihar, India.) (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Papules: Localized  81

●● Diascopy often reveals an apple-jelly nodule-like

appearance.
●● It is self-limiting and heals with pock-like scars.
●● DD: Sarcoidosis, acne, lupus vulgaris.

Jessner’s lymphocytic infiltration

●● This presents with asymptomatic, erythematous

papules/plaques that tend to take an annular or

horseshoe-like configuration.
●● The face is the most common site.

●● Some degree of photosensitivity may be associated.

●● DD: Pseudolymphoma, papular sarcoidosis, tumid LE,

PMLE.

Papulopustular rosacea
●● This is a chronic inflammatory skin disease commonly

affecting the face.

●● It is characterized by flushing, redness, papules,

pustules, and dilated blood vessels (Figure 5.11).

●● It is symmetrically distributed on central face.

●● Triggering factors are spicy/caffeinated food,

sunlight, stress, strenuous exercise, and some types

of cosmetics.
●● DD: Acne vulgaris, steroid dermatitis, demodicosis.

Acne vulgaris
●● This is a common skin condition starting in puberty.

●● The face is the most common site; the back and chest Figure 5.12  Erythematous papules on cheek in a teenager
may be affected. with acne.

●● Preadolescent acne mainly occurs on forehead,

adolescent acne on cheeks, and adult acne on
jaw. 6
●● A lesion begins with a microcomedo that may evolve
into a papule/nodule (Figure 5.12).
●● Other morphologies seen are comedones, pustules,
nodules, cysts, scars, and hyperpigmentation.
●● DD: Bacterial folliculitis, rosacea, LMDF.

Granular parakeratosis
●● This is a rare, idiopathic, benign skin condition.

●● Erythematous to brown, scaly, crusted, hyperkeratotic

papules coalesce to form plaques.

●● Intertriginous sites are most commonly affected,

especially the axilla.7

●● No definitive cause is known; it may be because

of physical irritation/contact allergy, or it could

be a disorder of cornification rather than contact
dermatitis.
●● DD: Darier disease, Hailey-Hailey disease (HHD),

pemphigus vegetans.

Darier disease
●● This is genodermatosis with autosomal dominant
Figure 5.11.  Erythema, papules, and few pustules are
symmetrically distributed over the face in rosacea. inheritance.
(Courtesy: Dr. Piyush Kumar, Katihar, India.) ●● Distribution is on seborrheic and intertriginous areas.
82  Papules: Localized

●● Coalescence of the papules produces irregular warty ●● Oral mucosa is involved in 50% of cases; white/red
plaques that, in flexures, become hypertrophic and papules appear on palatal/alveolar mucosa (cobblestone
malodorous with painful fissures (Figure 5.13a,b).8 appearance).
●● Associated nail abnormalities include nail fragility, red
and white longitudinal stripes, and V-shaped notches at
the free margin of nails.
●● DD: HHD, seborrheic dermatitis, acanthosis nigricans,
pemphigus vegetans.

Dermatosis papulosa nigra (DPN)

●● This is considered a variant of seborrheic keratosis,

more common in black people.

●● Sun exposure plays major role.

●● Multiple small, 1–5 mm, hyperpigmented,

asymptomatic papules appear (Figure 5.14).

●● It is mostly seen on the face (malar regions and

forehead, followed by chin) and less commonly over the

neck, upper back, and chest.
●● It develops during puberty and increases with age.

●● DD: Skin tags, plane warts.

Figure 5.13  (a) Darier disease presenting as dirty, warty Figure 5.14  Dermatosis papulosa nigra seen as multiple,
papules in elbow flexures. (b) Darier disease affecting hyperpigmented, small papules on face. (Courtesy:
axilla. Dr. Piyush Kumar, Katihar, India.)
 Papules: Localized  83

Reactive perforating collagenosis ●● It is usually self-healing in six to eight weeks without

●● It is a type of transepidermal elimination disorder any therapy but often recurs.
in which altered collagen is extruded from the ●● DD: Prurigo nodularis, Kyrle’s disease.
dermis.
●● It may be inherited or acquired. Lichen amyloidosis
●● The acquired form is associated with trauma, insect ●● This is the most common form of primary localized

bites, diabetes, or chronic renal failure. cutaneous amyloidosis.

●● It manifests as isolated papules with keratotic plugs in ●● The cause is unknown, maybe induced by scratching.

the center (Figure 5.15a,b).9 ●● Clinical features include multiple pruritic, firm,

●● It occurs mostly on the extremities and back, sometimes hyperpigmented, hyperkeratotic papules on the shins
on the face and neck. that later coalesce to give the appearance of a rippled
●● Extremely pruritic Koebnerization is seem in pattern (Figure 5.16).
Figure 5.15c. ●● DD: Prurigo nodularis, lichen planus.

Figure 5.15  (a) Reactive perforating collagenosis present-

ing as multiple papules with a central keratotic plug.
(b) Reactive perforating collagenosis. (c) Koebnerization Figure 5.16  Hyperpigmented, keratotic papules on shin in
seen in reactive perforating collagenosis. (a–c – Courtesy: lichen amyloidosis. (Courtesy: Dr. Piyush Kumar, Katihar,
Dr. Piyush Kumar, Katihar, India.) India.)
84  Papules: Localized

Figure 5.18  Smooth-surfaced, pigmented papules of

bowenoid papulosis. (Courtesy: Dr. Piyush Kumar, Katihar,
India.)

●● Clinical features include pigmented papules ranging in

size from 0.2–3 mm, resembling warts (Figure 5.18).
●● Frequent sites are the penis and vulva; other areas such
as oral, periungual, and neck region may be affected.
●● In females, it is referred to as multifocal vulvar
intraepithelial neoplasia.
●● DD: Genital warts, lichen planus, molluscum
contagiosum, seborrheic keratosis, and Bowen’s disease.

Acne keloidalis
●● This is a chronic inflammatory condition seen in dark-
Figure 5.17  Red-brown papules on the central face in facial skinned races with curly or kinky hair.
angiofibroma. (Courtesy: Dr. Piyush Kumar, Katihar, India.) ●● Males are predominantly affected, usually after puberty.

●● Androgen may play an important role; skin injuries

from irritation, occlusion, trauma, friction, and hair-

Adenoma sebaceum (facial angiofibroma) cutting practices are contributing risk factors.
●● This is a benign tumor with both angiomatous and ●● Clinical features include multiple papules and/

fibrous components. or pustules on nuchal and/or occipital areas (acne

●● It is one of the cutaneous hallmarks of tuberous sclerosis. keloidalis nuchae). Other sites, such as the upper trunk,
●● It starts appearing by 5–10 years of age and gradually too may be affected (Figure 5.19a,b).
increases till puberty. ●● It is extremely pruritic; bleeding may occur.
●● Skin-colored to hyperpigmented papules commonly ●● Secondary bacterial infection by Staphylococcus aureus

involve the central face, bilaterally and symmetrically is common.

covering the glabellar area, the dorsum of nose, ●● Recurrent episodes lead to scarring alopecia.

adjoining cheeks, upper lip, and chin (Figure 5.17).10 ●● DD: Keloid, tufted folliculitis.
●● DD: Trichoepithelioma, acrochordons, intradermal

nevi, acne, syringoma. Prurigo nodularis

●● This is chronic-relapsing, highly pruritic dermatosis.
Bowenoid papulosis ●● It is characterized by several to hundreds of

●● This is caused by the human papilloma virus (HPV) in hyperkeratotic, pruritic papules and nodules, sometimes
the sexually active age group. excoriated or ulcerated, with a tendency to symmetrical
●● The causative agent is HPV 16; HPV serotypes such as distribution on the shoulders, on the back, the buttocks,
18, 31, 33, 39, and 52 have also been implicated. and the upper and lower limbs (Figure 5.20a,b).
 Papules: Localized  85

●● Sparing of the upper mid back, known as “butterfly

sign,” is distinctive.
●● It may be associated with atopy, type 2 diabetes mellitus,
thyroid disorders, HCV infection, non-Hodgkin
lymphoma and psychiatric disorders, particularly
depression and anxiety.
●● DD: Lichen planus, nodular amyloidosis.

Acquired melanocytic nevus

●● Common acquired melanocytic nevi (CAMN) is a

common, largely acquired, condition resulting from

benign proliferation of nevus cells.
●● It may appear as macules, papules, and nodules.1

●● It is typically less than 1 cm (often < 6 mm) in

diameter, with smooth and regular borders

(Figure 5.21).
●● Compound melanocytic nevi are elevated in relation

to adjacent uninvolved skin and present as papules or

nodules and are often lighter in color, ranging from tan
to light brown.
●● Intradermal melanocytic nevi often display no

significant pigmentation, presenting as a flesh-colored

papule or nodule.
●● DD: Basal cell carcinoma, solar lentigo, melanoma,

seborrheic keratosis.

Figure 5.19  (a) Acne keloidalis nuchae seen as keloid-like

papules on the nuchal area. (b) Acne keloidalis lesions on
the chest. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 5.20  (a) Prurigo nodularis. (b) Hyperpigmented,

excoriated papules in prurigo nodularis. Older lesions Figure 5.21  Shiny, smooth, hyperpigmented papules in
have healed with scarring. (a,b – Courtesy: Dr. Piyush acquired melanocytic nevus. (Courtesy: Dr. Piyush Kumar,
Kumar, Katihar, India.) Katihar, India.)
86  Papules: Localized

Figure 5.22  (a) Solitary papule with rough, warty surface in

verruca vulgaris. (b) Multiple, flat-topped, warty papules on
the palm. (c) Filiform wart. (a–c – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

Eruptive vellus hair cyst ●● Clinical features include flat-topped, polygonal, brownish to
●● This is a rare follicular developmental abnormality of skin-colored papules and verrucous plaques (Figure 5.23).
the vellus hair follicles. ●● Sites are the back of the proximal and distal
●● Clinical features include multiple small normochromic interphalangeal joints of the hands and feet.
or hyperpigmented, dome-shaped papules, soft to firm ●● Punctate keratosis on the palms and soles may also be seen.
in consistency, ranging from 1 to 5 mm in diameter. ●● DD: Epidermodysplasia verruciformis, plane warts.
●● It may be topped with central puncta or a umbilicated

or hyperkeratotic crust.
●● Common sites are the chest and extremities, rarely

abdomen, neck, axillae, face, and groin.

●● DD: Comedones, keratosis pilaris, infundibular cysts.

Verruca vulgaris
●● These are hyperkeratotic, exophytic, and dome-shaped

papules or nodules (Figure 5.22a–c).

●● They are located on fingers, hands, knees, elbows, or any

other sites of trauma.

●● They exhibit pseudo Koebnerization.

●● DD: lichen planus, seborrheic keratosis, epidermal nevus.

Acrokeratosis verruciformis Figure 5.23  Flat-topped, skin-colored papules on dor-

●● This is rare genodermatosis with autosomal dominant sum of hand in acrokeratosis verruciformis. (Courtesy:
inheritance. Dr. Piyush Kumar, Katihar, India.)
 Papules: Localized  87

Figure 5.25  Brown papules on scalp coalescing to form

plaque in nevus sebaceous. Note verrucous surface
changes, indicating post-pubertal growth. (Courtesy:
Dr. Piyush Kumar, Katihar, India.)

Figure 5.24  Flat-topped, hyperpigmented papules with Juvenile xanthogranuloma

a stuck-on appearance in seborrheic keratosis. (Courtesy: ●● This condition belongs to the group of non-Langerhans
Dr. Piyush Kumar, Katihar, India.) cell histiocytosis.
●● It affects infants and small children.

●● It usually appears as yellow-brown papules and nodule,

Seborrheic keratosis
●● These are the most common benign epidermal tumors
often on the head and neck (Figure 5.26).11
that are associated with increased age.
●● Clinical features include solitary or multiple well-

demarcated brownish papules or plaques with a

verrucous surface (Figure 5.24).
●● Common sites are the head, neck, and trunk.

●● DD: Actinic keratoses, verruca vulgaris, lentigines.

Nevus sebaceous
●● This is a congenital hamartomatous lesion with an

epithelial and adnexal origin.

●● It is present in approximately 0.3% of newborns.

●● It may contain any component of skin, including sebaceous

and apocrine glands or hair follicles.

●● It appears as smooth or velvety yellow-orange well-

circumscribed grouped papules and plaques (Figure 5.25).11

●● Hormonal changes at puberty may lead to verrucous

changes.
●● The most common site is the vertex of the scalp.

●● It may be associated with syndromic features such

as mental retardation, central nervous system

abnormalities, oculocardiac defects, or skeletal
abnormalities (linear nevus sebaceous syndrome, or Figure 5.26  Yellow-orange solitary papule on trunk in
Schimmelpenning syndrome). juvenile xanthogranuloma. (Courtesy: Dr. Santosh Rathod,
●● Malignant changes may occur.
Smt. NHL Municipal Medical College, V.S. Hospital
●● DD: Warts, syringocystadenoma papilliferum. Ahmedabad, India.)
88  Papules: Localized

Figure 5.27  Papule of benign cephalic histiocytosis.

(Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Sporadically, internal organs may get affected, including

eyes, lungs, liver, central nervous system, and bones.
●● DD: Urticaria pigmentosa, xanthoma.

Benign cephalic histiocytosis

●● This is also known as papular histiocytosis of the head.

●● It is a rare self-limiting non-Langerhans histiocytosis

that typically starts at the end of first year of life.

●● Clinical features include asymptomatic eruption of

yellow to brown papules on the head and neck of infants

and young children (Figure 5.27).
●● Subsequently it spontaneously regresses, leaving

atrophic pigmented macules.

●● DD: Plane warts, multiple Spitz nevi, juvenile

xanthogranuloma, Langerhans cell histiocytosis,

urticaria pigmentosa, generalized eruptive histiocytosis,
and lichenoid sarcoidosis.

Milia-like idiopathic calcinosis cutis

●● Milia-like idiopathic calcinosis cutis (MICC) is a

peculiar subtype of idiopathic calcinosis cutis.

●● The lesions appear as multiple, round, whitish papules

resembling milia (Figure 5.28a,b).

●● The predilection sites are the hands and feet, but it can

occur on the any part of the body.

●● Calcified sweat ducts have been described in some patient

as the pathogenesis; another theory is that the lesions

represent micro epidermal cysts that secondarily generate Figure 5.28  (a) Milia-like calcinosis cutis in a child
an inflammatory reaction and calcium deposition. with Down syndrome. (b) Papules of calcinosis cutis.
●● May heal spontaneously, with or without scarring. (a – Courtesy: Dr. Piyush Kumar, Katihar, India;
●● DD: Warts, epidermal cysts, molluscum contagiosum, b – Courtesy: Dr. Dependra Kumar Timshina, Remedy
and syringomas. Clinics, Siliguri, India.)
 Papules: Localized  89

Figure 5.29  Milia seen as white papules on the cheek.

(Courtesy: Dr. Piyush Kumar, Katihar, India.)

Milia (singular: milium)

●● Milia are tiny white keratin-containing cysts.

●● They present as 1–2 mm asymptomatic, umbilicated,

white or yellowish, spherical subepidermal keratin cysts

occurring primarily on the face in all ages.
●● In neonatal milia many newborns have milia on face,

hard palate (Bohn nodules), and gum margin (Epstein

pearls). The lesions resolve spontaneously.
●● Primary milia is common in children and young

adults, and are usually found around eyes, cheeks, and

genitalia. They arise from an infundibular portion of
the hair (Figure 5.29).
●● Secondary milia may occur over the area of trauma or

skin injury as in second degree burn, epidermolysis

bullosa, porphyria cutanea tarda, bullous lichen planus,
radiotherapy, chronic steroid use, cosmetic procedure like
dermabrasion, etc. They arise from the eccrine sweat ducts.
●● Milia en plaque present as numerous, grouped milia

on erythematous and oedematous base. The lesions

are mostly seen in the post-auricular area, on eyelid,
or cheek.

Molluscum contagiosum
●● Molluscum contagiosum is caused by Molluscum

contagiosum virus (MCV) (MCV strain 1-4).

●● It is characterized by firm, white to flesh-colored,

dome-shaped, pearly papules, having a central

umbilication from which one can express a cheesy
material (Figure 5.30a,b).12
●● In children it commonly affects face, axilla, and

extremities.
●● In the sexually acquired form (usually in adults) lesions

occur on the genitalia and inner thighs (Figure 5.30c).

Figure 5.30  (a) Pearly white, umbilicated, dome-shaped
●● In immunocompromised patients, lesions may be
papules in molluscum contagiosum. Some of the bigger
atypical (like giant MC more than 1.5 cm) and may lesions appear erythematous. (b) Molluscum contagiosum.
involve mucosal surface. (c) Umbilicated papules of molluscum contagiosum affecting
●● DD: Acne, milia, warts, trichoepithelioma. the penis. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
90  Papules: Localized

Figure 5.31  Pearly penile papules. Pink, small papules

arranged around coronal sulcus.

Pearly penile papules

●● These are common, benign lesions that appear on the

corona of the glans penis during adolescence or early

adulthood.
●● Clinical features include uniform, skin-colored, dome-

shaped papules that orient in one to two rows around

the glans penis (Figure 5.31).
●● DD: Condyloma acuminata, molluscum contagiosum.

Lichen nitidus
●● Lichen nitidus is an inflammatory skin eruption of

unknown cause; it typically affects children and young

adults. Figure 5.32  Shiny, pinpoint papules in lichen nitidus
●● It presents as shiny, flat-topped, skin-colored, pinhead- exhibiting Koebner phenomenon. (Courtesy: Dr. Piyush
sized papules (Figure 5.32).4 Kumar, Katihar, India.)
●● These are typically arranged in groups on the chest,

abdomen, genitalia, and upper extremities.

●● Koebner phenomenon are seen. ●● They often develop at sites of friction.
●● Oral mucosa (white papules) and nail lesions ●● They are reported to be associated with many diseases,
(thickening, ridging, pitting) may be seen. including type 2 diabetes mellitus (DM) and obesity.
●● There is spontaneous resolution. ●● DD: Verruca plana, DPN.
●● DD: Lichen spinulosus, papular mucinosis, plane warts.

Lichen scrofulosorum
●● This is a rare tuberculid.

●● Tiny, skin-colored, perifollicular papules are arranged

in groups.13
●● They are normally smooth; they may have spiny

projections with fine scales.

●● Usually, they appear on the abdomen, chest, back, and

proximal parts of the limbs.

●● The condition shows as a strongly positive tuberculin test.

●● DD: Lichen nitidus, keratosis pilaris, lichen spinulosus.

Skin tags
●● These are also known as fibroepithelial polyps, or

acrochordons.
●● A common benign skin condition, it consists of a

bit of skin projecting from the surrounding skin Figure 5.33  Skin tag. Skin-colored, soft sessile, and
(Figure 5.33).1 pedunculated papules on neck.
 Papules: Localized  91

Piezogenic pedal papules

●● Skin-colored asymptomatic papules are observed in

usually healthy individuals, especially athletes and

marathon runners.
●● They are constituted of herniations of fat tissue into

weight-carrying connective tissue of the heels

(Figure 5.34a,b).
●● They occur as a result of continuous pressure on the

heels from lifting excessive weight, jumping, running,

or carrying heavy loads.
●● A few cases have been reported to occur in patients with

rheumatic heart disease and Ehler Danlos syndrome.

●● DD: Xanthoma, gouty tophi.

Steatocystoma multiplex
●● This is a hamartomous malformation of the

pilosebaceous duct junction.

●● Clinical features include dome-shaped, asymptomatic

papules; early lesions are translucent and change to a

yellowish color with age (Figure 5.35a,b).

Figure 5.34  (a) Piezogenic pedal papules. (b) Piezogenic

pedal papules (a – Courtesy: Dr. Sunil Patwardhan, Figure 5.35  (a) Yellow, dome-shaped papules on fore-
Pune, India; b – Dr. Anup Kumar Tiwary, Consultant head in steatocystoma multiplex. (b) Steatocystoma
Dermatologist, Yashoda Hospital and Research Center, multiplex over chest. (b – Courtesy: Dr. Piyush Kumar,
Ghaziabad, India.) Katihar, India.)
92  Papules: Localized

●● Common sites are axilla, groin, trunk, scrotum, and the

proximal extremities. The sternal region is commonly
affected in males.
●● A solitary lesion is known as steatocystoma simplex.
●● DD: Eruptive vellus hair cyst, milia, trichilemmal
cyst.

Neurofibromas
●● Neurofibromas are benign tumors that arise from

Schwann cells.
●● Dermal tumors are soft dome-shaped tumors but can

also present as pedunculated, nodular, or plaque-like

(Figure 5.36).1
Figure 5.37  Psoriatic papules and plaques arranged
●● They demonstrate a buttonhole sign.
linearly along Blaschko’s line. (Courtesy: Dr. PC Das and
●● Histologically, they are made of mixed cells like
Dr. Piyush Kumar, Katihar, India.)
Schwann cells, perineural cells, and fibroblasts.
●● They are associated with other features like café-au-

lait macules, Crowe’s sign, plexiform neurofibroma in that may coalesce to form plaque, causing thickening
neurofibromatosis-1 (NF1), and with schwannomas and and hardening of the skin.
meningioma in NF2. ●● DD: Lichen nitidus, scleromyxedema, eruptive LP,
●● DD: Lipoma, dermal melanocytic nevus,
mucinous LE.
acrochordons.

Localized papular mucinosis Linear psoriasis

●● Also called lichen myxedematosus, this is a primary ●● This is a rare form of psoriasis.

cutaneous mucinosis characterized by dermal mucin ●● It is characterized by late onset and rapid

deposition in absence of thyroid disorder. progression.

●● The pathogenesis is a proliferation of fibroblasts ●● There is linear distribution of psoriatic lesions along

with fibrosis and excessive deposition of mucin Blaschko’s lines (Figure 5.37).
(glycosaminoglycans) in the dermis. ●● DD: Linear LP, inflammatory linear verrucous

●● It is clinically characterized by a few to multiple, 2–5- epidermal nevus (ILVEN) (no response to antipsoriatic
mm, skin-colored, firm, waxy, dome-shaped papules treatment).

Linear lichen planus

●● The etiology is unknown; however, it is associated with

HIV and hepatitis C infection.

●● It is much more common in children than adults.

●● Pruritic violaceous papules follow lines of Blaschko

(Figure 5.38).
●● It may co-exist with classic LP lesions.

●● Rarely, it may have zosteriform/dermatomal

distribution following herpes zoster infection.

●● DD: Linear Blaschkitis, lichen striatus, linear

porokeratosis.

Figure 5.36  Small, soft, skin-colored papule of neurofi- Figure 5.38  Linear lichen planus. (Courtesy: Dr. Piyush
broma on the chin and a nodule over upper lip. Kumar, Katihar, India.)
 Papules: Localized  93

Linear verrucous epidermal nevus (linear VEN) Inflammatory linear verrucous epidermal nevus
●● This is present at birth or in infancy. (ILVEN)
●● Lesions are flat, velvety, papillomatous in the ●● This is characterized by early onset and slow progression.

newborn. ●● It is extremely pruritic.

●● They are keratotic and verruciform in adolescence. ●● Erythematous and hyperkeratotic verrucous/lichenoid/

●● Color may range from skin color to brown. psoriasiform papules appear along lines of Blaschko15
●● Lesions are arranged in streaks or swirls along Blaschko (Figure 5.40).
lines (Figure 5.39a,b).14 ●● DD: Linear psoriasis, linear VEN.

●● When generalized, it is known as “systematized

epidermal nevus.”
●● Multiple lesions may be associated with defects in the

skeletal and central nervous system; this is known as

“epidermal nevus syndrome.”
●● DD: Linear psoriasis, linear porokeratosis, lichen

striatus.

Figure 5.39  (a) Linear verrucous epidermal nevus on the Figure 5.40  Grouped erythematous scaly and crusted
neck. (b) Linear verrucous epidermal nevus on the calf. papules along the lines of Blaschko. (Courtesy: Dr Piyush
(b – Courtesy: Dr. Piyush Kumar, Katihar, India.) Kumar, Katihar, India.)
94  Papules: Localized

Lichen striatus
●● This is usually seen in young children with female

preponderance.
●● Affects the age range between 5 and 15 years.

●● Tiny 1- to 2-mm flat-topped scaly erythematous papules

appear along Blaschko’s lines.

●● It is commonly seen on limbs.

●● It is asymptomatic or mildly pruritic.

●● It will spontaneously regress in about one year.

●● DD: Linear LP, linear VEN, linear psoriasis.

Linear porokeratosis
●● Porokeratosis is a disorder of keratinocyte growth and

differentiation.
●● It presents as sharply demarcated scaly papules with a

distinctive hyperkeratotic ridge containing thread-like

grooves (Figure 5.41).
●● It follows Blaschko’s lines.

●● DD: Linear lichen planus, linear psoriasis, linear

VEN.

Nevus comedonicus
●● Nevus comedonicus is a rare hamartoma of the

pilosebaceous unit.
●● In most cases, it has been present since birth, but it may

also develop later.

●● It features bundles of papules and dilated follicular

openings containing epidermal residues and firm

pigmented horny plugs, similar to comedones
(Figure 5.42).
●● It typically presents a linear or zosteriform distribution,

but extensive areas have also been described.

●● The most frequently affected sites are the face, trunk,

neck, and upper limbs.

●● It is a part of nevus comedonicus syndrome, which

also consists of ocular, ostial, and neurological

defects.
●● DD: Nevus sebaceous, familial dyskeratotic
Figure 5.41  Linear porokeratosis. (Courtesy: Dr. Piyush
Kumar, Katihar, India.)
comedones.

Lobular capillary hemangioma (granuloma

pyogenicum)
●● Also known as lobular capillary hemangioma, this is

a benign vascular tumor that occurs on the skin and

mucous membranes.
●● It can arise spontaneously, in sites of injury, or within

capillary malformations.
●● It is associated with medications such as oral

contraceptives, retinoids, gefitinib, capecitabine, and

afatinib.
●● Clinical features include small or large, smooth or

lobulated, reddish exophytic vascular nodules that can Figure 5.42  Nevus comedonicus. (Dr. Anup Kumar
grow rapidly (Figure 5.43a,b).16 Tiwary, Consultant Dermatologist, Yashoda Hospital and
●● They tend to bleed profusely. Research Center, Ghaziabad, India.)
 Papules: Localized  95

Figure 5.43  (a) Lobular capillary hemangioma presenting as vascular papule with hyperkeratotic surface on lip mucosa.
(b) Infected lobular capillary hemangioma. (a – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Common sites are hands, lower lips and gingiva most

frequently affected; intraoral lesions may occur during
pregnancy.
●● DD: Cherry angioma, Kaposiform
hemangioendothelioma, hemangioma.

Angiokeratomas
●● These are ectasias of dermal capillaries with an

acanthotic and hyperkeratotic overlying epidermis.

●● Clinical features include red to brown verrucous

papules that may bleed.16

●● There are four types of localized angiokeratomas.

●● Solitary papular angiokeratoma typically occurs on the

lower extremities.
●● Localized angiokeratoma appears on the scrotum and

vulva (Fordyce type) (Figure 5.44a).

●● Angiokeratoma circumscriptum naeviforme presents as

multiple hyperkeratotic papular and plaque-like lesions,

usually unilaterally on the lower leg, foot, thigh, and
buttock (Figure 5.44b).
●● Bilateral angiokeratomas occur on the dorsa of the

fingers and toes (Mibelli).

●● DD: Verruca vulgaris, hemangioma, Spitz nevus,

pigmented basal cell carcinoma (BCC).

Bacillary angiomatosis
●● This is an infection with Bartonella henselae or B.

quintana presenting as vaso-proliferative lesions, mostly

in immunocompromised hosts, including advanced
HIV patients.
●● Clinical features include single or multiple bright-red
Figure 5.44  (a) Multiple vascular papules on scrotum in
to deep-purple, dome-shaped papules, nodules, or angiokeratoma of Fordyce. (b) Multiple hyperkeratotic
plaques. vascular papules coalescing to form plaque in angiokera-
●● Lesions may bleed profusely with trauma. toma circumscriptum naeviforme. (a – Courtesy: Dr. Piyush
●● DD: Cherry angioma, Kaposi sarcoma. Kumar, Katihar, India.)
96  Papules: Localized

Figure 5.45  (a) Multiple bright-red papules in cherry angioma. (b) Cherry angioma in a middle-aged man. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

Cherry angioma ●● Clinical features include a small violaceous papule

●● This is the most common type of acquired vascular surrounded by a pale, thin area and a peripheral
proliferation of the skin. ecchymotic ring.
●● It is also known as senile angioma or Campbell de ●● It is commonly located on the trunk or extremities.
Morgan spots. ●● DD: Hemangioma, dermatofibroma, melanoma.
●● It usually develop after the third decade; the number of

lesions increases with increasing age. Angiolymphoid hyperplasia with eosinophilia

●● Eruptive cherry angioma is seen in pregnancy, (ALHE)
malignancy, mustard gas poisoning, and ●● This is a rare, benign vascular tumor.
immunosuppression with cyclosporine. ●● It is clinically manifested by 2- to 3-cm light brown to
●● Clinical features include early lesions that appear as flat,
pink dermal papules or nodules.
red macules, resembling petechiae (Figure 5.45a,b).16 ●● Lesions occur preferentially on the face, scalp, auricular
●● As the lesions develop, they become 1- to 5-mm red
region, and neck (Figure 5.46a,b).17
papules. ●● DD: Kimura disease, pleomorphic adenoma (Figure 5.46c),
●● They are usually asymptomatic but may bleed with trauma.
pyogenic granuloma, hemangioma.
●● They most commonly occur on the trunk or proximal

extremities.
●● DD: Angiokeratoma, pyogenic granuloma.

Tufted angioma
●● This rare, benign angiomatous condition is also called

angioblastoma of Nagakawa.
●● Lesions usually arise between the ages of one and five

years; occasionally they may be present at birth.

●● Clinically characterized by erythematous papules,

plaques, and nodules, most commonly involving the

neck, upper trunk, and proximal part of extremities.
●● It may show increased sweating or growth of fine lanugo

hair.
●● DD: Kaposi sarcoma, Kaposiform

hemangioendothelioma, and low-grade angiosarcoma.

Targetoid hemosiderotic hemangioma

●● This is also known as hobnail hemangioma.

●● It presents as a vascular, benign solitary lesion of

unknown origin, probably lymphatic, affecting young Figure 5.46  (a) Erythematous papules on pinna in angio-
or middle-aged people. lymphoid hyperplasia with eosinophilia. (Continued)
 Papules: Localized  97

Figure 5.46 (Continued)  (b) Angiolymphoid hyperpla-

sia with eosinophilia. (c) Pleomorphic adenoma. A close
differential of angiolymphoid hyperplasia with eosino-
philia. (a – Courtesy: Dr. RR Madhukar, Patna, India;
c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Lymphomatoid papulosis Gottron papules

●● This is a continuous self-healing, papular ●● These are small purple/red flat papules on extensor

eruption. surfaces, particularly the elbows and joints of the hand

●● It is clinically benign and histologically (Figure 5.47).
malignant. ●● They are pathognomonic for dermatomyositis.18

●● Erythematous papules and/or nodules progress to form ●● Other features for dermatomyositis must be looked for:

esicular/crusted/hemorrhagic lesions. heliotrope rash, muscle weakness.

●● They undergo spontaneous healing with scarring. ●● DD: Psoriasis, lupus erythematosus, lichen

●● They usually occur over the trunk and limbs. planus.

●● DD: Pityriasis lichenoides et varioliformis

acuta (PLEVA), guttate psoriasis, insect

Multicentric reticulohistiocytosis
dermatitis.
●● This is a rare systemic granulomatous disorder.

●● Cutaneous features are pink/red/brown/papules the

dorsum of hand and face.

●● Papules often occur in a periungual distribution,

producing a characteristic “coral beads”

appearance.
●● Mucosa are involved in one-third of cases.

●● This is often associated with severe and rapidly

destructive arthritis.
●● DD: lichen planus, dermatomyositis.

Gianotti-crosti syndrome
●● Also known as papular acrodermatitis of childhood,

this is a rare and self-limited dermatosis.

●● The cause is herpesvirus type B, EBV.

●● Peak incidence occurs in infants between one and six

years of age.
●● Clinical features include symmetrical and multiple,

monomorphic and erythematous papular eruption with

Figure 5.47  Flat-topped, slightly erythematous papules acral distribution; the torso generally remains intact
on interphalangeal joints in dermatomyositis. (Figure 5.48a).
98  Papules: Localized

●● Systemic manifestations are unusual and include

low-grade fever, generalized lymphadenopathy,
hepatomegaly, and splenomegaly.
●● DD: insect dermatitis, scabies, papular urticaria
(Figure 5.48b).

Additional image – Figure 5.49

Figure 5.49  Bilateral, grouped, skin-colored papules

Figure 5.48  (a) Gianotti-crosti syndrome with monomor- on the scapular area in a child with Hunter syndrome.
phic papulovesicles on the extremities. (b) Papular urti- “Pebbly papules with a cobblestone appearance” pres-
caria with papulovesicles on the lower extremities. Prior ent on the upper trunk and shoulder are a characteristic
lesions have healed with hyperpigmentation and scarring. feature of Hunter syndrome. Note short neck and hyper-
(a – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India; trichosis in the child. (Courtesy: Kumar P, Das PC, Das A.
b – Courtesy: Dr. Piyush Kumar, Katihar, India.) Hunter syndrome. JAMA Dermatol 2022;158(12):1438.)
 Papules: Localized  99

REFERENCES 10. Curatolo P, Bombardieri R, Jozwiak S. Tuberous sclerosis. Lancet

2008;372(9639):657–668.
1. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: 11. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
Chapter 6. Papular Eruptions: No Scale. p. 83–96. Chapter 33. Yellow Lesions. P. 553–564.
2. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: 12. Bubna AK. Umbilicated lesions in dermatology. Clin Dermatol Rev
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. 2019;3:99–103.
Chapter 7. Scaly Papular Lesions. P. 97–112. 13. Santos JB, Figueiredo AR, Ferraz CE, Oliveira MH, Silva PG,
3. Lee HJ, Sinha AA. Cutaneous lupus erythematosus: Understanding Medeiros VL. Cutaneous tuberculosis: Epidemiologic, etio-
of clinical features, genetic basis, and pathobiology of disease pathogenic and clinical aspects - Part I. An Bras Dermatol
guides therapeutic strategies. Autoimmunity 2006;39:433–444. 2014;89(2):219–228.
4. Ely JW, Stone MS. The Generalized Rash: Part I. Differential 14. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
Diagnosis. Am Fam Physician 2010;81(6):726–734. Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier;
5. Nath AK, Sivaranjini R, Thappa DM, Basu D. Lupus miliaris dissemi- 2018. Chapter 17. Linear and Serpiginous Lesions.
nates faciei with unusual distribution of lesions. Indian J Dermatol P. 289–300.
2011;56:234–236. 15. Tiwary AK. Adult onset of inflammatory linear verrucous epi-
6. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: dermal nevus: Truly a rare experience. J Pak Assoc Dermatol
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. 2019;29(2):253–256.
Chapter 23. Follicular Disorders. P. 375–384. 16. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
7. Ramesh A, Sampath V, Kasiviswanathan P, RajanBabu D. Overview Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
of axillary dermatoses: Case series in a tertiary care institution. Int Chapter 32. Vascular Tumors. P. 535–552.
J Res Dermatol 2019;5(4):784–791. 17. Tiwary AK, Kumar P. Report of two cases of angiolymphoid
8. Tüzün Y, Wolf R. Commentary: Fold (intertriginous) dermatoses: hyperplasia with eosinophilia and review of literature. Int J Health
When skin touches skin. Clin Dermatol 2015;33(4):411–413. Sci Res 2017;7(8):503–506.
9. Tiwary AK, Mishra DK, Chaudhary SS. A rare case of familial 18. DeWane ME, Waldman R, Lu J. Dermatomyositis part I:
reactive perforating collagenosis. Indian J Paediatr Dermatol Clinical features and pathogenesis. J Am Acad Dermatol
2017;18:230–233. 2019;82(2):267–281.
 6
Facial papules

PIYUSH KUMAR, RIZWANA BARKAT

INTRODUCTION Acne vulgaris

●● Acne vulgaris (AV) is a common condition caused by
The etiopathological processes causing facial papules can follicular blockage (due to hyperkeratinization) and/
be diverse, ranging from benign (infective, inflammatory, or inflammation of pilosebaceous units. Acne can
or neoplastic) to malignant conditions. The list of clini- present as non-inflammatory lesions (comedones),
cal differentials of facial papules can be overwhelming, inflammatory lesions (papules, pustules, and nodules),
but a careful consideration of the number of lesions, age or a mixture of both.
of presentation, relative distribution on the face, presence ●● AV is most commonly seen in adolescents and
or absence of other lesions and scars, and clinical charac- less commonly in adults. Of note, AV shows male
teristics of the individual papule often helps in arriving at predilection in adolescent population and female
a clinical diagnosis in majority of cases (Figures 6.1 and preponderance in adult population. Female patients
6.2, Tables 6.1 and 6.2). This chapter discusses a clinical may have features of polycystic ovarian syndrome
approach to the diagnosis of facial papules, followed by a (PCOS).
brief description of some important conditions presenting ●● AV is most common on the face but affects the back
as papules on the face.1–4 and chest too. Lesions on the face are most severe

Multiple facial
papules

Predominantly
affected site

Lateral face/ Lower face/ Bilaterally

Centrofacial Upper face Periorificial Periauricular Nose
cheeks Beard area symmetrical

AV Cylindroma Periorificial ALHE Sycosis barbae

Trichostasis Leprosy
AD Red/Red-brown Flesh-colored Darier disease dermatitis Milia en Tinea barbae
spinulosa Granuloma
EFF Sebaceous Syringoma plaque
Pseudofollicu annulare
hyperplasia Milia* Chloracne litis
Secondary
DPN Traumatic syphilis
Rosacea anserine
Hydrocystoma Cryptococcosis
Angiofibroma folliculosis
Trichoepithelioma Papular scar Histoplasmosis
Demodicosis Steroid acne
LMDF
LMDF
Acneiform FM
Sarcoidosis
PKDL

Figure 6.1  Clinical approach to multiple facial papules (AV – acne vulgaris, AD – atopic dermatitis, EFF – erythromelanosis
follicularis faciei, LMDF – lupus miliaris disseminatus faciei, DPN – dermatosis papulosa nigra, FM – follicular mucinosis,
PKDL – post-kala azar dermal leishmaniasis, ALHE – angiolymphoid hyperplasia with eosinophilia).

100 DOI: 10.1201/9781351054225-12

 Facial papules  101

Facial papules

Usually single
Multiple
(or few)
(Figure 6.1)

Flesh-colored/white Erythematous Pigmented Verrucous

Intradermal nevus LCH Melanocytic nevus

Fibrous papule of nose Infantile Pigmented BCC Wart
BCC hemangioma Eccrine
Pilomatricoma Keratoacanthoma hydrocystoma
Epidermoid cyst Cutaneous Dilated pore of
Apocrine hydrocystoma leishmaniasis winer
Hydrocystoma Granuloma faciale
Trichofolliculoma

Figure 6.2  Clinical approach to solitary facial papule (BCC – basal cell carcinoma, LCH – langerhans cell histiocytosis).

on the cheeks. However, in women with PCOS,

Table 6.1  Facial papules and usual age of
perioral, or jawline, areas are affected with increased
presentation
frequency.
●● The disease is clinically characterized by non- Age of
inflammatory and skin lesions, mainly in the sebum- presentation Diseases
rich areas, such as face, back and chest. There may be
Infancy/ early Infantile hemangioma, neonatal
associated soreness, itching, and pain.
childhood cephalic pustulosis, common
●● Comedones – These are the pathognomonic lesions
acquired melanocytic nevus (CAMN),
of AV and may be a whitehead (closed comedone)
childhood granulomatous periorificial
(Figure 6.3a,b) or a blackhead (open comedone)
dermatitis (CGPD), Darier disease,
(Figure 6.3c) without any clinical signs of
keratosis follicularis spinulosa
inflammation.
decalvans (KFSD), inherited
●● Inflammatory lesions are characterized by
cylindroma, lipoid proteinosis (with
erythematous papules and pustules (Figure 6.3d,e).
vesicobullous lesions)
In severe cases, nodules and cysts too may be seen
Adolescence Acne vulgaris, verruca plana,
(Figure 6.3f,g).
trichoepithelioma, angiofibroma,
●● Post-inflammatory hyperpigmentation and atrophic or
neurofibromas, traumatic anserine
hypertrophic scars are the sequelae of acne.
folliculosis, erythromelanosis
●● Neonatal cephalic pustulosis – acne lesions may be
follicularis faciei, dermatosis
present in the first few weeks of life too (because
papulosa nigra
of maternal hormones) and tend to resolve
Adults/ • Solitary – basal cell carcinoma (BCC),
spontaneously.
elderly solitary cylindroma, hydrocystoma,
●● DD:
pilomatricoma
●● For inflammatory lesions – seborrheic dermatitis
• Multiple – sebecous hyperplasia,
(yellowish greasy scales), rosacea (photosensitivity,
rosacea, demodicosis, trichostasis
telangiectasia, central face affection), demodicosis,
spinulosa, lymphoma-associated FM
bacterial folliculitis (Figure 6.3h).
102  Facial papules

Table 6.2  Clinical characteristics of facial papules

Clinical features Diseases

Waxy papules • Solitary – BCC, colloid milium,
keratoacanthoma
• Multiple – trichoepithelioma,
lipoid proteinosis
Translucent Hidrocystoma, colloid milium,
papules papular sarcoidosis, lupus
miliaris disseminatus faciei
(LMDF)
Umbilicated Molluscum contagiosum (MC),
papules sebaceous hyperplasia,
cryptococcosis, histoplasmosis
Ulcerated /crusted Atopic dermatitis, BCC, lobular
papules capillary hemangioma,
keratoacanthoma (central
keratotic plug), cryptococcosis,
histoplasmosis, cutaneous
leishmaniasis
Verrucous/ • Linearly arranged – verrucous
keratotic epidermal nevus, nevus
papules sebaceous (in post-pubertal
life), warts
• Randomly arranged –
seborrheic keratosis, Darier
disease, keratosis follicularis
spinulosa decalvans
Linearly arranged Molluscum contagiosum, verruca
papules plana and common wart,
verrucous epidermal nevus
Papulopustular Acne vulgaris, rosacea, sycosis
lesions barbae, pseudofolliculitis
barbae, tinea barbae, tinea
faciei, periorificial dermatitis,
demodicosis, secondary
syphilis, cryptococcosis,
histoplasmosis, chloracne,
acneiform follicular mucinosis
Expressed • White solid material – MC
contents • Cheesy, smelly, semisolid
material – epidermoid cyst
• Oily material – steatocystoma
multiplex
• Gelatinous – colloid milium
Papules healing Acne vulgaris, LMDF, sycosis
with scarring barbae, tinea barbe,
keratoacanthoma, infantile
hemangioma, lipoid
proteinosis, KFSD, chloracne,
cutaneous leishmaniasis

●● For comedones – syringoma (skin-colored or

lightly pigmented angulated papules), sebaceous Figure 6.3  (a) Closed comedones seen as discrete, skin-
hyperplasia (yellowish papules in elderly), colored papules on the face. Few isolated inflammatory
trichoepithelioma, steatocystoma multiplex, milia, papules and papulopustules are seen. (b) Closed comedo-
molluscum contagiosum (Figure 6.3i). nes. (Continued)
 Facial papules  103

Figure 6.3 (Continued)  (c) Open comedones seen as skin-colored papules with dilated follicular opening and central
pigmented, keratotic material. (d) Erythematous papules and pustules in acne vulgaris. (e) In severe cases, lesions tend to
become confluent. (f) Soft nodules and cystic in a young girl. (Continued)

Atopic dermatitis ●● DD: Scabies, seborrheic dermatitis, contact

●● Facial atopic dermatitis is usually seen in babies and dermatitis.
children. The cheeks and forehead are often affected.
●● Skin is dry, red, itchy, and irritable. Patients may Erythromelanosis follicularis faciei (EFF)
have erythematous papules and plaques, or vesicular, ●● EFF is an under-recognized condition of unknown

oozing, crusted lesions (Figure 6.4). Other body parts etiology, characterized by a triad of follicular papules,
are often affected. well-demarcated erythema, and hyperpigmentation.
104  Facial papules

Figure 6.3 (Continued)  (g) Nodulocystic lesions on face. Note presence of open comedones and scarring. (h) Erythematous
papules, pustules, and nodules of bacterial folliculitis. (i) This particular patient has both acne vulgaris – mid face pustule
(orange arrow), open comedones (yellow arrow), post-acne erythema (black arrow) – and molluscum contagiosum (lower
face, blue arrow). (i – Courtesy: Dr. PC Das, Katihar, India.)

●● The condition is mostly seen in adolescents of ●● Middle-aged women with fair skin, blue eyes, and
Asian ancestry and typically affects lateral face and blonde hair are more commonly affected.
preauricular areas. When it affects the neck too, it ●● Various triggers, such as sunlight, heat, cold,
is called erythromelanosis follicularis faciei et colli wind, spicy food, alcohol, and stress, aggravate the
(EFFC). disease.
●● Patients present with grouped, tiny follicular papules ●● The condition is characterized by varying degrees
over a background of well-defined erythema and of erythema, flushing and telangiectasia, and
hyperpigmentation (Figure 6.5). Telangiectasia too may inflammatory lesions, and is divided into four subtypes.
be observed at times. In many patients, different types may coexist, and one
●● Patients may have associated keratosis pilaris over type may progress another type.
extremities. ●● Erythematotelangiectatic rosacea – It is the earliest
●● DD: Poikiloderma of Civatte, Riehl’s melanosis, and change in rosacea and is initially transient, later
pigmented peribuccal erythrosis of Brocq. becoming persistent. Clinically it is characterized
by flushing and persistent central facial erythema
Rosacea (sparing the periocular area) and is accompanied
●● Rosacea is a chronic, idiopathic, inflammatory facial by a burning or stinging sensation. At times,
condition showing periodic remission and relapses. the surface may be scaly because of low-grade
 Facial papules  105

dermatitis. Patients often complain of exacerbation

of a burning or stinging sensation when topical
agents are applied.
●● Papulopustular rosacea – This stage is
characterized by persistent erythema (> 3 months),
and erythematous papules and pustules in the
central area of face are associated with burning and
stinging (Figure 6.6).
●● Phymatous rosacea – This is an end stage
of the disease, seen in patients with long-
standing disease, and is clinically characterized
by thickened skin with irregular nodular
surface. The condition usually affects the nose
(rhinophyma), chin (gnathophyma), forehead
(metophyma), cheeks, and ears. One particular
patient may or may not develop phymatous
changes of all sites.
●● Ocular rosacea – Ocular rosacea may precede
or may develop concurrently with skin lesions.
Patients with ocular rosacea complain of eye
stinging or burning, dryness, irritation with
light, or foreign body sensations. Ocular findings
include blepharitis, conjunctivitis, inflammation

Figure 6.4  Erythematous scaly papules on the face in

atopic dermatitis.

Figure 6.5  Erythromelanosis follicularis faciei presenting Figure 6.6  Persistent erythema with erythematous pap-
as multiple tiny follicular papules over a background of ules and pustules over the bilateral cheek, nose, and fore-
well-defined erythema and hyperpigmentation present head. (Courtesy: Dr. Hiral Shah, Baroda Medical College,
over the right cheek. Vadodara, India.)
106  Facial papules

of the lids and meibomian glands, interpalpebral angiofibromas are also seen in multiple endocrine
conjunctival hyperemia, and conjunctival neoplasia type 1 (MEN-1) and Birt-Hogg-Dubé
telangiectasias. syndrome.
●● DD: Acne vulgaris. ●● Facial angiofibromas appear in childhood, increase in
number and size until adolescence, and then become
Facial angiofibroma stable.
●● Cutaneous angiofibroma refers to a group of clinically ●● Clinically, it manifests as multiple symmetrical,
different lesions with the same histologic findings – reddish-brown, dome-shaped papules with smooth
proliferation of stellate and spindled cells, thin-walled surface occurring over the central face, which often
blood vessels, and concentric collagen bundles. The concentrates around alar grooves, nose, cheeks, nasal
clinical presentations include facial angiofibromas, fibrous opening, and chin with relative sparing of upper lip and
papule of nose, pearly penile papules, and Koenen tumors. lateral face (Figure 6.7a,b). The lesions may coalesce to
●● Facial angiofibromas, previously known as adenoma form plaques (Figure 6.7c).
sebaceum (a misnomer), are cutaneous hallmarks ●● A few cases of unilateral, nevoid facial angiofibroma
of tuberous sclerosis complex (TSC). Multiple facial have also been reported (Figure 6.7d).5

Figure 6.7  (a) Facial angiofibroma seen as multiple dome-shaped, red-brown papules on the cheeks and nose. (b) Numerous
facial angiofibromas in a young lady. (c) Plaques of facial angiofibroma on the forehead. (d) Unilateral facial angiofibroma.
 Facial papules  107

●● DD: Verruca plana (pseudo koebnerization, randomly

distributed), rosacea, acne vulgaris.

Demodicosis6
●● Demodicosis is a cutaneous infestation caused by

Demodex folliculorum and Demodex brevis (collectively

known as Demodex), a saprophytic mite that is a
commensal organism of pilosebaceous units. D.
folliculorum infestation is more common and is usually
localized to the face, while D. brevis is found on the
neck and chest.
●● It can occur as a primary disease or may develop

secondary to immunosuppression or topical

glucocorticoids/calcineurin inhibitors use.
●● Primary demodicosis presents in middle-aged to

elderly populations and has facial involvement,

typically affecting periorificial areas. On the other
hand, secondary demodicosis has an early onset and
is characterized by more diffuse facial distribution or
truncal involvement.
●● Primary demodicosis may have one of these

presentations:
●● Pityriasis folliculorum-like (spinulate demodicosis)
– Patients develop discrete fine, whitish or
yellowish, spiky changes involving sebaceous hair
follicles (Figure 6.8a). It may have associated faint
erythema and little inflammation.
●● Rosacea-like (rosaceiform; papulopustular
demodicosis) – Patients have papulopustular lesions
in perioral, periorbital, and periauricular regions
(Figure 6.8b).
●● Demodicosis gravis (nodulocystic demodicosis) –
Patients develop nodular lesions, pus formation,
and/or abscess-like lesions.
●● DD: Acne vulgaris (comedones, not

periorificial localization), seborrheic dermatitis

(yellow greasy scales), rosacea (photosensitivity,
telangiectasia).

Lupus miliaris disseminatus faciei (LMDF) (Facial

idiopathic granulomas with regressive evolution
[FIGURE])
●● LMDF is an uncommon, benign, granulomatous,

inflammatory skin disease of unknown etiology

affecting mainly the central area of face showing a
characteristic tendency to involve the eyelids.
●● The exact prevalence is not known, but the disease may

be more common in Asians.

●● Both the sexes can be affected with LMDF; however,

young males in their twenties are predominantly

affected.
●● The skin lesions are multiple, monomorphic, smooth,

discrete, reddish-brown or brown to yellowish

Figure 6.8  (a) Demodicosis presenting as erythema and
dome-shaped translucent papules, nodules, and tiny whitish projections from the follicles. (b) Multiple ery-
pustules which are symmetrically distributed in the thematous papules present over the cheek and forehead
central area of the face (lower area of forehead, lower in demodicosis. (a,b – Courtesy: Dr. Shekhar Neema,
portions of eyelid, nasolabial folds, cheeks, and the Armed Forces Medical College Pune, India.)
108  Facial papules

Figure 6.9  Lupus miliaris disseminatus faciei seen as mul-

tiple, monomorphic, discrete, reddish dome-shaped translu-
cent papules on the face. Note involvement of lower eyelids.

perioral areas) (Figure 6.9). Occasionally other sites,

such as axillae, chest, neck, arms, hand, legs, and
groin, can also be involved. The condition resolves
spontaneously over a few months to years, leaving
behind characteristic pock-like scars.
●● DD: Acne vulgari, rosacea, secondary syphilis.

Trichoepithelioma
●● Trichoepithelioma is a benign hamartomatous tumor

of pilosebaceous follicle usually occurring in childhood

or early adolescence as solitary or multiple types.
Multiple trichoepithelioma is usually transmitted as an
autosomal dominant trait.
●● Females are more commonly affected than males.

●● The lesion mainly occurs on the central face – nasolabial

folds, nose, upper lip, forehead, and eyelids. It may

occasionally involve the scalp, neck, and upper trunk.
●● The lesions present as skin-colored to pink, firm,

rounded, shiny, translucent, well-demarcated

papulonodular lesions with slightly depressed center
(Figure 6.10a,b). Rarely, multiple lesions may coalesce to
form large lesions, giving an appearance of leonine facies.
●● Rarely, dermatomal distribution may be noted.
●● Syndromes associated are Brooke-Spiegler syndrome,
Figure 6.10  (a) Trichoepithelioma seen as multiple skin-
Rombo syndrome, and Bazex syndrome. colored, round, shiny, translucent papules with a slightly
●● DD: BCC (usually solitary, hyperpigmented lesion,
depressed center over the central face. (b) Grouped
mainly confined to sun-exposed area), closed yellowish papulonodular lesions of trichoepithelioma on
comedones, steatocystoma multiplex. central face. (b – Courtesy: Dr. PC Das, Katihar, India.)
 Facial papules  109

Cylindroma ●● DD: Keloid, pleomorphic adenoma, trichilemmal cyst

●● Cylindroma is a benign skin-appendageal tumor, (pilar cyst), trichoepithelioma.
originating from the sweat glands. It can be solitary
Darier disease (Keratosis follicularis, Darier-White
(sporadic) or multiple (autosomal dominant inheritance).
●● Solitary cylindroma is usually seen in middle-aged and
disease)
●● Darier disease is a rare autosomal dominant
elderly individuals; inherited cylindroma has its onset in
childhood. The tumor is more prevalent among females. genodermatosis that occurs as a result of mutation in
●● The lesions are mostly seen on head and neck region. the ATP2A2 gene.
●● The onset of disease is usually in childhood or
●● The patient presents with asymptomatic, solitary or

multiple, firm, rubbery, smooth, pink to red hairless adolescence, with no sexual predilection.
●● There is a history of disease aggravation by heat, sweat,
papules, nodules, and/or tumors. Multiple lesions over
scalp can cover the entire scalp giving an appearance of humidity, sunlight, UVB exposure, mechanical trauma,
disfiguring turban, hence named “turban tumor.” and pregnancy.
●● Clinically, the lesions are symmetrically distributed
●● Rarely, there may be a malignant transformation to

cylindrocarcinoma. hyperkeratotic, greasy papules that coalesce to form

●● Brooke-Spiegler syndrome is an autosomal dominant irregular warty plaque or papillomatous masses mainly
syndrome with multiple skin-adnexal neoplasms, involving the seborrheic areas and intertriginous areas
including cylindroma, trichoepitheliomas, and rarely (Figure 6.12a,b). The lesions may become malodorous,
spiradenomas (Figure 6.11). with painful fissures.
●● Zosteriform, or linear vesiculobullous, isolated acral

hemorrhagic, acrokeratosis verruciformis of Hopf,

comedonal and hypopigmented/leukodermic macules
are its rare morphological variants.
●● Pitting or punctate keratosis may be seen on palms and

soles.
●● The oral lesions are seen in 50% of cases as

asymptomatic, multiple white or red firm papules

that may coalesce, giving an appearance of
“cobblestones,” and affect primarily the alveolar and
buccal mucosa.
●● Associated nail findings include nail fragility, red and

white longitudinal bands, and V-shaped notches at the

free margins of the nails.
●● Neuropsychiatric abnormalities include mild mental

retardation and epilepsy.

●● DD: Seborrheic dermatitis, acne vulgaris, confluent and

reticulated papillomatosis.

Figure 6.11  A lady with Brooke-Spiegler syndrome.

Cylindromas are seen as erythematous papulonodular lesions
(red arrow) on the lower face and forehead, and trichoepi-
theliomas are seen as flesh-colored papulonodular lesions
(black arrow) on the central face, in perinasal area. (Courtesy: Figure 6.12  (a) Dirty-looking, hyperkeratotic papules on
Dr. Hiral Shah, Baroda Medical College, Vadodara, India.) the face in Darier disease. (Continued)
110  Facial papules

Figure 6.12 (Continued)  (b) Lesions tend to get conflu-

ent to form plaques in summer. Also, the surface may get
macerated.

Sebaceous hyperplasia
●● Sebaceous gland hyperplasia (SH) is a benign

hamartomatous condition characterized by presence of

≥4 sebaceous lobules attached to the infundibulum of
each pilosebaceous unit.
●● It starts appearing by middle age and increases in

number with age. Familial cases may have an earlier

onset and widespread lesions.
●● Most commonly, it affects the face (forehead, cheeks,

and nose), but occasionally it may involve chest,

areola, mouth, scrotum, foreskin, shaft of penis,
and vulva. The lesion consists of single or multiple,
asymptomatic, small, yellowish papules with a central
dell (Figure 6.13a,b). Surface may show telangiectasia.
●● Variants include giant form, linear or zosteriform

arrangement, diffuse form, familial form.

●● Juxtaclavicular beaded lines are another unique

clinical presentation of SH and present as closely placed,

tiny, papules arranged in parallel rows on the neck,
around the clavicle.
●● DD: Epidermoid cyst, vellus hair cyst, steatocystoma

multiplex, basal cell carcinoma.

Figure 6.13  (a) Multiple small, yellow papules on the face
Periorificial dermatitis (perioral dermatitis) in an elderly lady with sebaceous hyperplasia. (b) Multiple
●● Perioral dermatitis (“air hostess” dermatitis) is a chronic small, skin-colored papules of sebaceous hyperplasia on
eczematous facial dermatitis of an unknown etiology, the face.
 Facial papules  111

Figure 6.14  (a) Papulopustules of periorificial dermatitis on an erythematous base over the perioral area, nose, bilateral
cheek, and chin, with sparing of vermilion border of lips. (b) Periorifical dermatitis presenting as closely set erythematous
papules around the lips. (b – Courtesy: Dr Esther Nimisha, Jamshedpur, India.)

typically affecting the skin around the eyes, the nostrils, erythema and scaling. CGPD may persist for months
and the mouth. The disease runs a chronic course. and then resolve spontaneously without any sequelae.
●● It is common in children and in women aged ●● DD: Rosacea, seborrheic dermatitis, atopic dermatitis,
20–45 years. steroid induced rosacea (periorificial dermatitis spares
●● Prior history of use of topical application or inhalation cheeks, and forehead).
of corticosteroids, fluorinated toothpaste, paraffin-based
skin care ointments and cosmetics, physical sunscreens, Syringoma
and sunlight exposure may be observed. ●● Syringoma is a benign adnexal tumor originating from

●● The characteristic lesions are monomorphic, dome- intraepidermal eccrine duct.

shaped, skin-colored to yellowish-brown papules, ●● The disease usually occurs during puberty or

papulovesicles, and papulopustules on an erythematous during third and fourth decade of life, with female
base with or without scales affecting the perioral area, preponderance.
periorbital area, nasolabial folds, and chin. When the ●● There may be a history of summer aggravation.

perioral area is involved, classical sparing of vermilion ●● Lesions typically involve the periorbital area. Other

border of lips is noted (Figure 6.14a,b). The condition is commonly involved sites are axillae, chest, abdomen,
associated with a burning or stinging sensation and rarely penis, and vulva.
pruritus. Patients may develop unilateral lesions too. ●● The lesions are characteristically seen as multiple,

●● Extra-facial sites, such as neck, trunk, extremities, and small, ill-defined to well-defined, angulated, smooth-
genital areas, have also been reported. surfaced, skin-colored, yellowish, or brownish
●● It usually heals without scarring, though in some cases papules (Figure 6.15a,b). The surface may be flat
atrophic pits and scarring are noted. or rounded. The lesions are usually bilaterally
●● Childhood granulomatous periorificial dermatitis symmetrical.
(CGPD)/facial Afro-Caribbean childhood eruption ●● The eruptions are usually asymptomatic, although

(FACE) is the granulomatous variant that presents with pruritus has been reported in some cases.
flesh-colored to erythematous to yellow-brown papules ●● Four major clinical variants are

and plaques in the same distribution. It typically affects ●● Localized

prepubertal children and is more common in males ●● Generalized
and possibly in colored skin. It differs from periorificial ●● Down syndrome associated
dermatitis by absence of pustules and absence of ●● Familial form
112  Facial papules

Figure 6.15  (a) Syringoma seen as multiple aggregated, small, brownish papules present over the periorbital areas.
(b) Pigmented lesions of syringoma in periorbital area. (a – Courtesy: Dr. Rashid Shahid, Katihar Medical College, Katihar.)

●● Morphologically, uncommon variants include lichen dome-shaped papules measuring 1–2 mm in diameter
planus-like, milium-like, and plaque-like syringoma. (Figure 6.16a).
●● Associated diseases include ●● Congenital milia are common on face (especially nose)
●● Down syndrome and scalp and show spontaneous resolution within a
●● Diabetes mellitus couple of weeks (Figure 6.16b).
●● Ehlers-Danlos syndrome ●● Primary milia of children and adults favor cheeks and
●● Marfan syndrome eyelids, and tend to persist.
●● Hyperthyroidism. ●● Secondary milia arise as a cutaneous reaction to
●● Nicolau-Balus syndrome – syringoma in association traumatic stimuli and are localized to the involved body
with milia and atrophoderma vermiculata site.
●● Brooke-Spiegler syndrome ●● Milia en plaque is a rare variant of primary milia,
●● DD: Verruca plana (pseudo Koebnerization can be seen), characterized by numerous tiny milia within an
eruptive xanthoma (yellowish lesions), lichen planus erythematous base, arising spontaneously on a healthy
(pruritic, violaceous lesions, often showing Koebnerization). skin. It commonly affects the head and neck region,
especially periauricular and periorbital areas and on the
Milia nasal bridge.
●● Milia (singular: milium) are small, benign, keratin-filled ●● Multiple eruptive milia refer to the appearance of
cysts and may be primary with spontaneous onset or numerous milia over weeks to months. The lesions affect
secondary appearing after trauma, some inflammatory head and neck, upper trunk, and upper extremities.
skin diseases, or use of certain topical or systemic drugs. ●● Genodermatoses associated with extensive milia include
Primary milia are believed to originate from obstruction Bazex-Dupre-Christol syndrome, Rombo syndrome,
of vellus hair (lower infundibulum), while secondary Brooke-Spiegler syndrome, pachyonychia congenita
milia are derived from eccrine sweat ducts mostly. type II and basal cell nevus syndrome.
●● Milia are seen in all age groups; even congenital milia ●● Drugs precipitating development of milia include
are known. topical corticosteroid, fluorouracil, penicillamine,
●● The lesions are clinically seen as asymptomatic, single cyclosporine, dovitinib, sorafenib, benoxaprofen, and
or multiple, randomly arranged, discrete, pearly white, acitretin.
 Facial papules  113

●● Women are more frequently affected. There is often a

family history of DPN.
●● DPN presents as multiple, asymptomatic, small (1–5
mm), dome-shaped, smooth-surfaced, black papules
over sun-exposed areas, including the face. Lesions
on the face are symmetrically distributed over malar
areas, temples, and forehead (Figure 6.17a,b). The size

Figure 6.16  (a) Milia seen as multiple, discrete, pearly

white, dome-shaped papules present over periocular
areas. (b) Numerous pearly white papules of congenital
milia on the face more prominent over the chin.

●● DD: Molluscum contagiosum (central umbilication),

vellous hair cyst (flesh-colored papule), miliary
calcinosis cutis.

Dermatosis papulosa nigra (DPN)

●● DPN, a clinical variant of seborrheic keratosis (SK), is
Figure 6.17  (a) Dermatosis papulosa nigra seen as mul-
a benign epidermal growth, common in Asians and
tiple dome-shaped, hyperpigmented papules randomly
Africans. distributed over the face. (b) Dermatosis papulosa nigra
●● In contrast to SK, DPN has its onset in adolescence and
as pigmented dome-shaped papules. Patient has junc-
the number of lesions peaks in sixth decade. tional and compound melanocytic nevi too.
114  Facial papules

and number of papules usually increase over time. The Table 6.3  Apocrine and eccrine hidrocystoma
lesions do not resolve spontaneously.
Apocrine Eccrine
●● DD: Verruca plana, syringoma, angiofibroma.
Features hidrocystoma hidrocystoma
Hidrocystoma Number Single (rarely, Single (Smith and
●● Hidrocystoma are benign cystic dilatations of the glandular multiple) Chernosky type)
or ductal part of sweat glands and are classified into or multiple
two types depending on the origin – apocrine (cystic (Robinson type)
proliferation of apocrine glands) and eccrine (cystic Size Large (3–15 mm) Small (1–6 mm)
dilatation of intradermal sweat ducts) types. The eccrine Color Flesh-colored to Dark-blue tint to
type is further classified into two groups – the Smith and brown and black
Chernosky type (solitary) and the Robinson type (multiple). light-blue tint
●● Hidrocystomas are mostly seen in middle-aged to
Typical site Eyelid margin, Periorbital area
elderly persons. inner canthus
●● Hidrocystomas present as dome-shaped, translucent,
(cysts of Moll’s
smooth-surfaced lesions in the head and neck region glands)
(Figure 6.18). The clinical features of eccrine and Other sites Chest, shoulder, Ear, trunk, scalp,
apocrine hidrocystoma are summarized in Table 6.3. axilla, umbilicus, and upper limbs
●● Eccrine hidrocystoma typically appears in the periorbital
foreskin, penal
area, but does not affect eyelid margin. Apocrine shaft, vulva, and
hidrocystoma is mainly seen on the eyelid margin near fingers
the inner canthus rather than periorbital area. Gender None Females (specially
●● Multiple apocrine hidrocystomas have been described
predilection Robinson type)
in Goltz-Gorlin syndrome and Schopf-Schultz-Passarge Summer No Yes
syndrome. aggravation May disappear
●● DD: Intradermal nevus, epidermoid cyst, appendageal
completely in
tumor, basal cell carcinoma (for solitary lesion) cooler weather
and vellus hair cysts, Favre-Racouchot syndrome,
syringoma, fibrofolliculoma (for multiple lesions).
Papular scar
●● Scarring is one of the persistent sequelae of various

inflammatory processes, and it assumes a different

morphology.
●● Papular scars on face present as asymptomatic multiple

non-scaly, skin-colored, soft, small fibrous papules,

distributed typically over the nasal skin and the chin
(Figure 6.19).

Figure 6.19  Multiple skin-colored papular scars inter-

Figure 6.18  Single, skin-colored cyst of hidrocystoma spersed with a few erythematous papules on the chin in
present over the left upper eyelid. gnathophyma.
 Facial papules  115

●● Papular scars usually follow acne vulgaris and rosacea.7 ●● Lymphoma-associated FM is typically seen in the older
Other types of scars may be associated. population and does not resolve spontaneously. Lesions
●● DD: Trichoepithelioma, open comedones. are often multiple, spread over the trunk and extremities.

Follicular mucinosis (FM) Sarcoidosis

●● Follicular mucinosis is characterized by mucinous ●● Sarcoidosis is a multisystem granulomatous disease of

degeneration of follicles on histopathology and an unknown etiology characterized by non-caseating

clinically presents as follicular papules, alopecia epithelioid cell granuloma with few or no inflammatory
of hairy areas, and/or erythematous infiltrated cells (“naked granuloma”). It commonly affects the
plaques. lungs, lymph nodes, liver, eyes, bones, and skin.
●● FM may present as a benign primary disorder or ●● The disease occurs more commonly in second to fourth

be associated with malignant lymphoproliferative decades of life, though it can be seen in any age group
processes such as cutaneous T- and B-cell lymphomas. showing female predominance.
●● Primary FM is seen in the younger population and ●● Papular sarcoidosis is common on the face but may

shows spontaneous resolution. The most common affect any site. It clinically presents as multiple scattered
presentation is a solitary (or a few) papule in the head or confluent, indurated papules of various colors,
and neck region. including red, reddish-brown, violaceous, translucent,
●● Acneiform FM has been described as a rare variant of or hyperpigmented (Figure 6.21). Most lesions exhibit
primary FM that presents with erythematous-to-skin- little or no surface changes.
colored papules on the face (Figure 6.20). The disease ●● Lupus pernio – Patients present with chronic, indurated

runs a chronic course, and the lesions may persist for papules or plaques that affect the mid-face, particularly
months to years.8 the alar rim of the nose and are more likely to have
●● DD: Perioral dermatitis (reddish papules, vesicles, more severe systemic involvement.
and pustules on an erythematous base with a ●● Associated erythema nodosum and alopecia may be

tendency to coalesce), granulomatous periorificial seen.

dermatitis (yellow-to-brown papular lesions over ●● Diagnosed cutaneous sarcoidosis requires a work-up for

perioral, perinasal and periocular regions, common systemic involvement, followed by periodic monitoring.
in children), sarcoidosis. ●● DD: Leprosy, PKDL, LMDF.

Figure 6.21  Red-brown papules and plaques of sarcoid-

Figure 6.20  Acneiform follicular mucinosis. osis on the face.
116  Facial papules

Post-Kala azar dermal leishmaniasis (PKDL) ●● Lesions progress to involve the trunk and upper
●● PKDL presents as hypopigmented macules and extremities.
erythematous, juicy papules, first appearing on the face, ●● DD: Lepromatous leprosy, sarcoidosis.
especially the muzzle area of the face. Over time, lesions
coalesce with each other and grow in size to form Angiolymphoid hyperplasia with eosinophilia (ALHE)
papulonodular lesions (Figure 6.22a,b). ●● ALHE is a rare, benign, idiopathic skin tumor

characterized by vascular proliferation and

eosinophil invasion. The condition appears to be
common in Asians and is uncommon in colored skin.
●● The condition usually affects young to middle-aged

adults, with a slight female predominance.

●● The classical site of affection is the preauricular region.

The lesion is mostly seen in the head and neck region

(forehead or scalp) but may affect hands, shoulders,
breasts, penis, oral mucosa, and the scrotum too.
●● It presents with solitary or multiple, flesh-colored

to erythematous or hyperpigmented, dome-shaped,

smooth-surfaced, grouped dermal papules or nodules
clustered on a normal-appearing skin. The condition is
usually asymptomatic but can be associated with pain or
pruritus in a few cases. Uncommon symptoms include
bleeding, pulsation, ulceration, and lymphadenopathy.
●● DD: Kimura’s disease (the lesions are broad subcutaneous

masses and is more deep), eruptive lobular capillary

hemangioma (pyogenic granuloma), bacillary angiomatosis.

Chloracne (metabolizing acquired dioxin-induced

skin hamartomas/MADISH)
●● Chloracne is a systemic toxic disease caused by

exposure to halogenated aromatic hydrocarbons either

by inhalation, ingestion, or direct contact, affecting the
skin and internal organs such as eyes, central nervous
system, and liver.
●● Chloracnegens include polychlorinated naphthalenes,

polychlorinated biphenyls, polychlorinated phenols, etc.,

and are commonly found in fungicides, insecticides,
herbicides, and wood preservatives.
●● The lesions usually affect the cheeks, retroauricular

areas, axillae, and groin.

●● The disease starts clinically as erythema and edema

of the affected area followed by formation of fine

comedones involving almost every follicle giving a slate-
gray pigmentation. Soon, lesions progress to open and
closed comedones, nodules, and cysts. Lesions heal with
scarring (Figure 6.23a,b).
●● Additional findings include hyperpigmentation of nails,

hypertrichosis, hyperhidrosis, and porphyria cutanea tarda.

●● Systemic features are anemia, fatigue, anorexia,

arthritis, peripheral neuropathy, impotence,

hyperlipidemia, and ophthalmopathy.
●● DD: Steroid acne, acne vulgaris.

Figure 6.22  (a) Post-kala azar dermal leishmaniasis pre-

Trichostasis spinulosa
senting as multiple confluent, shiny, erythematous pap-
●● TS results from hyperkeratosis of the infundibulum
ules present over the muzzle area of the face. (b) Post-kala
azar dermal leishmaniasis presenting as multiple conflu- and subsequent retention of numerous vellus hairs
ent, shiny, erythematous papules over the chin. surrounded by a keratinous sheath, in a dilated follicle.
 Facial papules  117

Figure 6.24  Multiple black papules on nose in trichostasis

spinulosa.

●● The clinical lesions are itchy or painful, erythematous,

grouped, follicular papules and pustules appearing
predominantly on the upper lip and below the angles of
the mouth (Figure 6.25a,b). If left untreated, the lesions
may progress to abscess formations and may leave scars
on healing. Recurrence is common.
●● DD: Tinea barbae, acne vulgaris.
Tinea barbae
●● Tinea barbae is dermatophytic infection of the beard

and moustache areas of the face and generally affects

only adult men.
●● It clinically presents as follicular erythematous papules,

nodules, pustules, and crusting around the hairs

Figure 6.23  (a) Chloracne presenting as numerous closed

and a few open comedones, erythematous nodules with
slate-gray pigmentation present over the face and ear.
(b) Chloracne with erythematous nodules. (a,b – Courtesy:
Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)

●● Elderly males are mostly affected, and the nose and

interscapular areas are most commonly affected sites.
●● The patients present with typical 1-mm or smaller, black
follicular papules or plugs. The lesions may have protruding
tufts of fine hairs, which may not be visible to naked eye and
may require a hand lens or dermatoscope for visualization.
These tufts of hairs can be easily removed without causing
any discomfort to the patient (Figure 6.24).
●● DD: Open comedones, keratosis pilaris.

Sycosis barbae
●● Sycosis barbae is a bacterial infection of the hair follicle of

the beard area, usually caused by Staphylococcus aureus. Figure 6.25  (a) Sycosis barbae seen as multiple grouped
●● The disease affects the men who shave irregularly. Those
erythematous papules and pustules over an erythema-
who shave daily or not at all rarely experience the disease. tous nodular base present over the upper lip and below
●● It occurs more commonly in males 20–40 years of age. angles of mouth. Note satellite pustules. (Continued)
118  Facial papules

Figure 6.25 (Continued)  (b) Severe cases may show

involvement of other hair-bearing areas. Note scarring Figure 6.26  Pseudofolliculitis barbae seen as multiple firm
and alopecia from previous lesions. hyperpigmented papules present over the moustache area.

(kerion). The hairs from the affected areas can be pulled ●● Chin, jaws, and neck are the most commonly
out easily without any pain. affected sites.
●● Involvement of upper lip is rare. ●● It is characterized by multiple closely set grouped
●● DD: Sycosis barbae. follicular papules with anserine (goose skin-like)
appearance (Figure 6.27a–c).
Pseudofolliculitis barbae (PFB) (shaving bumps)
●● PFB is a foreign-body inflammatory reaction against

the hair keratins that have entered the dermis through

extrafollicular or transfollicular penetration.
●● The condition is mostly seen in post pubertal males with

thick curly hairs and in populations with dark-colored

skin. Hirsute females too can develop pseudofolliculitis
barbae after shaving. The sites common to both sexes
include axillary and pubic areas.
●● History of improper shaving techniques (e.g., against

the grain) is common.

●● The patients present with painful, flesh-colored or

erythematous papules with a hair shaft in the center,

and lesions usually appear after shaving. The hair
shaft can be gently lifted up to free the free end of hair
embedded in the dermis (Figure 6.26).
●● The condition may progress to pustules and abscess

formation. Long-standing cases develop post-inflammatory

hyperpigmentation, scarring, and keloid formation.
●● DD: Folliculitis.

Traumatic anserine folliculosis

Figure 6.27  (a) Traumatic anserine folliculosis seen as
●● Traumatic anserine folliculosis is thought to result from
multiple grouped follicular papules localized to an area
friction and trauma, and is more common in children over the right jaw. Note clear-cut demarcation between
and adolescents. lesional and non-lesional skin. (Continued)
 Facial papules  119

●● DD: Keratosis pilaris, lichen spinulosus, disseminate

and recurrent infundibular folliculitis.

Steroid acne
●● It is an acneiform eruption seen after corticosteroid

therapy (systemic, topical, or intravenous).

●● No age is spared, but most patients are young adults.

Steroid acne is less common in colored skin.

●● The lesions are characterized by monomorphic,

papulopustular eruptions at the same stage of

development. Comedones are absent in the acute
inflammatory stage but may appear later on
(Figure 6.28a–c).
●● The face and trunk are commonly affected. In the case

of inhaled steroids, lesions occur in and around the

nose and mouth.
●● DD: Acne vulgaris, chloracne.

Lepromatous leprosy
●● Leprosy (Hansen’s disease) is a chronic infectious

disease caused by Mycobacterium leprae and

affects mainly the skin and peripheral nervous
system.

Figure 6.27 (Continued)  (b) Localized grouped follicular

papules in traumatic anserine folliculosis. (c) Traumatic
anserine folliculosis in an adolescent. (c – Courtesy: Figure 6.28  (a) Erythematous monomorphic papules in
Dr. Deverashetti Srinivas, Nizamabad, India.) steroid-induced acne. (Continued)
120  Facial papules

Figure 6.29  Multiple erythematous papulonodules over

the face in lepromatous leprosy.

●● DD: Post-kala azar dermal leishmaniasis, sarcoidosis,

secondary syphilis, lymphoma.

Granuloma annulare
●● It is an idiopathic dermatosis characterized by
Figure 6.28 (Continued)  (b) Chest involvement in steroid-
annular lesions with asymptomatic, skin-colored
induced acne. (c) Upper back involvement in steroid-
induced acne.
papules at the periphery and central clearing
(Figure 6.30a,b).
●● It is usually asymptomatic in nature; however,

occasional pruritus has been reported.

●● Lepromatous leprosy presents with generalized ●● The central part is slightly depressed and skin colored/

hypopigmented macules, erythematous hyperpigmented.

papulonodular lesions, erythematous plaques, and ●● Although the face can be involved, the dorsal hands,

diffuse infiltration, including facial involvement. The feet, and extensor aspect of upper and lower limbs are
lesions are bilaterally symmetrical (Figure 6.29). In the commoner sites.
severe cases, the face may assume the appearance ●● There are several variants:

of leonine facies. Other associated findings ●● Localized

include madarosis, infiltration of ear lobules with ●● Generalized (diabetes mellitus, thyroid disease)
papulonodular lesions, and blocked nose with ●● Perforating
occasional bleeding. Late cases may have saddle-nose ●● Patch type
deformity and Buddha ears. ●● Subcutaneous
●● Nerve trunks on the face and peripheral nerve trunks ●● Acute-onset, painful
are thickened, with sensory loss in gloves-and-stockings ●● DD: Closed comedones, Tinea faciei, molluscum

pattern. contagiosum, deep fungal infections.

 Facial papules  121

with a normal duration of two to ten weeks but can last

for up to 12 months.
●● The cutaneous manifestations of secondary syphilis
are variable, classically comprising four major types
of rashes: macular, papular, papulosquamous, or
pustular.
●● Papular lesions are the most prominent cutaneous
manifestation of the secondary syphilis that follow an
evanescent coppery-red macular rash. The lesions are
usually asymptomatic, erythematous, or brownish-red,
discrete, papules, often showing a collarette of scales
(Figure 6.31a,b). The eruption is generalized, involving
both skin and mucous membrane, is bilaterally
symmetrical and more prominent on upper extremities
followed by the abdomen and lower extremities, with a

Figure 6.30  (a) Multiple skin-colored papules on face in

granuloma annulare. (b) Similar papules on neck.

Secondary syphilis9
●● Syphilis is a sexually transmitted disease caused by the

spirochete Treponema pallidum, occurring in primary,

secondary, and tertiary stages, with a long and chronic
course in the untreated patients. Figure 6.31  (a) secondary syphilis seen as multiple ery-
●● The secondary stage (secondary syphilis) appears three thematous, shiny papulonodular lesions with crust over
weeks after the appearance of primary lesion (chancre) few lesions. (Continued)
122  Facial papules

●● Cutaneous cryptococcosis can be primary

(through direct inoculation) or secondary (through
dissemination).
●● Primary cutaneous cryptococcosis develops at the site
of trauma, and the lesion is usually solitary and on the
exposed parts. The lesion starts as a papule, grows to
become a nodule, and then ulcerates, accompanied by
regional lymphadenopathy.
●● Disseminated cutaneous cryptococcosis is common in
immunocompromised patients. Though lesions may be
generalized, they are predominant on the head and neck
region. The most common presentation is umbilicated
papules and nodules of varying size that develop
a central hemorrhagic crust (Figure 6.32). Other
presentations include acneiform papules or pustules,
warty crusted plaques, and ulcers.
●● Cutaneous lesions in disseminated disease precede
other symptoms of disseminated disease by two to eight
months. Central nervous system (CNS) involvement is
common and manifestations include meningeal signs,
nerve palsies, vertigo etc.

Figure 6.31 (Continued)  (b) Same patient – side profile.

(a,b – Courtesy: Dr. Hiral Shah, Baroda Medical College,
Vadodara, India.)

predilection for the palms and soles. Large papular and

nodular lesions on the face are also seen.
●● Lesions may have a lichenoid or psoriasiform
appearance and may assume annular
configuration.
●● Patients often have condylomata lata – flat, moist
papules and plaques in groin and perianal region.
●● Systemic features include low-grade fever, generalized
lymphadenopathy, headache, and malaise.
●● DD: Leprosy, sarcoidosis, psoriasis.

Cryptococcosis
●● Cryptococcosis is an opportunistic infection caused
Figure 6.32  Cryptococcosis in an immunocompromised
by the encapsulated yeast Cryptococcus neoformans. lady, seen as multiple umbilicated papules and nod-
The infection is mostly seen in immunocompromised, ules of varying sizes with central hemorrhagic crust.
but immunocompetent patients too may develop (Courtesy: Dr. Hitesh Khatri, Gauhati Medical College
the disease. and Hospital, India.)
 Facial papules  123

●● DD: Basal cell carcinoma and molluscum contagiosum keratotic plaques, pustules, and nodules. Mucosa may
(for single lesions), molluscum contagiosum, acne show painful nodules, erosions or ulcers. There may
vulgaris, secondary syphilis, and histoplasmosis (for be an associated erythema multiforme or erythema
multiple lesions). nodosum.
●● DD: Acne vulgaris, molluscum contagiosum,
Histoplasmosis (Darling disease, cryptococcosis, lymphoma.
reticuloendotheliosis, Ohio Valley disease, bat
disease, caver’s and miner’s fever) Common acquired melanocytic nevus (CAMN)
●● Histoplasmosis is a deep mycosis caused by Histoplasma (mole)
capsulatum and is prevalent in tropical countries. ●● CAMN is a benign proliferation of a type of melanocyte

Infection is acquired after inhalation of conidia, and known as a “nevus cell” (which forms nests and lacks
lungs (followed by spleen and liver) are the most dendrites).
commonly affected organ. Immunocompromised ●● Based on the location of nests of nevus cells, CAMN is

persons may develop disseminated disease. further classified into three types – junctional (nests
●● People at risk are miners, engineers, farmers, at the dermo-epidermal junction [DEJ]), compound
archeologists, guano collectors, anthropologists, (nests at DEJ and in dermis), and intradermal (nests in
builders, and persons with certain interests, such as dermis). Junctional CAMN presents as macules; both
cavers, birders, etc. compound and intradermal CAMN present as facial
●● Mucocutaneous lesions are usually seen in disseminated papules.
histoplasmosis; primary cutaneous histoplasmosis ●● No race is spared, but the mean number of CAMN is

(painless ulcer with regional lymphadenopathy) is very lower in people with colored skin.
rare. ●● It starts appearing after the first six months of life,

●● Generalized involvement with the face and mucosae increases in number during childhood and adolescence,
(oral, perianal, and genital) affection is common in and then slowly regresses with age.
disseminated disease. ●● Compound nevus presents as smooth-surfaced, dome-

●● The range of cutaneous lesions is diverse and shaped, round to oval pigmented papules with sharply
includes, but is not limited to, papules (umbilicated, demarcated border and homogenous pigmentation.
with hemorrhagic crusts), acneiform eruptions, The color varies from tan to dark brown to black
erythematous papules, plaques with or without crusts, (Figure 6.33a–c). In smaller lesions the elevation is subtle.

Figure 6.33  (a) Compound nevus seen as dome-shaped, black papules (black arrow) of varying sizes. Additionally, the
patient has pigmented macules of junctional melanocytic nevus (red arrow). (b) Gradually the lower part of compound
nevus may lose pigment and become skin-colored. (Continued)
124  Facial papules

Figure 6.33 (Continued)  (c) Large compound nevus with partly pigmented and non-pigmented skin. (d) Soft to firm skin-
colored papulonodule of intradermal melanocytic nevus.

●● Intradermal nevus presents as skin-colored to ●● DD: Intradermal melanocytic nevus, warts, nodular
tan, dome-shaped papules with a soft, rubbery basal cell carcinoma.
texture (Figure 6.33d). Sometimes lesions may be
Basal cell carcinoma (BCC)
papillomatous, pedunculated, or cerebriform.
●● BCC is the most common non-melanoma skin cancer,
●● Cerebriform intradermal nevus may occur in
association with various syndromes such as arising from the pluripotential cells in the basal layer
Noonan syndrome, Beare-Stevenson syndrome, of the epidermis or follicular structures. It is a slow-
Ehlers-Danlos syndrome, Michelin tire baby growing, locally destructive tumor of the epidermis,
syndrome, Turner syndrome, and fragile X having a metastatic rate ≤ 0.1%.
●● BCC is common in elderly males and in light-skinned
syndrome.
●● DD: Pigmented basal cell carcinoma, melanoma (for individuals.
compound nevus), osteoma, basal cell carcinoma,
fibrous papule of nose, appendageal tumor (for
intradermal nevus).

Fibrous papule of nose

●● Fibrous papule of the nose (fibrous papule of

the face, sporadic angiofibroma) is a benign

angiofibroma affecting the nose most commonly,
hence the name.
●● It is mostly seen in the middle-aged patients.

●● It usually presents as solitary asymptomatic,

flesh-colored, dome-shaped, firm papule seen

predominantly on the nose (mainly over ala, alar
groove, and tip of the nose) (Figure 6.34). Also, it
may occur over the chin, cheeks, forehead, and neck.
Sometimes, a central hair may be seen. It persists Figure 6.34  Fibrous papule of the nose. Note trichostasis
lifelong unchanged. spinulosa on the tip of nose. (Courtesy: Dr. Divya Sachdev,
●● Rarely, multiple lesions may be noted. Raipur, India.)
 Facial papules  125

●● It is usually seen on the face, head (scalp included), and Dilated pore of Winer
neck. ●● It is a benign adnexal tumor showing follicular
●● Nodular BCC is the most common form and presents differentiation.
as a solitary asymptomatic, round, shiny, flesh-colored ●● It is clinically characterized by an asymptomatic,
papule with telangiectasia. The lesion grows very slowly, solitary, enlarged pore with a central keratin plug,
and central ulceration may develop, leaving a rolled, surrounded by normal skin.
pearly border with telangiectasia (a valuable clinical ●● The most common sites are the face and neck; however,
clue) (Figure 6.35a). truncal distribution has been reported in middle-aged
●● Pigmented BCC appears to be very common in subjects (males more than females).
colored skin and is characterized by partial or ●● The exact etiology remains unknown; some consider
complete pigmentation of the tumor. The lesion it to be an epidermal inclusion cyst with epithelial
mimics melanoma, and the presence of a rolled, hyperplasia, while others consider it to be a variant of
pearly border allows clinical differentiation from the nevus comedonicus.
latter. ●● DD: Epidermal inclusion cyst, melanocytic nevus.
●● As the BCC is mostly pigmented in our experience and
the pigmented papule of BCC mimics many common Pilomatricoma (pilomatrixoma, or calcifying
conditions such as acquired melanocytic nevus epithelioma of Malherbe)
and DPN (and seborrheic keratosis) (Figure 6.35b), ●● Pilomatricoma is a benign tumor with differentiation
we do not see many cases with BCC in the papule toward the matrix of the hair follicle.
stage. Patients usually present late with plaque or ●● It can develop at any age, showing bimodal peak
noduloulcerative lesions. presentation during first and sixth decades of life and
●● Lack of awareness among the population is another affects women more commonly than men.
reason for late diagnosis. We have diagnosed BCC ●● The most commonly affected sites are the head and
incidentally in patients presenting with less serious neck, followed by upper extremity, trunk, and lower
conditions, such as tinea corporis or miliaria rubra. extremity.
●● The clinical variants are superficial, morphoeic ●● The lesion is usually an asymptomatic (sometimes
(sclerosing), micronodular, and fibroepithelioma of tender), firm, deeply seated papule, nodule, or tumor,
Pinkus. adherent to the skin but not to the underlying tissue.
●● DD: For non-pigmented BCC – intradermal nevus, The color of overlying skin varies from flesh-colored to
keratoacanthoma, appendageal tumor, fibrous papules red to bluish-purple (Figure 6.36).
of the face, solitary trichoepithelioma, molluscum ●● Stretching of the skin over the tumor shows multiple facets
contagiosum; for pigmented BCC – CAMN (compound and angles known as “tent sign.” Additionally, applying
type), melanoma, seborrheic keratosis. pressure over one edge of the lesion causes the opposite

Figure 6.35  (a) Small basal cell carcinoma seen as solitary shiny, hyperpigmented papule with an annular plaque with
rolled, pearly border. (b) small annular plaque of basal cell carcinoma near ala nasi. Note multiple acquired melanocytic
nevi on the cheek.
126  Facial papules

Figure 6.36  Pilomatricoma seen as flesh-colored

nodule with central ulceration over the left eyebrow.
(Courtesy: Dr. Raghuraj Hegde, Ophthalmic plastic sur-
gery and Ophthalmology Oncology, Manipal Hospital,
Bangalore, India.)

edge to protrude from the skin, giving an appearance of

a teeter totter. Both these signs are pathognomonic and
relate to calcification within the lesion.
●● DD: Appendageal tumor, epidermoid cyst, foreign
bodies granuloma.

Epidermoid cyst (epidermal inclusion cyst,

sebaceous cyst)
●● An Eepidermoid cyst (EC) is a benign encapsulated

lesion filled with keratin and has its origin in

infundibular portion of pilosebaceous unit. The
preferred term for this entity is epidermoid cyst.
Sebaceous cyst is a misnomer. Very small, superficial
epidermoid cysts are called milia.
●● EC appears to be more common in men and in third

and fourth decades of life.

●● There may be a history of discharge of foul-smelling,

“cheese-like,” semi-solid material.

●● EC presents as asymptomatic, single or multiple,

flesh-colored or yellowish, soft to firm, round lesions

of variable size. The most characteristic feature is a Figure 6.37  (a) Epidermoid cyst seen as single, flesh-colored
central, dark, punctum (Figure 6.37a,b) that may not be round cyst with central, dark, punctum over the right
obvious in smaller lesions. cheek. (b) Multiple bluish cysts with central punctum.
 Facial papules  127

Table 6.4  Clinical characteristics of appendageal tumor

Onset Clinical features Sites Comments

Trichofolliculoma Adulthood Solitary flesh-colored papule or Face (specially
(Figure 6.38a,b) nodule around the nose)
Clinical hallmark is tuft of white hair
emerging from the central pore
Dilated pore of Adulthood Enlarged solitary comedo Face – mostly on the Common in light -skinned
Winer A keratotic plug can be expressed. upper lip, cheek, people
This allows further removal of or forehead Male predilection
caseous, white keratin.
Trichodiscoma/ Young Solitary or multiple small (2–4 mm), Face – chin, nose, Multiple lesions may occur
fibrofoliculoma adults flesh-colored, smooth-surfaced, cheeks, ears, and in isolation or may be a
dome-shaped papules eyebrows part of Birt-Hogg-Dubé
syndrome – triad of
multiple fibrofolliculomas,
trichodiscomas, and
acrochordons
Trichoadenoma Adulthood Solitary skin-colored papule or Face is most
of Nikolowski nodule commonly involved
site followed by
buttock
Trichilemmoma Adulthood Single or multiple small, flesh-colored Face, neck May arise within nevus
papules that are 1–5 mm in sebaceous
diameter Multiple lesions are a part
As the lesion grows, it develops a of Cowden syndrome
hyperkeratotic surface.
Tumor of Elderly Solitary form – scaly papule or Head and neck Slight female
follicular patients nodule preponderance
infundibulum Eruptive form – sudden onset of
multiple variably scaling,
hypopigmented macules and papules

●● The lesions are mostly asymptomatic but may get ● There is a history of repeated bleeding from the
ruptured/infected and then become red and painful. lesion.
●● Malignancy may develop in long-standing cases. ● Clinically it appears as an asymptomatic, usually
●● Multiple EC are seen in Gardner syndrome, solitary, bright-red to brownish-red, vascular papule
Gorlin syndrome, basal cell nevus syndrome, and
pachyonychia congenita.
●● DD: Trichilemmal cyst, dermoid cyst, pilomatricoma,
lipoma, BCC.

Trichofolliculoma
See Table 6.4 and Figure 6.38a,b.

Lobular capillary hemangioma (LCH) (pyogenic

granuloma)
● LCH (pyogenic granuloma, or granuloma

pyogenicum, granuloma telangiectaticum – a

misnomer) is a common, usually solitary, lobulated,
benign vascular proliferation of skin and mucous
membranes.
● The disease usually occurs in the second decade of life

but may affect any age group and has a predilection for Figure 6.38  (a) Trichofolliculoma seen as a skin-colored
males. Mucosal lesions are more common in females. papule. (Continued)
128  Facial papules

Figure 6.38 (Continued)  (b) Dermoscopy image showing

a wisp of white hairs emerging from the central part of the
lesion.

or nodule with a glistening surface (Figure 6.39a,b).

The lesion is partially compressible without any
pulsation, rapidly growing, and friable and may be
sessile, or pedunculated. A surrounding collarette of
scales is seen at the base of the lesion in the majority of
cases. Bleeding, erosion, ulceration, and crusting are
frequent.
●● It may bleed very easily, and growth ceases after a few
weeks of an initial phase of rapid evolution, rarely
showing spontaneous resolution.
●● DD: Angiolymphoid hyperplasia with eosinophilia,
infantile hemangioma, basal cell carcinoma.

Infantile hemangioma Figure 6.39  (a) Lobular capillary hemangioma seen as a

●● Infantile hemangioma (IH) is a true neoplasm of solitary bright-red vascular papule over the lower lip.
(b) A solitary lesion of lobular capillary hemangioma on
endothelial cells and is the most common benign
the cheek. The lesion often bleeds spontaneously.
soft-tissue tumor of infancy and childhood. It is well
known for its rapid growth during the first few weeks to
months of a child’s life, followed by a spontaneous but ●● It is most commonly seen in the head and neck region.
slow involution. Most infantile hemangiomas resolve by ●● IH may be present at birth or appear in the first few
the age of nine years. weeks after birth and usually manifests as a solitary,
●● It occurs in greater frequency in girls, whites, premature asymptomatic pale patch that grows rapidly to an
infants, twins, and children born to mothers of high erythematous papule. As it grows, it may assume a
maternal age. dome-shaped, bosselated, plaque-like, or tumoral
 Facial papules  129

grows rapidly, achieving a size of 1–2 cm within

weeks and assumes the morphology of a dome-
shaped nodule with a smooth shiny surface and
a central crateriform ulceration or keratin plug
(Figure 6.41a,b). The surface may show telangiectatic

Figure 6.40  (a) Infantile hemangioma seen as solitary

dome-shaped erythematous papule present over the
face. (b) Infantile hemangioma over the nose.

appearance (Figure 6.40a,b). The lesion may be firm,

rubbery, and tense.
●● Involution starts at the center and spreads centrifugally.
With involution, the lesion becomes less red and
assumes a flesh-colored appearance. Also, the lesion
becomes softer and more compressible. Finally, the
lesion may resolve completely with no evidence of a
previous pathologic process.
●● However, about 50–60% of lesions resolve incompletely,
and residual changes like telangiectasia, stippled scarring,
anetoderma or epidermal atrophy, hypopigmentation,
and redundant skin with fibrofatty tissue may persist.
●● DD: For popular IH – lobular capillary hemangioma
(granuloma pyogenicum), angiokeratoma.

Keratoacanthoma
●● Keratoacanthoma mostly occurs on sun-exposed sites, such

as the face, neck, and dorsum of the upper extremities, and Figure 6.41  (a) Keratoacanthoma seen as a solitary dome-
is less common in darker-skinned individuals. shaped nodule with central keratin plug over the left upper
●● Keratoacanthoma originates as a solitary, firm, eyelid. (b) Solitary keratoacanthoma over nose. (Courtesy:
dome-shaped, skin-colored or reddish papule. It Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
130  Facial papules

Figure 6.42  (a) Cutaneous leishmaniasis seen as a solitary erythematous papule with central adherent crust over the right
cheek. (b) Cutaneous leishmaniasis presenting as multiple erythematous papules with satellite lesions over the face.
(a,b – Courtesy: Prof. Reza Yaghoobi, Ahvaz Jundishupor University of Medical Sciences, Iran.)

blood vessels, and the keratin plug may grow to form ●● Facial MC is commonly seen in children and
a cutaneous horn. immunosuppressed patients. In immunocompetent
●● The lesion may remain static for months and then may adults, MC usually presents as a sexually transmitted
heal with atrophic scarring. It may rarely progress to infection, and lesions are limited to the perineum,
invasive or metastatic carcinoma. genitalia, lower abdomen, or buttocks.

Cutaneous leishmaniasis (CL)

●● CL starts as an erythematous papule at the site of a bite

on exposed parts, including face. The papule increases

in size, becomes a nodule, and finally ulcerates with an
adherent crust (Figure 6.42a,b). Satellite lesions with a
nodular lymphangitis may develop.
●● Lesions may show spontaneous resolution over months

to years and heal with atrophic scarring.

●● Immunosuppressed patients may develop disseminated

lesions.
●● DD: Lupus vulgaris

Granuloma faciale
●● Granuloma faciale presents with multiple or solitary,

soft, well-circumscribed papules, plaques, or nodules

(Figure 6.43). The surface of the lesion is smooth and
may show prominent follicular openings. Extrafacial
lesions may occur.
●● DD: Hansen’s disease, sarcoidosis, PKDL.

Molluscum contagiosum (MC)

●● Molluscum contagiosum (water warts) is an infection Figure 6.43  Granuloma faciale presenting as erythema-
of skin and mucosa, caused by a DNA poxvirus tous edematous plaque over the right cheek. Surface is
molluscum contagiosum virus (MCV). remarkable for follicular prominence and telangiectasia.
 Facial papules  131

Figure 6.44  Multiple discrete, dome-shaped, pearly white,

umbilicated papules molluscum contagiosum over the face.

●● In children and immunocompetent patients, the lesion

usually presents as asymptomatic, single or multiple,
discrete, dome-shaped, pearly white papules of variable
size, with central umbilication and usually resolves
spontaneously over weeks or months (Figure 6.44).
Pseudo-Koebnerization may be observed and, when
present, is a valuable clinical clue.
●● In immunosuppressed patients, atypical variants
such as giant molluscum (> 1.5 cm), mucosal lesions,
molluscum presenting as an abscess, molluscum
without an umblication, tender molluscum, nodular
molluscum, or rarely agminate type have been reported.
●● DD: Milia (absence of umblication), closed
comedones, vellus hair cyst, epidermoid cyst,
cryptococcosis (in immunosuppressed patients).

Neurofibroma
●● Patients with neurofibromatosis type 1 (NF1) may

develop facial neurofibromas, along with generalized

neurofibromas and other features of NF1.
●● Facial neurofibromas present as asymptomatic, soft,

skin-colored to pigmented, papulonodular lesions

which may be sessile, or pedunculated (Figure 6.45a,b).
●● DD: PKDL, lymphoma, lepromatous leprosy.

Nevus sebaceous
Figure 6.45  (a) Skin-colored papules of neurofibromatosis
●● Nevus sebaceous may present on the face. Initially the
type 1. (b) Neurofibromatosis type 1 with multiple,
lesion starts as hairless, solitary, linear or round, slightly slightly pigmented, papulonodular lesions over the face.
raised, yellow, orange or tan plaque since birth or soon (b – Courtesy: Dr. PC Das, Katihar, India.)
132  Facial papules

after. At puberty, the lesion starts growing and develops be pigmented, and affecting mainly the face,
papulonodular lesions with a verrucous surface or neck, and extremities (Figure 6.47a–c). Pseudo
mammillated appearance (Figure 6.46). Koebnerization is seen in most of the cases.
●● Later in life, the lesion may develop various appendageal ●● DD: Syringoma, pityriasis versicolor.
tumors; syringocystadenoma papilliferum and ●● Filiform warts are digitate papules with verrucous
trichoblastoma are most common tumors developing in tops. These warts are particularly common on the face
nevus sebaceous. (Figure 6.47d).
●● DD: Wart, verrucous epidermal nevus. ●● DD: Skin tag.
●● Verruca vulgaris (common warts)
Viral warts ●● These are hyperkeratotic papules with an irregular
●● Verruca plana (plane warts) are benign skin tumors surface.
caused by infection with human papillomavirus, type 3, ●● They can occur on any part of body, hands being
and less commonly, 10, 27, and 41. the commonest site. They can be seen on face too
●● The disease is more common in children and (Figure 6.47e)
adolescents and has no sexual or racial predisposition. ●● DD: Seborrhoeic keratosis, lichen planus,
Immunocompromised individuals and those who keratoacanthoma.
are on immunosuppressant treatment are at risk of
developing large, extensive, and resistant warts.
●● The lesions are smooth, flat-topped, round or
polygonal in shape, varying from 1–5 mm or
more in diameter, usually skin-colored or may

Figure 6.46  Nevus sebaceous presenting as multiple

linear tan plaque and grouped papulonodular lesions with Figure 6.47  (a) Multiple flat-topped, round or polygonal,
verrucous surface over the right auricular area. slightly pigmented papules of verruca plana. (Continued)
 Facial papules  133

Figure 6.47 (Continued)  (b) Skin-colored papules of verruca plana. Note linear arrangement of lesions (pseudo-Koebnerization,
black arrow). (c) Multiple coalescing hypopigmented papules of verruca plana. (d) Multiple digitate papules of filiform
warts on face. (e) Solitary verrucous papule of common wart on face.
134  Facial papules

Seborrheic keratosis (SK) with adenocarcinoma of gastrointestinal tract. But

●● SK is the most common benign epidermal tumor, it may be rarely seen with non-cancerous conditions
resulting from a clonal expansion of a mutated such as inf lammatory dermatoses and drug
epidermal keratinocyte and appearing on reactions.
hair-bearing areas. Mucosa, palms, and soles are ●● Clinical variants of SK:
spared. ●● Stucco keratosis (keratosis alba) – superficial gray
●● SK is more common in Caucasian populations with no to light-brown flat keratotic lesions over acral
gender predilection. They start to appear in middle age areas (dorsa of the hands and feet, the ankles,
and increase in number throughout life. forearms).
●● SK presents as multiple asymptomatic (sometimes ●● Melanoacanthoma – deeply pigmented SK.
pruritic), well-circumscribed, light-tan to dark-brown ●● Polypoid lesions – small polypoidal lesions at the
or black, round to oval macules, patches, papules, site of friction (neck, under the breast, or in the
or plaques with a keratotic (rough) or waxy surface. axillae). They mimic skin tags but have furrowed
Lesions have a “stuck-on” appearance, a valuable rough surfaces.
clinical clue for diagnosis (Figure 6.48a,b). Many ●● Dermatosis papulosa nigra.
lesions develop keratotic plugging of the surface and ●● DD: Verruca vulgaris, junctional nevus, compound
become thicker over time. nevus.
●● Manipulation might lead to swelling, oozing or

bleeding, crusting and darkening of the lesion (irritated Verrucous epidermal nevus
SK). ●● This is clinically characterized by skin-colored to dirty-

●● Sign of Leser-Trélat (eruptive SK) is known as a sign brown or black irregular/verrucous papules distributed
of internal malignancy, most commonly associated along the lines of Blaschko.

Figure 6.48  (a) Multiple pigmented papules and plaques of seborrheic keratosis. Note stuck-on appearance of lesions.
(b) Seborrheic keratosis seen as sharply demarcated, pigmented, papules and plaques with a keratotic surface over the face.
 Facial papules  135

Figure 6.49  (a) Verrucous epidermal nevus seen as dirty-looking, pigmented, verrucous papules distributed along the
lines of Blaschko. (b) Multiple linear bands of verrucous epidermal nevus along the lines of Blaschko.

●● They often coalesce to form linear plaques ●● Localized papules with associated alopecia
(Figure 6.49a,b). ●● Generalized papules associated with myasthenia
●● They usually appear at birth or in childhood. gravis and alopecia (Brown-Crounse syndrome)
●● They can occur on any part of body, unilaterally or ●● Generalized autosomal dominant familial type
bilaterally. without associated disorder, but with other
●● DD: Nevoid psoriasis, linear lichen planus, Blaschkitis. associated findings including milia, comedone-
like lesions, palmar pitting, hypohidrosis, and
Basaloid follicular hamartoma (BFH) hypotrichosis
●● BFH is a rare, benign neoplasm of the hair follicle, ●● Multiple BFH are also noted in nevoid basal cell
caused by mutation of the PTCH gene. carcinoma syndrome (Gorlin syndrome or basal cell
●● BFH usually presents as single or multiple 1- to 2-mm nevus syndrome) and Bazex-Dupré-Christol syndrome.
brown to dark-colored papules on the face, scalp, and
neck. Keratosis follicularis spinulosa decalvans
●● Five clinical forms are recognized: ●● KFSD is a disorder of epidermal differentiation caused by

●● Solitary or multiple papules impaired cholesterol and lipid metabolism in epidermis.

●● Localized linear papules/plaques (linear unilateral It is an X-linked condition, and males are mostly affected.
basaloid follicular hamartoma (LUBFH) or linear It is one of the entities included under the umbrella term
unilateral basal cell nevus) keratosis pilaris atrophicans (KPA) (Box 6.1).

BOX 6.1: Keratosis pilaris atrophicans (KPA)

KPA is characterized by follicular keratotic papules that heal by atrophy and/or alopecia. Additional findings common
to this group of disorders are an atopic tendency and keratosis pilaris on the extensor surfaces of the extremities. It
includes the following:
●● Keratosis pilaris atrophicans faciei (KPAF)/ulerythema ophryogenes – onset in first few months of life; common in
boys; presents with erythema and follicular keratotic papules on the lateral third of the eyebrows. Disease pro-
gresses to atrophy and alopecia, which may extend to the cheeks and forehead.
●● Atrophoderma vermiculatum (AV) – usually in children (before puberty); erythematous follicular keratotic papules
that slowly progress to the characteristic reticular atrophy; described as worm-eaten, reticular, or honeycomb atro-
phy. Occurs on the cheeks, preauricular area, and forehead.
●● Keratosis follicularis spinulosa decalvans (KFSD).
136  Facial papules

●● The skin lesions develop within the first month of life ●● Histiocytoses are broadly grouped into Langerhans
and are characterized by follicular keratotic papules, cell histiocytoses (LCH) and non-LCH, based on
especially on the scalp and face, associated with predominant infiltrating cells – Langerhans cell in LCH
scarring alopecia of the scalp, eyelashes, and eyebrows. and dermal dendritic cells (and cells with phenotype of
●● Ocular symptoms are prominent and include other than Langerhans cells and dermal dendrocytes) in
photophobia, keratitis, conjunctivitis, congenital non-LCH.
glaucoma, lenticular cataract, and corneal dystrophy. ●● The major histiocytoses that may present with or
●● DD: Ichthyosis follicularis alopecia photophobia (IFAP) develop facial papules are summarized in Table 6.5.
syndrome.
Colloid milium
Histiocytoses ●● Colloid milium refers to a group of degenerative,

●● The histiocytoses are a group of disorders characterized cutaneous deposition disorders related to excessive
by abnormal proliferation and accumulation of cells sun exposure (and hydroquinone use) and is
of the mononuclear phagocytic system consisting of characterized by amorphous, hyaline-like deposits in
Langerhans cells, macrophages, and dendritic cells. the dermis.

Table 6.5  Facial papules in histiocytoses

Predominantly Extracutaneous/
Disease Onset Cutaneous features affected sites Systemic features
Langerhans cell histiocytosis
Letterer-Siwe Children Yellow-brown scaly papules, Seborrheic area, Fever, anemia,
Disease 2-3 years petechiae and crust intertriginous thrombocytopenia,
(Figure 6.50a,b) areas pulmonary infiltrates,
hepatosplenomegaly,
lymphadenopathy.
Hashimoto-Pritzker At birth or Multiple, red-brown papules, Generalized Lymph nodes, bones,
disease Infancy nodules, or vesicles liver, and lungs
Spontaneous resolution is noted involvement
Non-Langerhans cell histiocytosis
Juvenile Infants and Solitary or multiple firm rubbery, Head and neck Eye, lungs, central
xanthogranuloma young yellow brown papules and (including face) nervous system, and
(Figure 6.50c,d) children nodules bone lesions
Benign cephalic Infants and Yellow to red-brown macules and Face (cheeks, Diabetes insipidus
histiocytosis young papules forehead, ears), (rare)
children Lesions heal with hyperpigmentation and neck
and may vanish completely over
months or years.
Generalized Adults Recurrent crops of red to brown Generalized – face, None
eruptive papules trunk, proximal
histiocytosis Lesions resolve with hyperpigmented extremities
macules or small scars Flexures are spared
Papular xanthoma Adults Yellow or yellow-red papulonodular Head, back Patients are
lesions with no tendency to normolipemic
coalesce No systemic
Lesions may heal with anetoderma- involvement
like scarring
Progressive nodular Adults Superficial yellow-orange papules Random Oral cavity, larynx, and
histiocytosis conjunctival mucosa
(Figure 6.50e) involvement
Xanthoma Young Round to oval, orange to yellow- Flexures – neck, Diabetes insipidus
disseminatum children brown papules and nodules axilla, antecubital
(Figure 6.50f,g) occurring over flexures fossa, perianal
area, and groins
Mucosal involvement
 Facial papules  137

Figure 6.50  (a) Langerhans cell histiocytosis in a one-

and-a-half-year-old child (front view). (b) Langerhans
cell histiocytosis in a one-and-a-half-year-old child (back
view). (c) Juvenile xanthogranuloma presenting as solitary
erythematous nodule. (d) Erythematous papule of juve-
nile xanthogranuloma on the neck. (e) Erythematous
papulonodular lesions of progressive nodular
histiocytosis. (Continued)
138  Facial papules

Figure 6.50 (Continued)  (f) Yellow-orange confluent pap-

ules, plaques, and nodules in Xanthoma disseminatum in
a young man. (g) Mucosal lesions in Xanthoma dissemi-
natum. (a,b,f – Courtesy: Dr. Sudhir Singh, Department
of Dermatology and Venereology, Jawahar Lal Institute
of Medical Sciences, Nagpur, India; c,e – Courtesy:
Dr. Neena Khanna, Department of Dermatology and
Venereology, All India Institute of Medical Science, New
Delhi, India; d – Courtesy: Dr. Santosh Rathod, SVPIMSR,
Smt. NHL Municipal Medical College, V.S. Hospital,
Ahmedabad.) [a–c, e–g: Reprinted by permission from
Springer International Publishing [Atlas of Dermatology,
Dermatopathology and Venereology] by [Bruce R. Smoller
and Nooshin Bagherani] [COPYRIGHT] (2021).]

●● The condition is more common in light-skinned

individuals, and the adult form is more common in
males.
●● Lesions on the face are common on the cheeks,
periorbital area, nose, and ears. Other commonly
affected sites are the neck, hands, and forearms.
●● Clinically, it is characterized by waxy, partially
translucent, firm papules ranging from 1–5 mm in
diameter (Figure 6.51). The lesions appear in crops.
Gelatinous material can be expressed. The underlying
skin shows signs of actinic damage and may be
thickened, furrowed, and hyperpigmented.
●● Five clinical forms are recognized:
●● Adult colloid milium – described above.
●● Juvenile colloid milium – clusters of small, yellow-
brown, translucent papules and plaques on the face
and neck. Lesions bleed on minor trauma.
●● Pigmented colloid milium – gray-black clustered
papules on the face.
●● Nodular colloid milium – solitary nodule
or plaque on chronically sun-exposed skin,
particularly the face.
●● Acral form – painless, slowly progressive,
hyperkeratotic papules on the top of the fingertips.
●● DD: Epidermal inclusion cyst, molluscum Figure 6.51  Colloid milium seen as multiple waxy,
contagiosum, sebaceous hyperplasia, steatocystoma firm, yellowish papules below the right lateral canthus.
multiplex. (Courtesy: Dr. PC Das, Katihar, India.)
 Facial papules  139

Lipoid proteinosis ●● Clinically it appears as single or multiple erythematous,

●● Lipoid proteinosis is a rare autosomal recessive scaly, annular, or polycyclic plaque with papules,
genodermatosis caused by mutations in the gene pustules, and crusting at the margin. The lesion spreads
encoding extracellular matrix protein 1 (ECM1) and centrifugally, with a clear, hypopigmented center
is characterized by the deposition of an amorphous (Figure 6.53a,b). It may be associated with itching,
hyaline material in the skin, mucosa, and viscera. burning, or photosensitivity.
●● There is no racial or sex predilection. Patients usually

present in infancy and early childhood. The pathognomonic

feature is a hoarse cry due to laryngeal infiltration.
●● The earliest skin findings are vesicobullous lesions,

erosions, and crusts on skin and mucosa. Face and

extremities are most commonly involved, and lesions
heal with pox-like atrophic scarring.
●● As the child grows, there is dermal infiltration resulting

in thickened, waxy, yellowish skin and the appearance

of papules, plaques, and nodules on the face. Moniliform
blepharosis (multiple papules along the eyelid margins,
resembling a string of pearls) is considered a cutaneous
hallmark of the disease (Figure 6.52).
●● Patients may develop hyperkeratotic plaques on sites of

trauma – elbows, knees, and dorsum of the hands.

●● Associated findings are woody firmness and impaired

mobility of the tongue, cobblestone appearance of

mucosa, and patchy or diffuse hair loss.
●● CNS features include seizures, behavioral changes,

learning difficulties, and rage attacks may be observed.

●● DD: Colloid milium, Lichen myxedematosus.

Tinea faciei
●● Tinea faciei is a dermatophytic infection that occurs

over the face in women and prepubertal boys, and non-

bearded areas of men.
●● It can affect all age groups, with two peaks – children

between 2 and 14 years, and adults 20–40 years of age.

Figure 6.53  (a) Steroid-modified tinea presenting as ery-

thematous scaly papules, erosions, and crusts on a back-
Figure 6.52  Papules on the eyelid margin in lipoid pro- ground of erythematous skin. Note the margin of the lesion
teinosis. Note waxy, yellowish thickened skin and pock- (black arrow), a clue to the diagnosis. (b) Erythematous
like scars at places. papules on an erythematous face in steroid-modified tinea.
140  Facial papules

●● In steroid-modified lesions, central clearing is not

obvious. The diagnosis rests on a high index of
suspicion.
●● DD: Seborrheic dermatitis, atopic dermatitis, contact
dermatitis.

Additional image – Figure 6.54

REFERENCES
1. Marks R. Facial Skin Disorders. 1st edition. Oxford: Informa
Healthcare; 2007. P. 1–164
2. Zeichner JA. Acneiform Eruptions in Dermatology A Differential
Diagnosis. 1st edition. New York: Springer; 2014. P. 3–404.
3. Ubriani R, Grossman ME. Facial papules as a marker of internal
malignancy. Med Clin North Am 2009;93(6):1305–1331.
4. Foreman RK, McDivitt Duncan L. Appendage Tumors of the
Skin. In: Kang S, Amagai M, Bruckner AL, Enk AH, Margolis DJ,
McMichael AJ, Orringer JS, editors. Fitzpatrick’s Dermatology
in General Medicine. 9th edition. New York: McGraw Hill; 2019.
P. 1820–1856.
5. Basu D, Kumar P, Das A. Unilateral Facial Angiofibromas. Skinmed
2019;17(1):52–53.
6. Chen W, Plewig G. Human demodicosis: Revisit and a proposed
classification. Br J Dermatol 2014;170(6):1219–1225.
7. Kumar P, Das A. Gnathophyma. Skinmed 2018;16(1):45.
8. Kumar P, Das A, Barkat R. Persistent perioral papules in a young
man. Indian J Dermatol Venereol Leprol 2019;85:407–409.
9. Baughn RE, Musher DE. Secondary Syphilitic Lesions. Clin
Microbiol Rev 2005;18(1):205–216.

Figure 6.54  Senile comedones – clustered and confluent

open comedones near the medial canthus of eye.
 E5
Papules: Generalized

NIHARIKA RANJAN LAL

ABSTRACT
Many conditions may present as generalized papular rash. Various clinical characteristics, such as surface features (scaling,
crusting, necrosis), associated vesicular or pustular lesions, and systemic features, are helpful in arriving at clinical diagnosis.
This chapter discusses common and uncommon causes of generalized papular rash on the basis of presence/absence of consti-
tutional symptoms and other morphological characteristics.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-13 141

 E6
Follicular papules

RAMESH CHANDRA GHARAMI

ABSTRACT
Follicular papules are centered on the pilosebaceous units. They may present with or without inflammation and have vari-
able clinical features. Often these lesions present a diagnostic challenge due to their similar clinical appearance. This chapter
discusses the different conditions presenting with follicular papules and appendageal tumors along with their salient features.
Dermatologists need to be aware of these entities for early and accurate diagnosis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

142 DOI: 10.1201/9781351054225-14

 7
Plaques: Localized

ANIRBAN DAS

INTRODUCTION ●● DD: lichen simplex chronicus, hyperkeratotic

eczema (less-defined margin, history of vesicles).
Conditions presenting as plaques may be localized or gener- ●● Scalp psoriasis – another notable localized form of
alized in distribution, and may be infective, inflammatory, psoriasis.
genetic, or neoplastic in nature. Important clinical pointers ●● Whole scalp may be diffusely involved, or multiple
include duration of lesions, color, presence or absence of sur- discrete plaques of varying size may be seen
face changes (scales, follicular plugging, scarring, verrucos- (Figure 7.1f).
ity), and consistency of the lesions (succulent, hard). Also, ●● Usually restricted to hair-bearing areas, extending a
distribution of lesions gives us important input in making a short distance beyond the hairline and around the ear.
clinical diagnosis. For example, tuberculosis verrucosa cutis is ●● DD: Pityriasis amiantacea, seborrheic dermatitis.
mostly seen on trauma-prone sites, whereas plaques of eryth- ●● Genital psoriasis – a notable localized form of psoriasis.
rokeratodermia variabilis are seen over joints, such as knees. ●● Males: The glans penis is the most affected part. In
This chapter discusses the clinical approach to the diagnosis circumcised men, lesions are similar to appearance
of localized plaques, outlined in Table 7.1, and salient clinical at other sites. In the uncircumcised, the plaques lack
features of different entities are discussed below.1–7 the scale but the color and well-defined edge are
usually distinctive (Figure 7.1g). DD: Erythroplasia
of Queyrat, plasma cell balanitis.
ERYTHEMATOUS PLAQUES ●● Females: The most common presentation is a
symmetrical, non-scaly, well-demarcated thin
Psoriasis
plaque affecting the labia majora, associated with
●● It is a common, chronic, inflammatory, and proliferative
marked pruritus.
condition of the skin. ●● DD: chronic erythematous vulvitis without vaginitis.
●● Psoriasis presents with well-demarcated, erythematous

scaly plaques. Scales are loosely attached and have a

typical silvery/micaceous character (Figure 7.1a,b). Leprosy8
●● Auspitz phenomenon – Repeated scratching with a ●● Varied manifestations depending on underlying cell-

glass slide results in a wet surface with characteristic mediated immunity and degree of proliferation of
pinpoint bleeding (Auspitz sign) on removal of the scaly Mycobacterium leprae.
membrane. Demonstration of Auspitz sign is considered ●● Tuberculoid pole (TT and BT leprosy) has a few lesions

diagnostic (Figure 7.1c). and a few thickened peripheral nerve trunks. Lesions in
●● Koebnerization may be seen (Figure 7.1d). tuberculoid pole show lesional hypo/anesthesia, loss of
●● The lesions may be localized (affecting any part of the hairs and loss of sweating, and appear dry.
body) or generalized, with striking involvement of ●● The lesions in lepromatous pole (BL and LL leprosy) are

extensor aspects of the extremities (Figure 7.1e). smaller and numerous, consisting of macules, papules,
●● Occasionally, a white blanching ring around the lesion, plaques, and nodules arranged in a bilateral symmetric
known as the Woronoff ring, may be seen. manner (especially in LL leprosy). The lesions may not
●● Palmoplantar psoriasis – a notable localized form of show lesional hypoaesthesia (rather, sensory loss in a
psoriasis. May remain localized or may be associated gloves-and-stockings pattern is seen) and appear shiny
with a widespread disease. and infiltrated (in contrast to tuberculoid pole). Multiple
●● Typically scaly patches on which a fine silvery scale peripheral nerve trunks may be affected in a symmetrical
can be evoked by scratching. manner, again more so in lepromatous leprosy.
DOI: 10.1201/9781351054225-15 143
144  Plaques: Localized

Table 7.1  Clinical approach to localized plaque

Onset Striking feature Additional clues Diseases

Acquired Erythematous Scaly plaques • Silvery scales – psoriasis
• Greasy scales – seborrheic dermatitis
• Ichthyotic – pityriasis rotunda, erythrokeratodermia variabilis,
progressive symmetric erythrokeratoderma
• Scales on active margin – tinea corporis
• Collarette of scales – pityriasis rosea
• Adherent scales – actinic keratosis, discoid lupus erythematosus
• Violaceous – lichen planus
Associated with hair Leprosy, alopecia mucinosa
loss
Loss of sensation Leprosy
Sun-exposed area Discoid lupus erythematosus, polymorphous light eruption,
Jessner’s lymphocytic infiltration, tumid lupus erythematosus
Acral • Scaly – psoriasis (extensors), secondary syphilis
• Firm, around joints – xanthoma (yellowish lesions), erythema
elevatum diutinum
• Pagetoid reticulosis
Peau d’orange Sweet syndrome, erysipelas, cellulitis, urticaria*
appearance
Annular, skin-colored Granuloma annulare
Miscellaneous Sarcoidosis, granuloma multiforme, cutaneous lymphoid hyperplasia
Hyperkeratotic Itchy Lichen simplex chronicus, lichen planus hypertrophicus,
Usually multiple Verruca vulgaris (pseudo-Koebnerization seen)
Usually solitary • Healing with scarring – lupus vulgaris
• Lymphatic spread (in case of multiple lesions), sporotrichosis,
cutaneous leishmaniasis
• Trauma prone areas – tuberculosis verrucosa cutis, chromomycosis
• Mycobacterium marinum
• Verrucous xanthoma
• Benign lichenoid keratosis
Annular Porokeratosis (see Chapter 23)
Non-scaly Pigmented Seborrheic keratosis, melanocytic nevus
plaques Skin/pale-colored Mastocytoma, shagreen patch, granuloma annulare (annular)
Erythematous Mastocytoma, polymorphic eruption of pregnancy, urticaria*
Atrophic Morphea, lichen sclerosus et atrophicus (prominent follicular openings)
Ulcerated Erythematous Necrobiotic lipoidica, Bowen’s disease, Paget’s disease,
plaques extramammary Paget’s disease
Pigmented Basal cell carcinoma
Macerated Pemphigus vegetans
Congenital Yellow/ Milia en plaque, nevus sebaceous, nevus lipomatosus superficialis,
pale-colored collagen nevus
Hyperpigmented Verrucous epidermal nevus, verrucous hemangioma
Erythematous Hemangioma (vascular), inflammatory linear verrucous epidermal
nevus (linear)
* The lesion in urticaria is a wheal. It has been included here for clinical similarity with plaque.

●● Tuberculoid leprosy (TT) – The lesions consist of one ●● Borderline tuberculoid leprosy (BT) – Lesions of BT
or a few erythematous/hypopigmented patch or plaque. leprosy mimic those of TT leprosy but are more in
The lesions have a well-defined, elevated margin and are number and larger in size, with less-defined margin.
dry, and hairless with complete loss of sensation. Over In fact, lesions can be large enough to cover a big part
time, the center of the lesion may get atrophic, giving an of the extremity. Another characteristic feature is the
annular appearance (Figure 7.2a). margin being ill-defined at places, with finger-like
 Plaques: Localized  145

Figure 7.1  (a) Silvery scales in psoriasis. (b) Arcuate lesions of chronic plaque psoriasis. (c) Auspitz sign – pinpoint bleed-
ing on the removal of scales. (d) Koebnerization in psoriasis. (e) Annular plaques of psoriasis over extensor aspect of the
forearm. (f) Scalp involvement in psoriasis. (Continued)
146  Plaques: Localized

Figure 7.1 (Continued)  (g) Psoriasis over glans penis. (a,b,e–g –

Courtesy: Dr. Piyush Kumar, Katihar, India; c,d – Courtesy:
Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)

pseudopodia and satellite lesions. The lesions show

typical features of leprosy (i.e., hypoaesthesia, hair loss,
and loss of sweating) (Figure 7.2b–g).
●● Localized lepromatous leprosy – A relatively rare
presentation of lepromatous leprosy is the solitary
lesion with a high bacterial count. This can present as
erythematous plaque and may be tender to touch; it
is mostly localized over the face (cheek, preauricular
region, bridge of nose, and upper eyelid) (Figure 7.2h).

Figure 7.2  (a) Annular, erythematous plaque in TT leprosy. (b) Two annular plaques of BT leprosy. (c) BT leprosy with lesion
over dorsum of right hand. (Continued)
 Plaques: Localized  147

Figure 7.2 (Continued)  (d) Large plaque of BT leprosy with type 1 lepra reaction. (e) Erythematous edematous scaly plaque of
BT leprosy type 1 lepra reaction. (f) BT leprosy with type 1 lepra reaction. Note edema of surrounding areas. (g) Erythematous
scaly plaque of borderline tuberculoid leprosy with grossly thickened greater auricular nerve. (h) Localized lepromatous lep-
rosy. Localized plaques. (a – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India; b–f,h – Courtesy: Dr. Piyush Kumar,
Katihar, India. g – Courtesy: Dr PC Das, Katihar, India.)
148  Plaques: Localized

●● DD: Sarcoidosis (red or tan brown), psoriasis, tinea

corporis (itchy, central clearing, peripheral papules,
pustules, and scales), granuloma annulare (annular
plaque, margin has discrete papules).

Discoid lupus erythematosus (DLE)9

●● The term localized DLE is used to describe patients who

have lesions only on the head or neck.

●● Early and well-developed lesions are well-defined,

erythematous plaques with adherent scale, seen over

sun-exposed areas mostly (Figure 7.3a,b). Sometimes,
the lesions may develop into a verrucous plaque.
●● Follicular plugging is a characteristic feature accounting

for keratotic spikes under the scales (carpet-tack sign)

and wide follicular pits in ears.
●● In the evolution of lesion the plaques slowly expand,

with active indurated erythema at the periphery, leaving

depressed scars, telangiectasias, and depigmentation
that are often permanent. The central atrophic scarring
is very characteristic (Figure 7.3c,d).
●● In late lesions or after healing they appear with

hyperpigmented border and atrophic depigmented

center (Figure 7.3e).
●● On scalp, it may cause cicatricial alopecia.

●● These lesions occur most often on the head and

neck areas; in particular the ear canal and scalp

should always be examined. Mucosal involvement is
common. Lips may have focal or diffuse involvement
(Figure 7.3f,g).
●● Linear DLE is a rare variant (Figure 7.3h).

●● DD: Psoriasis (micaceous scales, Auspitz sign,

extensors), lichen planus (pruritic, purplish flat-topped

papules and plaques, flexures), seborrheic dermatitis
(scaly patches and plaques, scalp, face, upper back).

Figure 7.3  (a) Erythematous, scaly papules and plaques of discoid lupus erythematosus on the chest. (b) Early DLE lesions
on the upper back. (c) Well-developed lesion of DLE – bilaterally symmetrical large erythematous scaly plaque with well-
defined margin. (d) DLE lesions may grow to a large size and may have a pigmented margin. (Continued)
 Plaques: Localized  149

Figure 7.3 (Continued)  (e) Healed lesion of DLE – depigmented

patches on the scalp. (f) DLE. Erythematous plaque over left
lower eyelid. (g) Well-defined plaque of DLE on lip. (h) Linear
lesion of DLE. (a–h – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Polymorphic light eruption (PLE) margin of the plaque and are important clinical clues
●● Polymorphic light eruption is an idiopathic (Figure 7.4a–c).
photosensitivity disorder. ●● Another common presentation is erythematous plaques
●● PLE most commonly presents as itchy, closely set, over face, sides of the neck, and sun-exposed areas of
pinpoint papules, and lesions may coalesce to form arms and hands, appearing a few hours after intense sun
a scaly plaque. Tiny papules can be seen near the exposure (Figure 7.4d).
150  Plaques: Localized

Figure 7.4  (a) Polymorphous light eruption seen as scaly plaque with tiny, hypopigmented papules around its margin.
(b) The lesions typically involve extensor aspect of forearms bilaterally. (c) PLE lesions on the neck. (d) Erythematous
plaques of PLE on the nape of the neck. (Continued)
 Plaques: Localized  151

may remain unchanged for several years and then

spontaneously disappear, leaving no scars.
●● They commonly appear on the face, neck, and back.
●● DD: Lymphocytoma cutis (plum-colored plaques,
lymphoid follicles in histopathology), sarcoidosis (red
or tan-brown plaques, naked granuloma), polymorphic
light eruption (micropapules coalesced to form plaques,
extensors, sun-exposed area).

Sarcoidosis11
●● Cutaneous lesions in sarcoidosis exhibit many different

morphologies.
●● Plaques in sarcoidosis occur due to coalescence of

papules and appear as yellow, brown, red, or tan, with or

without mild scaling (Figure 7.5a). There is apple-jelly
appearance on diascopy.
●● Lesions may assume an annular shape because of

peripheral evolution and central clearing, with

hypopigmentation, atrophy, or scarring (Figure 7.5b,c).

Figure 7.4 (Continued)  (e) Facial lesion of PLE in a child.

(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; c–d,
Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata,
India; e – Courtesy: Dr. PC Das, Katihar, India.)

●● Common sites are the upper back and extensor arms.

●● Face involvement is commonly seen in children. The
lesions commonly involve skin near the lateral canthus
(Figure 7.4e).
●● Clinical variants are lichenoid plaque, targetoid lesions,
eczematous, vesicles, and prurigo like lesions.
●● Recurrence in spring is common.
●● DD: Jessner’s lymphocytic infiltration (non-scaly
plaques on face), seborrheic dermatitis (scaly ill-defined
patches over face, scalp), psoriasis (micaceous scales,
Auspitz sign, extensors).
Jessner’s lymphocytic infiltrate10
●● This is an idiopathic benign lymphocytic proliferation.

●● Characteristically lesions are single or multiple, Figure 7.5  (a) Erythematous, shiny plaque in sarcoidosis.
small, non-scaly, solid, pink or red plaques or (b) Annular lesion of the sarcoidosis with central depig-
nodules associated with photosensitivity. The lesions mented, atrophic scarring. (Continued)
152  Plaques: Localized

Figure 7.5 (Continued)  (c) Plaques of sarcoidosis on the Figure 7.6  Erythematous, translucent, plaque over the
face. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; nose in cutaneous lymphoid hyperplasia. (Courtesy:
c – Courtesy: Prof. Sanjay Khare, MGM Medical College, Dr. Piyush Kumar, Katihar, India.)
Indore, India.)

●● They occur mainly on the face, shoulder, and lower Erythema elevatum diutinum
limbs in irregular shapes. ●● This is a rare, chronic, cutaneous eruption characterized
●● There is a history of very slow progression or remaining by fibrosing plaques with histological evidence of
static over many months. leukocytoclastic vasculitis.
●● Lupus pernio: chronic, red-purple indurated papules ●● It presents with multiple, red-violaceous, red-brown,
or plaques that affect the mid-face, particularly the or orange-yellow plaques over elbows, knees, and small
alar rim of the nose, cheeks, ears, fingers and toes; joints of the hands and feet in symmetrical manner.
nasal lesions may be associated with granulomatous Initially, the lesions are soft, but eventually they
infiltration of nasal mucosa. fibrose and leave atrophic scars. Lesions are mostly
●● Angiolupoid type: a type of plaque psoriasis asymptomatic but may be painful at times.
characterized by prominent telangiectasia; solitary, on ●● DD: Xanthoma (yellow plaques and nodules, joints and
the bridge of nose and central face. fingers, hyperlipidemia), gouty nodulosis, rheumatoid
●● Other variants include papular, annular, subcutaneous, nodules (joint pain), lichen planus hypertrophicus
lichenoid, hypopigmented, ichthyosiform, psoriasiform, (bluish-black hyperkeratotic plaques shin, follicular
verrucous. papules in surroundings), Sweet’s syndrome (lesions
●● DD: Leprosy (hypopigmented, hypoesthetic patches), are acute, more often asymmetrical and located on the
psoriasis (micaceous scales, Auspitz sign, extensors), arms, face and neck).
Jessner’s lymphocytic infiltration (non-scaly
erythematous plaques on face). Erysipelas
●● Erysipelas is an acute β-hemolytic group A
Cutaneous lymphoid hyperplasia/lymphocytoma streptococcal infection of the skin.
cutis10 ●● There is local redness, heat, swelling, and a
●● It is a benign, cutaneous B-cell lymphoproliferative characteristic red, raised, erythematous plaque with an
condition. indurated border that spreads rapidly over the skin with
●● It consists of solitary or grouped, erythematous or constitutional symptoms like high fever and joint pain
violaceous papules, nodules or plaques that enlarge (Figure 7.7a,b).
slowly and may reach a diameter of 3–5 cm; they can be ●● DD: Cellulitis (margin is not well defined), erysipeloid
occasionally translucent and are mostly asymptomatic (polygonal plaques, central clearing, peripheral
but sometimes photosensitive lesions over the face, extension, fingers), urticaria (itchy, transient, evanescent
chest, and upper extremities (Figure 7.6). edematous hives/wheals).
●● DD: Jessner’s lymphocytic infiltration (non-scaly

erythematous plaques on face), sarcoidosis (red or Alopecia mucinosa12

tan-brown plaques, naked granuloma), TT leprosy with ●● In alopecia mucinosa, malignant T cells are highly

reaction (hypopigmented, hypoesthetic, tenderness, concentrated in and about hair follicles. There are
swelling), tumid lupus erythematosus, polymorphic often mucinous deposits in follicles, which are then
light eruption. destroyed, producing alopecia.
 Plaques: Localized  153

Figure 7.7  (a) Erysipelas seen as erythematous, shiny plaque with a palpable border over the left leg. (b) Erysipelas
with edematous surface. (c) Coalescing papules and plaque with hair loss in follicular mucinosis. (a – Courtesy: Dr.
Piyush Kumar, Katihar, India; b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India; c – Courtesy: Tanumay
Raychaudhury, The Skin Hospital, Westmead Skin and Cancer Foundation, Australia.)

●● Clinically, follicular papules and plaques are Pagetoid reticulosis

often associated with severe pruritus and with a ●● Pagetoid reticulosis has two clinical patterns.
predilection for the face and scalp. Prominent giant ●● Classic Woringer–Kolopp pagetoid reticulosis – one or
comedones are often a prominent feature with few acral plaques that are sharply bordered, scaly, and
acneiform lesions, and rarely mucinorrhoea may also stable.
be present. ●● Solitary persistent, hyperkeratotic, erythematous
●● Follicular papules and plaques with hair loss occur, plaque on acral area, mainly on palm/sole
typically over the face, neck, and scalp, rarely ●● Localized variant of cutaneous T cell lymphoma
on the trunk and limbs associated with hair loss ●● DD: Palmoplantar psoriasis (hyperkeratotic plaques
(Figure 7.7c). with fissures), hyperkeratotic eczema (diffuse
●● DD: Psoriasis (micaceous scales, Auspitz sign, plaques, deep-seated vesicles, side of the fingers),
extensors), leprosy with type 1 reaction (hypopigmented lichen planus (yellowish hyperkeratotic plaques over
and erythematous tender plaque +/– sensory loss), palms and soles)
nevus sebaceous (smooth hairless plaque, verrucous in ●● Disseminated Ketron–Goodman pagetoid reticulosis –
puberty, areas-scalp, face and neck). described in Chapter 8.
154  Plaques: Localized

●● They occur mainly on sun-exposed skin and in adult

African women.
●● DD: Granuloma annulare (annular plaque, discrete
papules in periphery), tinea corporis (central clearing,
peripheral papules, scales), leprosy (hypopigmented
hypoesthetic plaque).

Xanthoma
●● Xanthoma is a local deposition of lipid-laden

macrophages mainly due to hyperlipidemia.

●● It is localized to typical sites: xanthelasma palpebrum,

tuberous xanthoma, and tendinous xanthoma.

●● Generalized forms of xanthomas include eruptive

xanthoma and generalized plane xanthoma.

●● Xanthelasma palpebrum appears as bilateral and

occasionally symmetrical, soft papules and plaques

present on the eyelids (Figure 7.9a–c).
●● The upper eyelid and region around the medial
canthus are the most common sites of involvement.
●● It may present as associated conditions such as
localized cutaneous phenomenon or systemic
hyperlipidemia.
●● Tuberous xanthoma are yellowish nodules on bony

prominences (i.e., elbow and knee) and on knuckles and

buttocks (Figure 7.9d).
Figure 7.8  (a) Erythematous, annular plaque in granu-
loma annulare. Note that the margin appears beaded
with papules. (b) Granuloma annulare. (a – Courtesy:
Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. PC Das,
Katihar, India.)

Granuloma annulare
●● It is a benign, usually self-limiting, cutaneous disease

that presents as arciform-to-annular plaques.

●● It is characterized by asymptomatic, flesh-colored,

smooth, shiny, well-defined annular plaques, bordered

by closely set small papules (beaded appearance)
(Figure 7.8a,b).
●● The center may be slightly hyperpigmented and

depressed.
●● Plaques commonly appear on dorsal hands, feet, and

fingers and extensor arms and legs.

●● Variants include localized, generalized, perforating,

patch-type, subcutaneous, acute onset painful acral.

●● DD: Tinea corporis (itchy, central clearing, peripheral

papules, scales), leprosy (hypopigmented hypoesthetic

plaque).

Granuloma multiforme
●● This is a reactive skin disorder of unknown etiology,

characterized clinically by confluent annular plaques

and histologically by focal necrobiosis and histiocytic
granuloma.
●● Small papules evolve into round/oval plaques with
Figure 7.9  (a) Yellow, shiny plaques on upper and lower
raised active margin and depressed and hypopigmented eyelids in xanthelasma palpebrarum. (b) Bilaterally sym-
centers. metrical lesions in xanthelasma palpebrarum. (Continued)
 Plaques: Localized  155

●● DD: Erythema elevatum diutinum (yellow-orange

plaques appearing on elbow, knee, small joints of
hands, with leukocytoclastic histopathology), juvenile
xanthogranuloma (yellow papules/plaques, head-neck
region, histiocytic proliferation), nevus lipomatosus
cutaneous superficialis (soft, yellowish plaques, mainly
over buttocks/thighs).

Tumid lupus erythematosus9

●● This presents with single or multiple succulent, non-

scarring, erythematous plaques (Figure 7.10).

●● There is no follicular plugging as with discoid lupus

erythematosus (DLE).
●● Areas such as the face, neck, upper back, and forearms

are predominantly involved.

●● It heals spontaneously without atrophy, scarring, or

pigmentation.
●● DD: Lupus vulgaris (apple-jelly nodules, scarring

at one end and progression on another), sarcoidosis

(red/tan-brown plaques with naked granuloma),
Jessner’s lymphocytic infiltrate (erythematous
plaques over face).

Cellulitis
●● Predisposing factors include diabetes mellitus, HIV

infection/AIDS, chronic kidney disease, chronic liver

disease, and presence of foreign bodies, including
surgical devices.
●● It may develop after a trauma or may complicate a pre-

existing dermatosis.

Figure 7.9 (Continued)  (c) Extensive lesion of xanthelasma

palpebrarum extending beyond periocular area. Note the
presence of extensive senile comedones. (d) Tuberous
xanthomas on knuckles. (a–c – Courtesy: Dr. Piyush
Kumar, Katihar, India; d – Courtesy: Dr. Niharika Ranjan
Lal, ESIPGIMSR, Kolkata, India.)

●● Tendinous xanthoma is seen particularly on the Achilles

tendon and the dorsa of hands and feet.
●● Palmar xanthomas are plaques over the palm and
flexures of fingers, also yellow streaks along palmar
creases. They usually start as macules or slightly Figure 7.10  Erythematous, juicy plaque on face in tumid
palpable plaques; the condition is pathognomonic of lupus erythematosus. (Courtesy: Dr. Piyush Kumar,
type III/familial dysbetalipoproteinemia. Katihar, India.)
156  Plaques: Localized

Table 7.2  Differentiating cellulitis from erysipelas

Cellulitis Erysipelas
Presents with low-grade There is abrupt onset of
fever with a less abrupt fever accompanied by
onset. chills.
The skin appears brownish- Dark-skinned people may
black in dark-skinned have brownish-gray skin,
individuals and dull red while Caucasians may
in Caucasians. have bright-red skin.
The border of the infected The infected area is hot
area is less well defined and tender and spreads;
and fades into the the border is well-
surrounding skin. defined, and the surface
is shiny.
Blisters are uncommon. There are vesicles and bullae
(subcutaneous edema).
Cause is multibacterial, Group A haemolytic
such as staphylococci, streptococci is the main
streptococci, organism.
haemophilus.

HYPERKERATOTIC PLAQUES
Tuberculosis verrucosa cutis13
●● this is a localized form of cutaneous tuberculosis due to

inoculation of organisms into the skin of a previously

infected patient who usually has a moderate or high
degree of immunity.
●● Lesions are mostly seen on trauma-prone areas (e.g. feet,

knees, buttocks, etc.).

●● A lesion starts as an asymptomatic, indurated, warty

papule and progresses to verrucous plaque with finger-

like projections (Figure 7.12a–c).
Figure 7.11  (a) Ulcerated plaque in cellulitis in localized ●● Color is purplish, red, or brown. Consistency is generally
plaque. (b) Severe case of cellulitis skin hemorrhage firm, but there may be areas of relative softening.
and necrosis. (c) Another case of severe cellulitis with ●● The center may progress to massive, papillomatous
eroded surface. (a–c – Courtesy: Dr. Piyush Kumar,
excrescences or, uncommonly, may involute to leave a
Katihar, India.)
white atrophic scar.
●● The disease runs a chronic progressive course.

●● Patients may have malaise, chills, and fever. Lesions are

most commonly located on the lower extremity.
●● It presents as a painful ill-defined swelling of the
affected part. The skin appears erythematous and
warm. Regional lymphadenopathy is common
(Figure 7.11a–c).
●● Clinical signs of severe disease include violaceous
hue, presence of hemorrhage and bullae, skin
sloughing, sensory loss over the lesion, lymphatic
spread, rapid progression, and hemodynamic
instability.
●● DD: Erysipelas (Table 7.2), erysipeloid (polygonal
plaques, central clearing, peripheral extension, fingers),
urticaria (itchy, transient, evanescent edematous hives/ Figure 7.12  (a) Verrucous plaque in tuberculosis verru-
wheals). cosa cutis. (Continued)
 Plaques: Localized  157

Lupus vulgaris14
●● This is a chronic progressive primary form of cutaneous

tuberculosis
●● It consists of flat plaque composed of soft, reddish-

brown papules (Figure 7.13a) with irregular edges. The

surface of the lesion may be smooth or covered with
psoriasiform scale (Figure 7.13b–d).
●● There is apple-jelly appearance on diascopy.

Figure 7.12 (Continued)  (b) Solitary verrucous plaque of

tuberculosis verrucosa cutis. (c) Resolution of lesion with
antitubercular treatment. (a–c – Courtesy: Dr. Piyush
Kumar, Katihar, India.)

●● DD: Verruca vulgaris (multiple plaques, pseudo

Koebnerization), sporotrichosis (oozy plaque, lymphatic
spread), psoriasis (micaceous scales, fissures, Auspitz Figure 7.13  (a) Lupus vulgaris presenting as erythematous
sign, well-marginated), lupus vulgaris (gradual scaly plaque. Note scarring in the vicinity. (b) Erythematous,
extension at one margin, scarring in another margin). scaly plaque of lupus vulgaris on the buttock. (Continued)
158  Plaques: Localized

Figure 7.13 (Continued)  (c) Clinical resolution of lesion with treatment. Residual scarring is seen. (d) Erythematous scaly
plaque with crusting at places. (e,f) Lupus vulgaris with advancing edge showing hyperkeratotic plaque; inactive edge
healed with scarring. (a–d – Courtesy: Dr. Piyush Kumar, Katihar, India; e,f – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR,
Kolkata, India.)

●● There is gradual extension in some areas and healing ●● Morphological variants include ulcerative and
with scarring in other areas; it may cause tissue mutilating forms, vegetating forms, tumor like forms
destruction and deformity. Large plaques show irregular and papular-nodular forms.
areas of scarring with islands of active lupus tissue ●● DD: Tuberculosis verrucosa cutis (oozy plaque, areas
(Figure 7.13e,f). – sole, buttocks), sporotrichosis (lymphatic spread),
●● It is seen over face, extremities and trunk. sarcoidosis (red/brown plaque, naked granuloma).
 Plaques: Localized  159

Figure 7.14  (a) Erythematous crusted plaques in lymphocutaneous sporotrichosis. (b) Crusted plaques of sporotrichosis
localized to the dorsum of the hand. (a – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Sporotrichosis14 ●● Lesions are usually found on exposed sites, particularly

●● Sporotrichosis is an acute or chronic fungal infection feet, legs, arms, face, and neck.
caused by Sporothrix schenckii. ●● DD: Sporotrichosis (warty plaques, lymphatic spread),
●● Commonly affected populations include agriculturists, tuberculosis verrucosa cutis (oozy plaque, appears on
foresters, gardeners, florists, and nursery workers soles, buttocks), verruca vulgaris (warty plaque/papules,
handling plants or plant material. pseudo-Koebnerization), blastomycosis (by the absence
●● Traumatic inoculation is the typical mode for acquisition of sharp border containing minute abscesses).
of skin infection in immunocompetent hosts.
●● Clinically sporotrichosis manifests as 1)
Cutaneous Leishmaniasis (CL)
●● Leishmaniasis is a group of diseases caused by several
lymphocutaneous (most common type), 2) fixed
cutaneous, 3) multifocal or disseminated cutaneous, species of the genus Leishmania.
●● Most of the cases are from hot and dry areas like deserts
and 4) extracutaneous or systemic sporotrichosis.
●● The lymphocutaneous type is characterized by a of North and South America, the Mediterranean basin,
nodulo-ulcerative lesion (sporotrichotic chancre) at the
inoculation site and strings of similar lesions along the
lymphatics proximal to the initial lesion (Figure 7.14a).
●● Fixed cutaneous sporotrichosis is less common and

occurs as localized, asymptomatic, erythematous,

papulo-plaque, papulo-pustules, nodules or verrucous
plaque and occasionally, a non-healing ulcer or a small
abscess at the inoculation site (Figure 7.14b).
●● Multifocal (disseminated) cutaneous sporotrichosis (≥3

lesions involving two different anatomical sites) and

extracutaneous (systemic, osteoarticular, pulmonary,
meningeal) forms are very rare.
●● DD: Chromomycosis (warty papules, satellite lesions,

copper-penny bodies in histopathology), tuberculosis

verrucosa cutis (oozy plaque, appears on soles and
buttocks), verruca vulgaris (warty plaque/papules,
pseudo-Koebnerization), leishmaniasis, atypical
mycobacterial infection.

Chromomycosis (chromoblastomycosis)14
●● This is subcutaneous mycosis caused by traumatic

implantation of dematiaceous fungi.

●● Warty papules slowly enlarge to form painless

hyperkeratotic plaque, often with central scarring;

some forms may appear like psoriasiform plaques
(Figure 7.15a–c).
●● Satellite lesions and lymphatic spread to adjoining areas.

Large verrucous swellings and lymphedema develop in Figure 7.15  (a) Chromoblastomycosis. Warty
later stages. plaque. (Continued)
160  Plaques: Localized

Figure 7.15 (Continued)  (b) Chromoblastomycosis.

(c) Verrucous plaque of chromomycosis on the thigh and
leg. (a,b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR,
Kolkata, India.)

the Middle East, and Central Asia. In India, CL is

endemic to the Thar desert and neighboring areas of
Rajasthan.
●● Non-tender firm plaques or nodules form central
ulceration and crusting and a raised border
(Figure 7.16a–c). Lesions are wet or dry. The lesions heal
with fibrosis.
●● Variants include cutaneous, diffuse cutaneous,
mucocutaneous, leishmaniasis recidivans, and post
kala-azar dermal leishmaniasis.
●● In leishmaniasis recidivans (lupoid leishmaniasis)
brown-red or brown-yellow papules appear, usually close
to a scar of an old lesion of cutaneous leishmaniasis or
actually in the scar; these papules coalesce and form a
Figure 7.16  (a) Well -defined, erythematous crusted plaque
plaque closely resembling lupus vulgaris. of cutaneous leishmaniasis on the hand. (b) Two plaques of
●● DD: Tuberculosis verrucosa cutis (oozy plaque, appears cutaneous leishmaniasis on exposed sites. (c) Sporotrichoid
on soles and buttocks), sporotrichosis (warty plaques, spread of lesions in cutaneous leishmaniasis. (a–c – Courtesy:
lymphatic spread), lupus vulgaris (gradual extension at Prof. Reza Yaghoobi, Department of Dermatology, Ahvaz
one margin, scarring in another margin). Jundishupor University of Medical Sciences, Iran.)
 Plaques: Localized  161

Figure 7.17  (a) Erythematous, thick plaque on the dorsum of the foot, with prominent skin markings and excoriations
in lichen simplex chronicus. (b) Lichen simplex chronicus on the sacral area. Note lichenification of the surrounding
skin. (c) Lichen simplex chronicus over the nape of the neck. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Lichen simplex chronicus ●● They may be surrounded by follicular papules – an

●● Also known as neurodermatitis circumscripta, important clinical clue, when present.
lichen simplex chronicus is a chronic skin ●● Most often they appear on the pretibial area of the lower
disease characterized by lichenified plaques that extremity and the ankles. Lesions can also appear on
occur as result of constant scratching or rubbing of other areas of the lower limbs, upper limbs, trunk, or in
the skin. a generalized fashion.
●● The typical presentation of LSC is a circumscribed,

lichenified, pruritic plaque. Scales and excoriation are

often present (Figure 7.17a–c).
●● Typically located over the dorsum of the foot, nape of

the neck, wrist, and back.

●● DD: Tuberculosis verrucosa cutis (oozy plaque

that appears on soles and buttocks), sporotrichosis

(warty plaques, lymphatic spread), lichen planus
hypertrophicus (verrucous plaque with scaling on the
shins, with follicular papules in surroundings).

Lichen planus hypertrophicus

●● This is typically present as extremely pruritic red,

yellow-gray, or red-brown papules and verrucous,

f lat-topped plaques. They are firm to palpation
and display a verrucous or hyperkeratotic surface
(Figure 7.18a–c).
●● In dark skinned people, they often appear pigmented
Figure 7.18  (a) Hyperkeratotic verrucous plaque in lichen
with purplish to black in color. planus hypertrophicus. (Continued)
162  Plaques: Localized

Figure 7.18 (Continued)  (b) Lichen planus hypertrophicus.

(c) Lichen planus hypertrophicus. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India; c – Courtesy: Dr. Niharika
Ranjan Lal, ESIPGIMSR, Kolkata, India.)

●● DD: Prurigo nodularis, verruca vulgaris (warty plaques,

pseudo-Koebnerization), tuberculosis verrucosa cutis
(oozy plaque on the soles and buttocks), chronic plaque
psoriasis (micaceous scales, fissures, Auspitz sign, well-
marginated), pretibial epidermolysis bullosa pruriginosa.

Verruca vulgaris
●● This has a viral etiology caused by human papilloma

virus (HPV).
●● Hyperkeratotic papules/plaques with an irregular rough

surface occur on any part of the body (Figure 7.19a,b)

as solitary or multiple, endophytic, or exophytic lesions
(Figure 7.19c,d).
●● Although the lesions are usually 2–10 mm in diameter,

they can grow up to a giant verruca vulgaris, whose size

is larger than 1 cm.
●● Commonly found on the hands, acral areas, and

trauma-prone areas.
●● Pseudo-Koebnerization involves the spread of lesions to

the site of trauma (Figure 7.19e).

●● DD: Tuberculosis verrucosa cutis (oozy plaque on the

soles and buttocks), verrucous carcinoma (destructing

Figure 7.19  (a) Verrucous flat-topped papules coalesc-
surroundings, warty surface, bulldozing effect in ing to form plaque on the great toe in verruca vulgaris.
histopathology), lichen simplex chronicus (lichenified (b) Verrucous plaque on the thumb in verruca vulgaris.
plaque, caused by excoriations, occurring on the (c) Exophytic plaque on labia in condyloma acuminata.
dorsum of hands and feet, nape of neck). (Continued)
 Plaques: Localized  163

Table 7.3  Keloid and hypertrophic scar

Keloid Hypertrophic scar

Firm, smooth growth over Firm, red growth over
original wound but extends scar but does not cross
beyond wound area the original wound area
Itchy and claw-like Non-itchy and confined
projections; extends within scar and no
beyond months of wound projections; proliferates
up to six months
Happens in genetically Happens when there is
predisposed individual, lot of tension on
mainly over chest and healing wound
shoulders
Does not reduce on its own; Over several months scar
rather itching and becomes pale and flat
projections increase

Figure 7.19 (Continued)  (d) Flat-topped, verrucous

plaque showing pinpoint hemorrhagic points. (e) Warts
presenting multiple papules and plaques. Note pseudo-
Koebnerization. (a,b,d,e – Courtesy: Dr. Piyush Kumar,
Katihar, India; c – Courtesy: Dr. Niharika Ranjan Lal,
ESIPGIMSR, Kolkata, India.)

Benign lichenoid keratosis (lichen planus-like keratosis)

●● This is a common benign neoplasm of the skin.
●● Solitary, dusky-red to violaceous scaly patches occur on

the head and neck, trunk, or distal extremities; on the face

it may present as a solitary asymptomatic round lesion.
●● It may be rough or scaly in texture and can transition

from pink to violaceous to hyperpigmented as it regresses.

Multiple regression phases may be present within the lesion.
●● DD: Verruca vulgaris (warty plaques, pseudo-

Koebnerization), tuberculosis verrucosa cutis (oozy

plaque on the soles and buttocks), lichen planus
hypertrophicus (verrucous plaque with scaling on the
shins, with follicular papules in surroundings).

Keloids/hypertrophic scar
●● Keloids and hypertrophic scars are the result of aberrant

wound healing after injury to the reticular dermis

(Table 7.3).
●● Keloids are flesh-colored to reddish brown, firm

nodules (Figure 7.20a) and plaques with peripheral Figure 7.20  (a) Keloid. Smooth, shiny plaque on the
claw-like projections at sites of trauma or inflammation face. (b) Keloid. Claw-like projections extending beyond
(Figure 7.20b). plaque margin. (Continued)
164  Plaques: Localized

Figure 7.20 (Continued)  (c) Keloid. (d) Hypertrophic scar

at the site of trauma. (a–c – Courtesy: Dr. Niharika Ranjan
Lal, ESIPGIMSR, Kolkata, India; d – Courtesy: Dr. PC Das,
Katihar, India.)

●● Keloids spread beyond the margins of inciting trauma with loss of hair follicles and hyperpigmentation
even six months after the onset (Figure 7.20c). (Figure 7.21a–c).
●● In hypertrophic scarring there is also proliferation of ●● It is commonly seen over the trunk and
scar tissue but within six months of onset, and it doesn’t extremities.
spread beyond the margin of scar. It doesn’t have claw- ●● Other variants include morphea profundus, guttate
like extensions (Figure 7.20d). morphea, keloidal morphea, linear morphea,
●● DD: Lobomycosis (keloidal plaque/nodule, child’s pop- pansclerotic morphea, and generalized morphea.
beads–like arrangement of fungi), B-cell lymphoma ●● DD: Lichen sclerosus et atrophicus (ivory/white
(plum-colored nodules and plaques, follicular histology depressed papules, coalesced to form plaque,
with atypia and mitoses). genital and back), scar (wrinkled depressed surface,
collagen loss).
ATROPHIC PLAQUES
Lichen sclerosus et atrophicus (LSA)
Morphea ●● Whitish polygonal papules coalesce to form

●● Morphea is a localized form of scleroderma. plaques with follicular plugs on surface

●● It is characterized by oval or linear well-circumscribed (Figure 7.22a).
sclerotic lesions of varying sizes. ●● Older lesions are porcelain white and shiny

●● Morphea-en-plaque looks like circumscribed, (Figure 7.22b).

indurated, oval/round plaques surrounded by ●● Purpuric lesions may develop on the surface in active

a lilac-colored border that progresses to form lesions.

sclerotic, smooth, shiny, ivory-colored plaques, ●● It is asymptomatic, but genital lesions may itch.
 Plaques: Localized  165

Figure 7.21  (a) Morphea. Shiny, depigmented patch with

wrinkled surface. (b) Well-defined lesion of morphea
on the forearm. (c) Morphea lesion on the chest. (a–c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Variants include genital and extragenital. scaly patches, perifollicular area, appears on the
●● Extragenital LSA is most commonly seen on the back back, neck, and side of face), malignant atrophic
and shoulders. papulosis (depressed porcelain-white papules with
●● DD: Morphea (atrophic patch, shiny, wrinkled, livid red periphery, telangiectasia, ischemic infarcts in
tough skin), pityriasis versicolor (hypopigmented intestines, kidney).

Figure 7.22  (a) Shiny, atrophic plaque with prominent follicular opening in lichen sclerosus atrophicus. (b) Porcelain white
plaque with follicular prominence in lichen sclerosus atrophicus. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
166  Plaques: Localized

SCALY PLAQUES varying extents in regions rich in sebaceous glands,

such as the scalp (Figure 7.23a), the retro-auricular
Seborrheic dermatitis (SD)15 area (Figure 7.23b), face (nasolabial folds, upper
●● This is a common inflammatory condition that affect lip, eyelids Figure 7.23c and eyebrows), and the
the seborrheic areas of the body. upper chest. Distribution of the lesions is generally
●● SD often presents as well-delineated erythematous symmetrical. Truncal lesions may be oval and petaloid
plaques (or patches) with greasy, yellowish scales of scaly plaques.

Figure 7.23  (a) Greasy, yellow scales on the scalp in seborrheic dermatitis. (b) Scaling in the retroauricular area in sebor-
rheic dermatitis. (c) Seborrheic dermatitis presenting as scaling and erythema over the eyelid margin and a scaly plaque
near the eyebrow. (d) Greasy, crusted scales over the vertex of the scalp in an infant (cradle cap). (a,b,d –Courtesy: Dr.
Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India; c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Plaques: Localized  167

Table 7.4  Differential diagnosis of seborrheic dermatitis

Diagnosis Diagnostic Clues

Psoriasis Usually involves extensor, palmar, plantar, nails, and extensor areas. Thick plaques sharply limited
with silvery white scales. Positive family history. Arthritis is present in 10% of patients.
Uncommon in children.
Atopic Dermatitis First appearance after three months of age; pruritus and restlessness are common. Frequently
involves scalp, cheeks, and extensor areas. Flexures involvement is more frequent in older ages.
Family history of atopy such as eczema, allergic rhinitis, and asthma. Self-resolves by age 12.
Tinea Capitis Commonly seen in children, frequently accompanied by hair-loss patches with “black dots” (broken
hair). Highly contagious. KOH examination of the hair shaft and fungal culture confirm the
diagnosis. Household members of patient should be examined.
Rosacea Usually targets the face. Papulopustules and telangiectasias on the malar, nose, and perioral
regions with slight desquamation. Recurrent edema and flushing.
Systemic Lupus In acute stage, a butterfly rash on the face that spares the nose bridge or nasolabial folds.
Erythematosus Photosensitivity is common. Skin lesions are generally associated with other clinical signs of SLE.
(SLE) Histology and serologic tests, such as antinuclear autoantibodies, confirm the diagnosis.
Pemphigus Erythema, scaling, and crusting that first present on the scalp and face can expand to the chest and
Foliaceus back. Histology, direct immunofluorescence with anti-desmoglein antibodies confirm diagnosis.
Pityriasis Rosea Abrupt onset, appearance of herald patch, and resolution within weeks.
Secondary Syphilis Peripheral lymphadenopathy, mucosal lesions, and palmoplantar maculo-papules. Serology tests
such as VDRL/ RPR, FTA-ABS confirm diagnosis.
Diaper Dermatitis Occurs on convex skin surfaces in contact with diaper, such as lower abdomen, genitalia, buttocks,
and upper thighs. Spares skin folds. Pustules are common.
Langerhans Cell Multisystem disease. Brown to purplish papules prone to coalesce on the scalp, retro-auricular
Histiocytosis areas, axillae, and inguinal folds. Possible lytic bone lesions, liver, spleen, and lung involvement.
Histology confirms diagnosis.

●● In infants the most common form of SD is cradle cap. ●● Diseases like vitiligo and albinism with loss of
It appears as red-yellow plaques coated by thick, greasy protective melanocytes predispose the patient to
scales on the vertex, appearing within three months of develop AK.
birth (Figure 7.23d). ●● It is commonly seen over the nose, lips, bald scalp, and
●● DD – Table 7.4. extremities.

Herald patch of pityriasis rosea

●● This is an acute, asymptomatic or mildly pruritic

eruption.
●● It appears as single or multiple 2- to 5-cm oval plaque

with a fine collarette of scale inside the periphery of the

lesion (Figure 7.24).
●● Crops of similar but smaller lesions appear several

days to weeks later, arranged along the lines of cleavage

(“Christmas tree” appearance).
●● It resolves spontaneously after four to eight weeks.

●● DD: Small plaque parapsoriasis (scaly plaques, digitate

lesions over trunk), seborrheic dermatitis (scaly plaques,

seborrheic areas), secondary syphilis (palmoplantar
scaly lesions, VDRL +ve, rebound phenomenon).

Actinic keratosis (AK)

●● Actinic keratosis occurs as multiple, small, discrete
keratotic papules/plaques on sun-damaged skin
(Figure 7.25a–c).
●● It is mainly seen in tropical countries like Australia,
Figure 7.24  Herald patch of pityriasis rosea with collar-
New Zealand, and South Africa (skin color is lighter ette of scales surrounded by multiple daughter lesions.
and the UV rays are more intense). (Courtesy: Dr. Piyush Kumar, Katihar, India.)
168  Plaques: Localized

●● DD: Bowen’s disease (oozy, scaly plaque, wind-blown

appearance, atypical cells), squamous cell carcinoma
(fungating mass, atypical cells, mitoses).

Pityriasis rotunda
●● Pityriasis rotunda is disorder of keratinization

characterized by geometrically perfect circular

sharply defined patches of dry ichthyosiform
scaling.
●● Circular/oval, fixed, well-defined, scaly hypopigmented

or hyperpigmented plaques occur on the trunk and

extremities.
●● It is often associated with neoplasia (hepatocellular

carcinoma) and systemic diseases.

Figure 7.25  (a) Pink papules and plaques in early actinic

keratosis in an albino. (b) Multiple lesions of actinic
keratosis on a background of chronic photodamaged
skin. Note the relative absence of lesions on the left
chest which was usually covered by saree. (c) Closeup of
the lesions in the same patient. Note yellowish, thick-
ened photoaged skin on the right side of the neck. (a –
Courtesy: Dr. Piyush Kumar, Katihar, India; b,c – Courtesy:
Dr. Shekhar Neema, Associate Professor, Armed Forces
Medical College, Pune, India.)
 Plaques: Localized  169

●● DD: Tinea corporis (itchy, annular, scaly plaques with ●● Stucco keratoses: Multiple gray or white papules on
peripheral papules), parapsoriasis (digitate lesions, the dorsal aspect of lower extremities.
trunk), pityriasis rosea (collarette of scales, inverted fir ●● Melanoacanthoma: Large, pigmented variant.
tree appearance). ●● DD: Verrucous epidermal nevus (congenital, verrucous,
usually linear), verruca vulgaris (pseudo-Koebnerization,
PLAQUES WITHOUT SCALING warty plaque and papules), Bowenoid papulosis (genital,
warty papules and plaques, HPV infection, precancerous).
Pruritic urticarial papules and plaques of
pregnancy Mastocytoma
●● This condition is usually seen in primigravida in the ●● Mastocytosis is a group of disorders characterized

third trimester of pregnancy. by abnormal proliferation and accumulation of mast

●● Erythematous, urticarial papules and plaques occur on cells, involving the skin only (cutaneous mastocytosis)
the abdomen around the umbilicus. or the bone marrow and other extracutaneous organs
●● 1- to -2mm lesions appear within abdominal striae. (systemic mastocytosis).
●● DD: Urticaria (transient, evanescent, edematous ●● Mast cells produce mediators histamine, heparin,

plaque). tryptase, proteases, chymase, cytokines (tumor

necrosis factor and interleukins), chemokines to name
Seborrheic keratosis16 a few. These mediator-related symptoms are flushing,
●● Seborrheic keratoses are common, benign, pigmented itching, blistering, diarrhea, abdominal pain, vomiting,
epidermal tumors. hypotension, headache, and bone pain.
●● The condition is commonly seen in middle-aged to ●● Cutaneous mastocytosis presents as solitary or multiple

elderly people, especially of colored skin. skin-colored to yellowish papules or plaques, often with
●● Round to oval hyperpigmented macules evolve toward a peau d’orange appearance of the surface.
stuck-on brownish to black warty plaques with a greasy ●● Stroking or rubbing of lesions produces wheals

smooth surface showing plugged follicular orifices (Darier’s sign).

(Figure 7.26a,b). The lesions typically have a “stuck on” ●● Lesions occur most commonly on trunk, arm or neck.

appearance. ●● Bullae may occur over lesions (bullous mastocytosis).

●● They typically occur over the face and upper trunk. ●● Most lesions appear before one year of age and involute

●● Variants include the following: over several months to years.

●● Solar lentigo: flat, circumscribed, pigmented patches ●● DD: Erysipelas (peau d’orange, erythema, edema,

seen on sun-exposed areas. vesicles over limbs), urticarial wheals (transient,

●● Dermatosis papulosa nigra: small, sessile, black evanescent, edematous plaque), papular urticaria/insect
lesions on the head and neck. Common in bite hypersensitivity (erythema, papules and plaques,
colored skin. extremities).

Figure 7.26  (a) Seborrheic keratosis presenting as hyperkeratotic plaque showing plugged follicular orifices. (b) Seborrheic
keratosis on face. (a – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India; b – Courtesy: Dr. Piyush Kumar,
Katihar, India.)
170  Plaques: Localized

Melanocytic nevus ●● They typically develop during the first decade of life.
●● It can be congenital or acquired. ●● Other features of tuberous sclerosis, such as
●● Macules/plaques are dark-brown to black and may be angiofibroma and ash-leaf macules, are also seen.
hairy (Figure 7.27a). ●● DD: Collagenoma (trunk/buttocks, linear or
●● They can be small (<2 cm), medium (2–20 cm), or large zosteriform, single/multiple), xanthoma (yellow,
(>20 cm) (Figure 7.27b,c). Giant hairy nevi mostly hyperlipidemia).
occur on the trunk.
●● DD: Becker’s nevus (shoulder, hypertrichosis, after

puberty, hyperpigmentation, acneiform eruption),

acquired smooth-muscle hamartoma (elliptical,
lumbosacral area, hypertrichosis, smooth muscle
bundles at different angles), nevoid acanthosis nigricans
(verrucous, velvety, diffuse plaque).

Shagreen patch
●● It is a connective tissue hamartoma, found in

approximately half the patients with tuberous sclerosis

complex.
●● It presents as elevated pink to yellow-brown plaques

with an orange-peel–like texture, ranging from

several millimeters to several centimeters in length
(Figure 7.28).
●● Shagreen patches are asymmetric and usually appear on

dorsal surfaces, such as the back and the lumbosacral

regions, but occasionally occur on the chest or the
abdomen.

Figure 7.27  (a) Congenital melanocytic nevus on scalp. (b) Giant congenital melanocytic nevus on trunk. (c) Large congen-
ital melanocytic nevus on the lower back. Note lesional hypertrichosis and nodularity. (a,c – Courtesy: Dr. Piyush Kumar,
Katihar, India; b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
 Plaques: Localized  171

●● It is a slow-growing wavy, translucent nodule with

central ulceration (Figure 7.29a,b).
●● Pigmentation is a common finding (Figure 7.29c).
●● Crusting/bleeding may be seen.
●● Usually it is a solitary lesion.

Figure 7.28  Yellow soft plaque on the lumbosacral area in

tuberous sclerosis. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

ULCERATED PLAQUE
Basal cell carcinoma
●● Basal cell carcinoma (BCC) is the most commonly

occurring cancer in the world, generally a slow-growing

tumor for which metastases is rare.
●● Clinical features are dependent on the subtype of BCC.

Nodular or cystic BCCs present as raised red, pearly,

translucent lesions with peripheral telangiectasia.
Superficial BCC may mimic discoid eczema or Bowen’s
disease, while morphoeic BCC presents as a subtle scar-
like plaque.

Figure 7.29  (a) Pigmented, smooth-surfaced plaque of basal cell carcinoma with waxy, rolled border. (b) Large plaque
of basal cell carcinoma with central smooth surfaced, erythematous area and telangiectasia. The margin is typically
pigmented, waxy, and rolled. (c) Pigmented basal cell carcinoma with central ulceration and hemorrhagic crust. (a–c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)
172  Plaques: Localized

●● Typically appears over the head and neck region, such as ●● The lesions appear as yellow-brown, telangiectatic
nose, forehead, cheeks, periocular area, and ears. plaques with central atrophy and raised violaceous
●● It has a characteristic rolled border, and telangiectasia is borders. They occur most frequently on the shins or
seen over the lesion. the dorsa of the feet. Ulcers, which exist in about 30%
●● Variants include nodular, superficial spreading, of lesions, are often induced by trauma. In rare cases
morphoeic, fibroepithelioma of pinkus. squamous-cell carcinomas develop, typically in older,
●● DD: Porokeratosis (double-ridge with central crater, ulcerated lesions.
annular plaque, column parakeratosis), melanoma ●● DD: Psoriasis (micaceous scaly plaque, Auspitz sign,
(darkly pigmented, ulceration, mainly over feet in the extensors), morphea (atrophic, sclerosis, thickened
Indian subcontinent), seborrheic keratosis (verrucous, surface).
pedunculated/stuck on, pigmented).
Bowen’s disease
Necrobiosis lipoidica diabeticorum ●● It is an in situ squamous cell carcinoma (SCC).

●● Necrobiosis lipoidica diabeticorum is a chronic ●● Clinically a typical case of Bowen’s disease is a slowly

granulomatous dermatitis of unknown cause that is enlarging erythematous patch or plaque (Figure 7.30a)
most often associated with diabetes mellitus. that is well demarcated and has a scaling or crusted

Figure 7.30  (a) Erythematous scaly plaque in Bowen’s disease.

(b) Well-demarcated, crusted, erythematous plaque in Bowen’s
disease. (c) Bowen’s disease on the forearm. (d) Perianal
Bowen’s disease. (e) Nodules of squamous cell carcinoma devel-
oping within the lesion of Bowen’s disease. (a–c – Courtesy: Dr.
Piyush Kumar, Katihar, India; d – Courtesy: Dr. Niharika Ranjan
Lal, ESIPGIMSR, Kolkata, India; e – Courtesy: Dr. Ashutosh
  Ranjan, Consultant Dermatologist, Chhapra, India.)
 Plaques: Localized  173

surface (Figure 7.30b,c). In some cases it can be Paget’s disease

pigmented or verrucous. ●● Paget’s is an uncommon cutaneous intraepithelial
●● It is commonly located on the lower limbs and on adenocarcinoma involving the epidermis and extending
the head and neck; it may also be seen subungual into the dermis.
or periungual, palmar, genital, and perianal ●● There are slow-growing well-defined, erythematous
(Figure 7.30d). plaques with crusting resembles eczema
●● Frank SCC may develop (Figure 7.30e). (Figure 7.32a,b).
●● Multiple lesions are seen in association with chronic ●● An irregular, slightly raised border is unilaterally seen
arsenicosis. on nipple, areola, and adjoining skin of the breast.
●● DD: Psoriasis (micaceous scales, Auspitz sign, ●● It may cause retraction or destruction of nipple.
extensors), Paget’s disease (oozy plaques, crusting,
breasts, vulva).

Pemphigus vegetans
●● Vesicles or bullae evolve into hypertrophic granulating,

fissured plaques with erosions. Edges studded with

pustules (Figure 7.31).
●● Seen mainly in flexures (i.e., axilla, groin). Mucosal

erosions and hyperkeratotic tongue are seen.

●● DD: Darier-White disease (dirty, warty papules,

flexures, dyskeratosis), intertrigo (ill-defined plaques,

itchy, bacterial-fungal-dermatitis etiology), Hailey-
Hailey disease (oozy, crusts over flexures, dilapidated
brick wall, dyskeratosis).

Figure 7.32  (a) Paget’s disease presenting as crusted

Figure 7.31  Macerated plaques with fissures in axilla plaque on the nipple. Note partial destruction of the
in pemphigus vegetans. (Courtesy: Dr. Piyush Kumar, nipple. (b) Large lesion of Paget’s disease with complete
Katihar, India.) loss of nipple. (Continued)
174  Plaques: Localized

Figure 7.32 (Continued)  (c) Extramammary Paget’s disease in

perianal area. (a – Courtesy: Dr. Piyush Kumar, Katihar, India;
b – Courtesy: Dr Rajeev Ranjan, Ara, India; c – Courtesy: Dr.
Santoshdev Rathod, SVPIMSR, Smt. NHL Municipal Medical
College, V.S. Hospital, Ahmedabad, India.)

●● Subjacent breast lump may be palpated. Figure 7.33  Nevus sebaceous presenting as yellow-brown
●● Extramammary cases can be seen on vulva, perianal alopecic plaque on scalp with velvety surface. (Courtesy:
area (Figure 7.32c), scrotum, penis, and axilla. Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
●● DD: Bowen’s disease (well-defined plaques, crusts,
oozing, disarray of atypical cells), nipple eczema (diffuse
plaque, usually bilateral), nevoid hyperkeratosis of Verrucous epidermal nevus17, 18
nipple (congenital, scaly plaques), neurodermatitis, ●● Verrucous epidermal nevi (VEN) are non-inflammatory

periorificial tuberculosis, lichen simplex, psoriasis, keratinocytic hamartomas composed of keratinocytes

lichen planus, mycosis fungoides, and seborrheic ●● Skin-colored to dirty-brown or black verrucous papules

dermatitis. coalesce to form linear plaques along the lines of

Blaschko (Figure 7.34a,b).
●● They usually appear at birth or early childhood.

CONGENITAL PLAQUES ●● In systematized VEN, many linear lesions are limited to one

side of the body (Nevus unius lateris, Figure 7.34c) or have a

Milia-en-plaque bilateral symmetrical distribution (ichthyosis hystrix).
●● This appears as plaques of keratin-filled cysts in the

preauricular or periorbital area.

●● There is superficial, pearly white/yellowish, dome-

shaped, 1–2 mm papular coalescence.

●● DD: Nevus sebaceous (verrucous at puberty, sebaceous

proliferation).

Nevus sebaceous17, 18
●● It is a congenital hamartomatous lesion with an

epithelial and adnexal origin.

●● Solitary, hairless, linear or rounded, slightly elevated

yellowish plaques with a velvety surface (Figure 7.33)

occur.
●● It becomes verrucous at puberty.

●● Plaques usually appear on the scalp or face.

●● Appendageal tumors, such as syringocystadenoma

papilliferum, may develop in later life.

●● DD: Verrucous epidermal nevus (verrucous

from onset, hairs inside the structure),

seborrheic keratosis (verrucous, brown/black, Figure 7.34  (a) Hyperpigmented verrucous papules coalesc-
sun-exposed area). ing to form plaque in verrucous epidermal nevus. (Continued)
 Plaques: Localized  175

Figure 7.34 (Continued)  (b) Verrucous epidermal nevus

along the lines of Blaschko. (c) Nevus unius lateralis. (a–c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● DD: Nevus sebaceous (verrucous at puberty, sebaceous

proliferation), linear Darier, linear verruca vulgaris,
verrucous stage of incontinentia pigmenti.

Nevus lipomatosis superficialis

●● It is a rare hamartoma of adipose tissue.
Figure 7.35  (a) Grouped, smooth-surfaced, yellowish
●● It is divided into classical form and solitary form. It
plaques and papulonodules in a case of nevus lipoma-
usually presents as a clustered group of soft, fleshy, skin- tosus superficialis. (b) Large lesion of nevus lipomatosus
colored or yellowish nodules, which are either sessile superficialis on the forearm. (a – Courtesy: Dr. Piyush
or pedunculated growths with a smooth, wrinkled or Kumar, Katihar, India; b – Courtesy: Dr. Niharika Ranjan
cerebriform surface (Figure 7.35a,b). Lal, ESIPGIMSR, Kolkata, India.)
176  Plaques: Localized

●● The most common sites are pelvic girdle, buttocks, back more components of dermis are altered. Based on the
or abdomen. They are usually present at birth or emerge predominant tissue involved, these may be classified as
during the first two decades of life. collagenomas (collagen), elastomas (elastin), or nevus
●● DD: Connective tissue nevus (light yellow-orange plaques, mucinosis (proteoglycans).
congenital, lumbosacral area, linear or zosteriform, ●● Collagenomas may be acquired (eruptive and sporadic
shagreen leather appearance), xanthoma (yellowish type) or inherited (dermatofibrosis lenticularis
plaques, lipid laden macrophages, hyperlipidemia). disseminata in the Buschke-Ollendorf syndrome,
familial cutaneous collagenoma (FCC), and shagreen
Inflammatory linear verrucous epidermal nevus patches seen in tuberous sclerosis).
(ILVEN)17, 18 ●● The nevus is 1–15 cm diameter, light-yellow to
●● Extremely pruritic papules occur in linear streaks along orange, with the surface resembling shagreen
Blaschko’s lines (Figure 7.36). leather (Figure 7.37).
●● They appear mainly on the lower extremities. ●● Present on trunk, mainly lumbosacral area.
●● They are more common in girls; they may be congenital ●● If multiple, they may show in linear or zosteriform
or occur later. arrangement.
●● They are resistant to therapy. ●● The histopathology of collagenoma shows increased
●● DD: Verrucous epidermal nevus (non-itchy, verrucous amount of collagen fibers, with either normal or
plaques), nevus sebaceous (head and neck region, decreased elastic tissue.
verrucous after puberty), seborrheic keratosis ●● DD: nevus lipomatosus (plaques or tumoral
(verrucous, brown/black, sun-exposed area). mass, back/buttocks, peau d’orange appearance),
xanthoma (yellowish plaques, lipid laden
Connective tissue nevus19 macrophages, hyperlipidemia).
●● Connective tissue nevus are circumscribed ●● Certain elastic tissue nevi that may present as localized
hamartomatous malformations in which one or plaque are cutaneous features in Buschke Ollendorff
syndrome and inherited pseudoxanthoma elasticum.

Verrucous hemangioma
●● It is a rare form of vascular malformation that is usually

congenital.
●● The lesions appear as well-circumscribed erythematous

bluish macules that increase in size with time, acquiring

an erythematous violet color and the surface becoming
verrucous and hyperkeratotic (Figure 7.38).
●● They usually occur over the lower extremities.

●● They may bleed on trauma.

●● DD: Angiokeratoma circumscriptum neviforme

(congenital, hyperkeratotic, bluish-red, well-defined,

Figure 7.36  Linear erythematous scaly plaque on the Figure 7.37  Grouped, firm, plaques and papules of col-
neck in a case of inflammatory linear verrucous epidermal lagenoma. (Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR,
nevus. (Courtesy: Dr. Piyush Kumar, Katihar, India.) Kolkata, India.)
 Plaques: Localized  177

●● Ulceration, bleeding may occur.

●● They may cause life-threatening complications if
they occur over vital organs or dangerous areas like
the eye, ear, nose, or throat (Figure 7.39c,d).

Figure 7.38  Violaceous, hyperkeratotic plaque in verrucous

hemangioma. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

capillary/venous proliferation in dermal or

subcutaneous planes), lichen simplex, prurigo nodularis.

Hemangioma20
●● Hemangiomas are vascular neoplasms caused by the

abnormal proliferation of endothelial cells and aberrant

blood vessels architecture.
●● In infantile hemangioma (IH) risk factors include

female gender, prematurity, low birth weight, multiple

pregnancies, advanced maternal age, and in vitro
fertilization.
●● They mostly affect the head and neck region; they
can be classified into superficial, deep, and mixed.
●● Superficial hemangiomas, when fully formed, are
characterized by bright-red vascular plaques or
nodules. Deep haemangiomas manifest as partially
compressible, subcutaneous, bluish vascular
swellings. Mixed haemangiomas have both Figure 7.39  (a) Infantile hemangioma on abdomen.
superficial and deep components (Figure 7.39a,b). (b) Hemangioma. (c) Hemangioma over the lip. (Continued)
178  Plaques: Localized

●● Congenital hemangiomas are present and fully formed

at birth; they do not exhibit the postnatal proliferative
phase characteristic of IH. The two variants are the
noninvoluting congenital hemangioma (NICH) and
rapidly involuting congenital hemangioma (RICH).
●● They usually present with exophytic masses or
plaques on different parts of the body, such as the
head, limbs, or neck.
●● Noninvoluting congenital hemangiomas (NICHs)
present as well-circumscribed, oval, plaque-like masses
containing overlying telangiectasias with a rim of pallor;
they remain stable without growth or involution.
●● Rapidly involuting congenital hemangiomas
(RICHs) undergo a rapid involution phase beginning
Figure 7.39 (Continued)  (d) Grouped, smooth-surfaced ery- in the first year of life; they typically occur within
thematous papules and plaques in infantile hemangioma. (a,d –
the head, neck, and lower extremity regions.
Courtesy: Dr. Piyush Kumar, Katihar, India; b,c – Courtesy:
Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
●● DD: infantile hemangioma (histology shows lack of
glucose transporter 1 in congenital hemangioma).
●● IH has a characteristic natural course: a rapid Additional image – Figures 7.40 and 7.41.
proliferative phase in infancy that is followed by
a gradual involutional phase over the next several
years of life.
●● DD: Congenital hemangioma, venous malformation
(congenital, red/bluish-red, does not change over
time), Kaposi sarcoma (plaques/tumors, rubbery
consistency, brawny edema over the leg, may be
associated with AIDS).

Figure 7.40  Squamous cell carcinoma (SCC) presenting as

giant, pigmented verrucous plaque with focal ulcerations.
This SCC developed in a pre-existing lesion neglected for Figure 7.41  Amelanotic melanoma seen as erythema-
years. The pre-existing lesion could not be determined, and tous scaly and crusted plaque. (Courtesy: Dr. Tanumay
the patient was lost to follow-up. (Courtesy: Dr. Rajesh Kumar Raychaudhury, Skin and Cancer Foundation, The
Mandal, North Bengal Medical College, Darjeeling, India.) University of Sydney, Australia.)
 Plaques: Localized  179

REFERENCES 11. Karadağ AS, Parish LC. Sarcoidosis: A great imitator. Clin Dermatol
2019;37(3):240–254.
1. Lipsker D. Clinical Examination and Differential Diagnosis of Skin 12. Rudikoff D. Differential diagnosis of round or discoid lesions. Clin
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 35. Dermatol 2011;29(5):489–497.
Palpable Erythematous Lesions. P. 195–200. 13. Chen Q, Chen W, Hao F. Cutaneous tuberculosis: A great imitator.
2. Lipsker D. Clinical Examination and Differential Diagnosis of Skin Clin Dermatol 2019;37(3):192–199.
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 31. 14. La Hoz RM, Baddley JW. Subcutaneous fungal infections. Curr
Skin-Colored Palpable Lesions. P. 185–188. Infect Dis Rep 2012;14(5):530–539.
3. Lipsker D. Clinical Examination and Differential Diagnosis of Skin 15. Sampaio AL, Mameri AC, Vargas TJ, Ramos-e-Silva M, Nunes
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 32. AP, Carneiro SC. Seborrheic dermatitis. An Bras Dermatol
Brown, Black, Blue, or Gray Palpable Lesions. P. 189–190. 2011;86(6):1061–1074.
4. Lipsker D. Clinical Examination and Differential Diagnosis of Skin 16. Hafner C, Vogt T. Seborrheic keratosis. J Dtsch Dermatol Ges
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 33. 2008;6(8):664–677.
White Palpable Lesions. P. 191–192. 17. Garcias-Ladaria J, CuadradoRosón M, Pascual-López M. Epidermal
5. Lipsker D. Clinical Examination and Differential Diagnosis of Skin nevi and related syndromes – Part 1: Keratinocytic nevi. Actas
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 34. Dermosifiliogr 2018;109(8):677–686.
Yellow Palpable Lesions. P. 193–194. 18. Garcias-Ladaria J, CuadradoRosón M, Pascual-López M. Epidermal
6. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: nevi and related syndromes – Part 2: Nevi derived from adnexal
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. structures. Actas Dermosifiliogr 2018;109(8):687–698.
Chapter 8. Plaques with Scale. P. 113–122. 19. Arora H, Falto-Aizpurua L, Cortés-Fernandez A, Choudhary S,
7. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: Romanelli P. Connective tissue nevi: A review of the literature. Am
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. J Dermatopathol 2017;39(5):325–341.
Chapter 28. Papillomatous and Verrucous Lesions. P. 461–476. 20. Johnson EF, Davis DM, Tollefson MM, Fritchie K, Gibson LE.
8. Talhari C, Talhari S, Penna GO. Clinical aspects of leprosy. Clin Vascular tumors in infants: Case report and review of clinical, his-
Dermatol 2015;33(1):26–37. topathologic, and immunohistochemical characteristics of infantile
9. Filotico R, Mastrandrea V. Cutaneous lupus erythemato- hemangioma, pyogenic granuloma, noninvoluting congenital
sus: Clinico-pathologic correlation. G Ital Dermatol Venereol hemangioma, tufted angioma, and kaposiform hemangioendothe-
2018;153(2):216–229. lioma. Am J Dermatopathol 2018;40(4):231–239.
10. Mitteldorf C, Kempf W. Cutaneous Pseudolymphoma. Surg Pathol
Clin 2017;10(2):455–476.
 8
Plaques: Generalized

DHIRAJ KUMAR

INTRODUCTION Rupioid psoriasis

●● The term rupioid has been used to describe well-
Generalized plaques are seen in a wide range of dermato- demarcated, cone-shaped plaques with thick, dark,
logical diseases. Clinical clues that aid in diagnosis include lamellate, and adherent crusts on the skin that
symptoms (e.g., systemic features such as fever and mal- somewhat resemble oyster or limpet shells.
aise are seen in Sweet syndrome, lepra reaction, erythema ●● DD: reactive arthritis, disseminated histoplasmosis,
multiforme and urticarial vasculitis to name a few), color secondary syphilis, and photosensitive skin lesions in
of the lesions (erythematous, violaceous, yellow, hyperpig- association with aminoaciduria.
mented), surface changes (scaly, verrucous, velvety, edema-
tous, ulcerated, etc.), nature of scales (silvery, greasy, fine,
Pityriasis rubra pilaris
coarse), and shape of lesions (linear, annular), among oth-
●● Classical adult-onset PRP
ers. Also, it is important to recognize plaques developing
because of dermal changes including infiltration. Very often, ●● This is characterized by salmon-colored follicular
such plaques have thin, stretched, shiny epidermis and are hyperkeratosis on an erythematous base, often
better felt than seen. Notable examples include granuloma prominent on the backs of fingers.
annulare, sarcoidosis, Hansen’s disease, hypertrophic scar, ●● There is a predilection for the side of neck, trunk,
morphea, lichen sclerosus et atrophicus, etc. This chapter and extensor aspect of extremities.
discusses the clinical approach to diseases presenting with ●● It classically manifests first by scaling and erythema
generalized plaques (outlined in Table 8.1), followed by a of the scalp; it spreads in a cephalocaudal direction.
brief description of entities.1–7 ●● Papules are acuminate, reddish-brown with a
central horny plug, giving a nutmeg-grater feel.
●● These papules coalesce to form large red-to-orange-
Chronic plaque psoriasis red plaques that are often weeping, with distinctive
●● Chronic plaque psoriasis presents with well-defined, “islands of sparing” (nappes claires) (Figure 8.2a,b).
erythematous, circumscribed, dry scaly plaques of ●● It is associated with palmoplantar hyperkeratosis
various size with easily detachable, silvery white (yellow-orange waxy palmoplantar keratoderma).
lamellate scales on the surface (Figure 8.1a). Removal of ●● DD: Psoriasis (micaceous scales, Auspitz
scales may reveal bleeding points (Auspitz sign). sign, extensors), progressive symmetric
●● The eruption is usually symmetrical. It shows a erythrokeratoderma (autosomal dominant, sparing
predilection for the scalp, umbilical region, sacrum, of trunk), subacute cutaneous lupus erythematosus
and extensor aspect of extremities (Figure 8.1b). Nail (common in females, sun-exposed areas, and
involvement shows pitting, oil spot, salmon patch, polycyclic annular lesion with thin scales.).
subungual hyperkeratosis. ●● Juvenile-onset PRP

●● DD: Pityriasis rubra pilaris (peculiar yellow and ●● This can be classical (generalized) or circumscribed.
salmon-colored papules and plaques with moderate ●● Generalized form: The appearance is similar to
scale, nutmeg-grater feel, small islands of normal skin), adults with prominent thickening of the skin of the
small plaque parapsoriasis (fine scaly, finger-like plaques palms and soles.
on flanks), psoriasiform syphilid (copper-colored ●● Circumscribed form: The most common features
papules, rebound tenderness, condyloma lata, VDRL are well-circumscribed plaques of erythema and
positive, mucosal lesions), Reiter’s disease (reactive follicular hyperkeratosis occurring on elbows and
arthritis) (Figure 8.1c). knees.
180 DOI: 10.1201/9781351054225-16
 Plaques: Generalized  181

Table 8.1  Clinical approach to diseases presenting with generalized plaques

Feature Additional clues Diseases

Erythematous Scaly • Profuse silvery scales – psoriasis vulgaris
• Heaped up scales – rupioid psoriasis
• Adherent scales (predominantly photo distributed) – generalized chronic
cutaneous lupus erythematosus, subacute lupus erythematosus
• Mild scales with follicular hyperkeratosis – pityriasis rubra pilaris
• Others – Papulosquamous syphilid, parapsoriasis, progressive symmetric
erythrokeratoderma
Non-scaly • Edematous plaques – urticaria, urticarial vasculitis, urticaria pigmentosa,
urticarial phase of bullous pemphigoid, pruritic polymorphous papules
and plaques of pregnancy (PUPPP)
• Infiltrative plaques – sarcoidosis, lepromatous leprosy, borderline
lepromatous leprosy
Annular plaques Scaly Figurate erythema, tinea corporis
Non-scaly Annular urticaria, disseminated granuloma annulare, erythema
marginatum
Atrophic Generalized morphea, generalized lichen sclerosus et atrophicus
Hyperkeratotic Warty plaques Darier disease, epidermodysplasia verruciformis (EDV)
Along lines of Blaschko Systemized verrucous epidermal nevus, verrucous stage of incontinentia
pigmenti
Velvety soft Generalized acanthosis nigricans
Others Seborrheic keratosis
Ulcerated Kaposi sarcoma, vegetative pyoderma gangrenosum, necrotic erythema
nodosum leprosum, mycosis fungoides, disseminated leishmaniasis
Yellow Necrobiotic xanthogranuloma
Crusted plaques Norwegian scabies, Sulzberger-Garbe disease

Figure 8.1  (a) Erythematous plaques with silvery scales in psoriasis vulgaris. (b) Psoriasis vulgaris lesions on the
back. (Continued)
182  Plaques: Generalized

Figure 8.1 (Continued)  (c) Erythematous, scaly and

crusted, papules and plaques in reactive arthritis. (a–c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Atypical Juvenile PRP

●● Follicular hyperkeratosis is a prominent feature
with keratoderma. Erythema with scleroderma like
changes of hands and feet are seen.
●● Onset is in the first years of life.
Leprosy
●● Leprosy has varied manifestations depending on the

underlying cell-mediated immunity and degree of

proliferation of Mycobacterium leprae.
●● Borderline tuberculoid (BT) leprosy: There are a few,

asymmetrical, usually well-demarcated lesions with a Figure 8.2  (a) Generalized pityriasis rubra pilaris with skin
somewhat dry surface. They may be annular in shape, areas. (b) Close-up of lesions showing discrete keratotic
with a clearly defined outer border (Figure 8.3a). The papules near the margin of erythematous plaque. (a,b –
surface may be scaly, with marked sensory impairment Courtesy: Dr. Piyush Kumar, Katihar, India.)
and hair loss. Peripheral nerve involvement is
widespread or asymmetrical. nodules, and plaques with sloping edges. Sensation and
●● Mid-borderline (BB) leprosy: Presents with some, sweating over individual lesion are slightly affected. The
asymmetrical, less-well–demarcated, somewhat shiny nerve is enlarged and symmetrical (Figure 8.3b).
lesions, often annular with a characteristic, punched-out ●● Lepromatous (LL) leprosy: There are multiple, poorly
appearance. (The outer border is vague, while the inner defined, erythematous macules, papules, nodules,
border is clearly defined.) There is moderate sensory and plaques. They are symmetric in distribution, and
impairment and hair loss over lesions. Peripheral nerve common sites of involvement are the face, buttocks, and
involvement is widespread and asymmetrical. lower extremities, with gloves-and-stocking anesthesia.
●● Borderline lepromatous (BL) leprosy: There are many, Additional late signs are madarosis, saddle nose, and
roughly symmetrically distributed macules, papules, mega lobule of ear (Figure 8.3c,d).
 Plaques: Generalized  183

Figure 8.3  (a) Generalized papules and plaques in borderline tuberculoid leprosy. Plaques are erythematous, annular, and dry
with visible hair loss. (b) Copper-colored infiltrative plaques in borderline lepromatous leprosy. Annular punched-out lesions
resembling “inverted saucer” (marked with an arrow). (c) Erythematous, mildly scaly, infiltrative plaques with ulceration in lep-
romatous leprosy. (d) Lepromatous leprosy with infiltrative plaques on hands. (a – Courtesy: Dr. Piyush Kumar, Katihar, India;
b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Joka, Kolkata, India; c–d – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● DD: Sarcoidosis (red or tan-brown, naked granuloma ●● It involves follicular plugging, keratotic spikes under
in histopathology), tinea corporis (central clearing, the scales (carpet-tack sign), and wide follicular pits
peripheral papules and scales), granuloma annulare in ears.
(annular plaque, peripheral discrete papules). ●● It results in cicatricial alopecia when the scalp is
involved.
Chronic cutaneous lupus erythematosus (discoid ●● Sites of predilection include chronic sun-exposed areas:
lupus erythematosus) scalp, forehead, cheeks, ears, nose, upper lip, and chin.
●● This condition presents well-defined, erythematous In generalized chronic cutaneous lupus erythematosus,
plaques with follicular hyperkeratotic surface, lesions are present below the head and neck.
telangiectasias and adherent scales that heal with ●● DD: Psoriasis (micaceous scales, Auspitz sign,
scarring, peripheral hyperpigmentation, and central extensors), lichen planus (pruritic, purplish, flat-topped
hypopigmentation (Figure 8.4a–f). papules and plaques, flexures).
184  Plaques: Generalized

Subacute cutaneous lupus erythematosus ●● There are symmetrical widespread non-scarring

●● Photosensitive dermatosis commonly noted in females. erythematous patches and plaques with a tendency
●● Two types can present with generalized plaques: to confluence (psoriasiform). The lesions vary from
papulosquamous and annular. pink to red, covered with thin scales (Figure 8.5a).
●● Papulo-squamous type: May be psoriasiform or ●● It usually appears on light-exposed areas such as the
eczematous. trunk and arms.

Figure 8.4  (a) Generalized chronic cutaneous lupus erythematosus. Lesions extending below head and neck. Violaceous
plaques with adherent scales. (b) Generalized chronic cutaneous lupus erythematosus. (c) Erythematous plaques with
central adherent scales and hyperpigmented borders in chronic cutaneous lupus erythematosus. Central atrophy charac-
terized by wrinkled surface. (d) Well-developed lesions of discoid lupus erythematosus. (Continued)
 Plaques: Generalized  185

Figure 8.5  (a) Psoriasiform lesions of subacute lupus ery-

thematosus. (b) Annular erythematous plaques with mild
scales in subacute lupus erythematosus. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

●● Annular or target-like type: The plaques are annular in

configuration, with raised pink-red borders and central
clearing; rarely they may be erythema multiforme-like
with blisters (Rowell Syndrome) (Figure 8.5b).
●● Photosensitivity and arthritis are frequently associated.
●● DD: Photosensitive eczema (pruritus, lichenification),
tinea corporis (center clearing, peripheral papules,
scales), psoriasis (micaceous scales, Auspitz sign,
extensors), photolichenoid drug eruption.

Sweet syndrome
●● Sweet syndrome is an uncommon disease having a
Figure 8.4 (Continued)  (e) Generalized pigmented, scaly female preponderance.
papules and plaques of generalized discoid lupus erythe- ●● The association with infections, autoimmune diseases,
matosus in an elderly female. (f) Preferential involvement inflammatory bowel disease and malignancies, as
of extensor surface of upper extremities in discoid lupus
well as the greater incidence in women, suggests a
erythematosus; some lesions have verrucous surface.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; c – Courtesy:
hypersensitivity reaction.
●● An upper respiratory tract infection or flu-like
Dr. Niharika Ranjan Lal, ESIPGIMSR, Joka, Kolkata, India;
d – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India; illness frequently precedes the development of the
e–f – Courtesy: Dr. Piyush Kumar, Katihar, India.) syndrome.
186  Plaques: Generalized

●● It shows succulent plaques, as large as 10–15 cm, with may be slightly hyperpigmented and depressed.
irregular borders also known as rocky island pattern; Sometimes, in giant form, lesions may be as large as
sometimes there is an illusion of vesiculation – plaques 20 cm (Figure 8.6).
look vesicular but are solid when pressed; they may be ●● Generalized GA has a later age of onset, poorer response
dotted with pustules. to therapy, and predilection for the trunk and/or
●● Face, neck, upper trunk, and extremities are common extremities.
sites; oral lesions are seen in 20% of cases; more ●● Variants
common in drug-related cases. ●● Localized: papules
●● It includes systemic symptoms such as fever, arthralgia, ●● Perforating: papules
and myalgia. ●● Patch-type: patches
●● It involves peripheral leukocytosis and elevated ESR. ●● Subcutaneous: nodules
●● DD: Erythema multiforme (target lesion, oral lesion), ●● DD: Sarcoidosis (red or tan-brown plaques, naked
erythema nodosum, leukemic infiltrates, erythema granuloma), mycosis fungoides, lichen planus (pruritus,
elevatum diutinum (more chronic). violaceous plaque, Wickham’s striae), tinea corporis
(central clearing, peripheral papules, scales, KOH
Sarcoidosis mount), leprosy (hypopigmented hypoesthetic plaque,
●● Sarcoidosis is a systemic granulomatous disorder of thickened nerve, AFB present).
unknown origin that most commonly involves the
lungs; cutaneous manifestations are seen in up to one-
third of patients and may be the first clinical sign of
disease.
●● Red-brown to violaceous papules and plaques appear

most often on the face, in particular the nose, neck,

upper back and extremities. Upon diascopy, lesions
show the color of “apple jelly.”
●● The plaque form shows characteristically diffuse

lesions and are of irregular shape with more superficial

nodules superimposed, sometimes having a crescentic
or serpiginous outline, chiefly involving shoulders and
lower limbs.
●● Individual plaques can develop central clearing, leading

to an annular configuration, or they can contain

prominent telangiectasias (angiolupoid form).
●● Specific cutaneous lesions are mostly asymptomatic.

●● In lupus pernio, large bluish-red and dusky violaceous

nodules and plaques occur symmetrically on the nose,

cheeks, ears, fingers, hands, and toes; the lesions may
feel soft, doughy, or indurated. The surface of the
lesion is often glistening. Ulceration and mutilation
are rare. Involved ear lobes may become massive
(“turkey ears”).
●● Lichenoid, hypopigmented, ichthyosiform,

psoriasiform, and verrucous variants of sarcoidosis

present as plaque.
●● DD: Leprosy (hypopigmented, hypoesthetic

patches), psoriasis (micaceous scales, Auspitz sign,

extensors), granulomatous secondary syphilis
(erythematous non-scaly plaque, mucosal lesion,
positive VDRL test).

Generalized granuloma annulare (GA)

●● Granuloma annulare is a benign, usually self-limiting,

cutaneous disease that presents as arciform or annular

plaques.
●● The plaques may be skin-colored, pink, or violaceous
Figure 8.6.  Annular, arcuate erythematous plaques on the
in color and are formed by small papules measuring a back in disseminated granuloma annulare. (Courtesy:
few millimeters in diameter. The center of the lesion Dr. Piyush Kumar, Katihar, India.)
 Plaques: Generalized  187

Granuloma multiforme ●● Classic Woringer–Kolopp pagetoid reticulosis: One

●● This is a reactive skin disorder of unknown etiology, or few acral plaques sharply bordered, scaly, stable.
characterized clinically by confluent annular plaques ●● Disseminated Ketron–Goodman pagetoid
and histologically by focal necrobiosis and histiocytic reticulosis: Widespread plaques without patch stage
granuloma. lesions or pruritus; most do not distinguish this
●● The initial lesions are papules that soon evolve to form form from mycosis fungoides.
annular and polycyclic lesions with papular or nodular
edges. Elevated plaques may also occur. The lesions
tend to last for months or years, extend with central
healing, and often leave hypopigmented macules in
most cases.
●● Sun-exposed sites of upper trunk and arms are

predominantly affected.
●● DD: Granuloma annulare (annular plaque, discrete

papules in periphery), tinea corporis (central clearing,

peripheral papules, scales, KOH mount), leprosy
(hypopigmented hypoesthetic plaque, thickened nerve,
AFB), sarcoidosis, necrobiosis lipoidica diabeticorum.

Necrobiotic xanthogranuloma
●● This is a rare, progressive multisystem histiocytic

disease characterized by destructive cutaneous and

subcutaneous lesions affecting men and women equally.
●● The classic skin lesion is an asymptomatic indurated

papule, nodule, or plaque with a yellow “xanthomatous”

hue. Plaques are firm and indurated, with an active red
border and atrophic center.
●● The most common site affected is the periorbital area,

trunk, and proximal extremities; the condition begins at

around 50 years of age.
●● Lesions are yellow plaques and nodules that are deep,

firm, and indurated with active an red border and

atrophic center.
●● DD: Granuloma annulare (annular plaque, discrete

papules in periphery), sarcoidosis (red or tan-brown

plaques, naked granuloma, borderline leprosy (multiple
patches, nodules, and plaques with sensory impairment,
thickened nerves, AFB present), xanthoma (yellow
plaques and nodules affecting joints and fingers,
hyperlipidemia).

Mycosis fungoides (plaque stage)

●● It is an epidermotropic cutaneous T cell lymphoma

characterized by a proliferation of small- to medium-

sized CD4+ T cells with cerebriform nuclei. More
commonly seen in elderly males.
●● Lesions are macular to start with and gradually become

more infiltrated to form plaques and nodules.

●● Plaques are slightly erythematous and scaly, often with

a cigarette-paper surface (wrinkled appearance, also

called pseudo-atrophy); sites of predilection include
buttocks, trunk, upper thighs, and upper arms. Less
often, involvement of flexures, scalp, and palms is
seen. If confronted with erythematous scaly plaques Figure 8.7  (a) Erythematous scaly plaque of mycosis
with varying shades of color, always think of mycosis fungoides. (b) Hyperpigmented, atrophic, scaly plaques in
fungoides (Figure 8.7a–f). mycosis fungoides. (c) Large erythematous scaly plaque
●● Pagetoid reticulosis has two clinical patterns: with erosions at places. (Continued)
188  Plaques: Generalized

         

●● Any patient with persistent polymorphic plaques

involving the pelvic girdle area should have a skin
biopsy. Early stages of MF may mimic allergic
contact dermatitis, atopic dermatitis, and fungal
infection.
●● DD: Chronic plaque psoriasis (micaceous scales,
Auspitz sign, extensors), granuloma annulare
(annular plaque, discrete papules in periphery, no
lymphadenopathy), sarcoidosis (red or tan-brown
plaques, naked granuloma).

Figure 8.7 (Continued)  (d) Mycosis fungoides pre-

senting as multiple, erythematous scaly plaques of
varying sizes. Some of these plaques are ulcerated
and crusted. (e) Violaceous, scaly plaques healing
with dyspigmentation and atrophy in mycosis fun-
goides. (f) Classic reniform ulcerated plaques of
mycosis fungoides on the chest in the same patient.
(a–c – Courtesy: Dr Tanumay Raychaudhury The Skin
Hospital, Westmead Skin and Cancer Foundation,
Australia; d – Courtesy: Dr. Soumyajit Roychoudhury,
Berhampore, India; e,f – Courtesy: Dr. Bhuvaneshwari
Dewangan, Dr. Naveen Keshwani, Dr. Monica Jain, and
Dr. Siddhartha Dash. Shri Shankaracharya Institute of
Medical Sciences, Chattisgarh, India.)
 Plaques: Generalized  189

Figure 8.8.  Well-circumscribed, thick, erythematous areas

of scaling in a child in progressive symmetric erythrokera-
toderma. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

Progressive symmetric erythrokeratoderma (PSEK)

●● This is a rare, autosomal dominantly inherited

genodermatosis condition presenting early in childhood.

●● Cutaneous lesions in PSEK start early in infancy and

present as well-circumscribed, non-migratory areas of

erythema with scaling distributed symmetrically on the
knees, elbows, buttocks, head, and dorsal surfaces of
the hand and feet. Gradually the scaling progresses to
become hyperkeratotic plaques and becomes stable after
puberty (Figure 8.8). The trunk is spared.
●● DD: Erythrokeratodermia variabilis (EKV; lesions in

EKV fluctuate in their extent, and they can be induced

by external mechanical pressure and show seasonal
variation), chronic plaque psoriasis (seasonal variation,
micaceous scales, Auspitz sign, trunk), pityriasis rubra
pilaris (nutmeg-grater feel, island of normal skin).

Urticaria
●● Urticaria (or “hives” or “nettle rash”) consists of itchy,

blanchable wheals (Figure 8.9a,b).

●● These wheals vary in size from 1 mm to many

centimeters.
●● They are caused by vasoactive mediators, predominantly

histamine, released from mast cells.

●● In the vast majority of cases the wheals are transient,

lasting for only a few hours in any one place, but with
new wheals appearing in other places. Figure 8.9  (a) Annular, erythematous, edematous plaques
●● Etiology: lesions may be precipitated by physical factors in generalized urticaria in a child. (b) Generalized urti-
(cold, heat, sweating, exercise, pressure, sunlight, water carial plaques in a child. (a – Courtesy: Dr. Piyush Kumar,
Katihar, India; b – Courtesy: Dr. Niharika Ranjan Lal,
and vibration), food (salicylates in food/additives,
ESIPGIMSR, Kolkata, India.)
preservatives, colors), drugs (aspirin, NSAIDS,
infections (Helicobacter pylori, dental infections),
autoimmune conditions (systemic lupus erythematosus, ●● Clinically, erythematous indurated wheals with
Sjogren syndrome) to name a few. purpuric foci mostly occur on the trunk and proximal
●● DD: urticarial vasculitis, urticaria pigmentosa. limbs, with symptoms of pain rather than itch.
●● The condition is thought to be mediated via a type III/
Urticarial vasculitis immune complex hypersensitivity reaction, in which
●● This is a chronic disease that presents as urticarial antigen/antibody complexes deposit in vessel walls.
lesions that persist for more than 24 hours, often ●● DD: Urticarial phase of bullous pemphigoid (persistent,
demonstrate purpura, and leave behind post- vesicle formation), erythema multiforme (associated
inflammatory hyperpigmentation. with herpes infection, target lesion).
190  Plaques: Generalized

Figure 8.10.  Urticarial phase bullous pemphigoid.

(Courtesy: Dr. Piyush Kumar, Katihar, India.)

Bullous pemphigoid (urticarial phase)

●● Bullous pemphigoid is a common immunobullous

disorder that mostly occurs in elderly people.

●● Bullous pemphigoid commonly starts with itching

and a non-specific rash on the limbs that may be

either urticaria-like or occasionally eczematous
(Figure 8.10).
●● Commonly affected sites are lower abdomen, inner

thighs, and flexor of forearm.

●● The urticarial prodrome usually lasts one to three weeks

before blisters occur.

●● Mucosal lesions are less common and less severe

compared to pemphigus vulgaris.

●● DD: Urticaria (transient, evanescent, edematous

plaque), erythema multiforme (associated with herpes

infection, target lesion), dermatitis herpetiformis
(intensely pruritic, extensors, gluten hypersensitive
enteropathy, dramatic response to dapsone.)
Figure 8.11  (a) Multiple, shiny, atrophic, indurated
Generalized morphea plaques on abdomen and flank in generalized morphea.
●● This is a rare condition in which idiopathic sclerosis of (b) Large indurated plaques of morphea on the abdomen
in a middle-aged lady. (a,b – Courtesy: Dr. Piyush Kumar,
the skin occurs in a widespread manner.
Katihar, India.)
●● Classic morphea presents as well-circumscribed

sclerotic plaques with ivory center and red-violet

periphery (lilac ring), starting as an erythematous patch Generalized lichen sclerosus et atrophicus (LSA)
that slowly spreads, attaining size of 5–20 cm, in which ●● This is a dermatosis that presents with either porcelain-

spontaneous or therapy-induced regression occurs white papules and plaques or atrophy and classic
(Figure 8.11a,b). histological picture.
●● The main areas involved are the upper trunk, breasts, ●● The etiology is not clear, but immunoglobulin G (IgG)

abdomen, and upper thighs. antibodies against extracellular matrix protein 1 (EMP
●● It is more common in females and appears at the 1) have been identified.
age of 30 to 40 years. Systemic findings are not ●● Typical cutaneous lesions are small, ivory or porcelain-

common; rarely it includes malaise or Raynaud’s white, shiny round macules or papules that are semi-
phenomenon. It is most common on the trunk in translucent, resembling mother of pearl. These lesions
young girls. get aggregated into plaques that are slightly raised or
●● It resolves with atrophy and hyperpigmentation. level with the surface of the skin; typically their surface
●● DD: Pseudo-scleroderma (porphyria cutanea shows prominent dilated sweat ducts or pilosebaceous
tarda and graft versus host disease), drug-induced orifices containing yellow or brown horny plugs
sclerosis (Bleomycin), systemic sclerosis (Raynaud’s (Figure 8.12a–c).
phenomenon, face and extremities are more common, ●● The lesions on the skin are symptomless, and

worsen with age, systemic disease), eosinophilic extragenital lesions occur on the trunk, particularly on
fasciitis (acute onset, pain and edema, more in the upper part and around the umbilicus, the neck, the
male), generalized lichen sclerosus (papule, follicular axillae, and the flexor surfaces of the wrists. Genital
plugging). lesions may itch.
 Plaques: Generalized  191

Figure 8.12  (a) Extensive lesions of extragenital lichen

sclerosus on the abdomen. (b) Extensive plaques of extra-
genital lichen sclerosus on the upper back. The surface is
remarkable for follicular plugging. (c) Extensive plaques
of extragenital lichen sclerosus on the lower back. (a,c –
Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy:
Dr. Shekhar Neema, Armed Forces Medical College,
Pune, India.)

●● DD: Generalized morphea (lacks characteristic

papules of LSA), atrophic lichen planus (extremely
pruritic, raised, violaceous), annular atrophic plaque
type of DLE.

Darier disease
●● Darier disease is an autosomal dominant disorder with

primarily follicular keratoses and distinct histology.

●● It occurs due to mutation in a calcium channel

regulating gene ATP2A2, encoding SERCA2; that leads

to disturbance in calcium homeostasis and is thought to
interfere with desmosome stability.
●● Early lesions are discrete or confluent, rough, greasy,

skin-colored or yellowish brown papules that coalesce

particularly in the axillae, perineum, groin, and
natal cleft to form irregular, warty, fissured plaques,
sometimes becoming vegetating and malodorous
(Figure 8.13a–c). Figure 8.13  (a) Hyperpigmented, dirty, warty
●● White painless palatal papules appear as cobblestone in papules coalescing to form generalized plaques in Darier
the oral cavity. disease. (Continued)
192  Plaques: Generalized

Figure 8.13 (Continued)  (b) Macerated plaques and kera-

totic papules of Darier disease. (c) Severe facial involve-
ment in Darier disease in summer. (a–c – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

●● Nails show longitudinal red and white bands,

subungual hyperkeratosis, and triangular nicking of the
free edges.
●● DD: Seborrheic dermatitis (absence of family history,
spares mucosa and nails, appears less on acral area),
acanthosis nigricans, pemphigus vegetans, transient
acantholytic dermatosis (discrete, intertriginous area).

Tinea corporis
●● Tinea corporis is a dermatophyte infection of the skin of

trunk and extremities, excluding the palms, soles, and

Figure 8.14  (a) Erythematous, annular and polycyclic
inguinal region. plaques with scales on active border in extensive tinea
●● The infection spreads centrifugally, resulting in annular
corporis. (b) Annular and polycyclic erythematous scaly
scaly plaques of various shapes and sizes with pustules plaques in extensive tinea corporis. (a,b – Courtesy:
at the active border (Figure 8.14a,b). Dr. Piyush Kumar, Katihar, India.)
 Plaques: Generalized  193

●● Lesions start as erythematous flat, scaly spots and then a rapid rate (up to 1 cm/day) to develop a wood-grain or
develop a raised border that expands in all directions, Zebra-like pattern due to development of rings within
with healing in the center, giving the classical “ring- rings on trunk.
like” appearance. ●● It is a paraneoplastic sign of internal malignancy (EGR
●● There are variable degrees of inflammation, in some is commonly seen in association with the malignancies
instances with pustule formation, depending on nature of lung, breast, esophagus, and stomach).
of dermatophyte and host response. ●● In erythema marginatum (rheumaticum), lesions
●● Unjustified medications can obscure central clearing begin as erythematous macules that spread
and cause tinea incognito. peripherally and become patches or plaque with
●● Early lesions usually present as red papules or plaques no scale. They may be arranged polycyclically.
and develop into extremely itchy scaly plaques Sites affected are the trunk, axillae, and proximal
anywhere on the body. extremities, with sparing of the face. It is associated
●● DD: Granuloma annulare (annular plaque, discrete with the active phase of rheumatic fever and is
papules in periphery, central depression), borderline generally seen in conjunction with carditis.
tuberculoid leprosy (hypoesthesia, hypopigmentation, ●● DD: Granuloma annulare (annular plaque, discrete
thickened nerves), psoriasis (micaceous scales, Auspitz papules in periphery, central depression), urticaria
sign, extensor aspect of limbs), nummular eczema (oozy (transient, evanescent edematous hives/wheals), tinea
plaque, no central clearing, no peripheral papules). corporis (rarely wide spread, central clearing, KOH),
secondary syphilis (scaly plaques, rebound tenderness,
Figurate erythemas VDRL positivity).
●● These are patterned erythemas, often annular in shape

and occasionally migratory. Various patterns and forms Parapsoriasis

have been described. ●● Parapsoriasis is broadly divided into small and large
●● Erythema annulare centrifugum begins as pink papules plaque variants.
that expand centrifugally to develop central clearing; ●● Small-plaque parapsoriasis

they can reach sizes up to 6–10 cm in diameter over ●● A chronic asymptomatic condition mostly benign
a period of one to two weeks. In the superficial form in nature.
lesions are minimally elevated, with desquamation ●● Characterized by asymptomatic scaly patches or
at the inner margin (trailing scale) (Figure 8.15); in slightly raised papules and plaques over trunk
deep gyrate erythema, advancing edges are obviously and proximal extremities. Individual lesions are
elevated without associated scale or pruritus. monomorphic round or oval erythematous patches,
●● The usual affected sites are the trunk and proximal 2.5–5 cm in diameter, with slight scaling and waxy,
parts of limbs, rarely if ever involving palms, soles, yellow tinge.
scalp, or mucus membranes. ●● Usually occurs at middle age and evolves gradually
●● It is associated with Borrelia infection. over months to years; there is sparing of the pelvic
●● In erythema gyratum repens (EGR), patients usually girdle area.
have multiple annular or polycyclic erythematous ●● Large-plaque parapsoriasis

lesions that develop scale at their edges; they advance at ●● Associated with development of definite mycosis
fungoides, so long-term follow up of these cases is
required.
●● Characterized by presence of persistent, large,
yellow-orange atrophic patches and thin plaques on
the trunk and limbs.
●● If plaques show striking polymorphism and

poikiloderma, this suggests mycosis fungoides.

●● DD: Pityriasis rosea (pruritus, herald patch, collarette

of scale, hanging curtain sign), pityriasis lichenoides

(young adults, necrosis and varioliform scar, lateral
trunk, spontaneous resolution), guttate psoriasis
(sudden onset, pharyngitis, thicker plaque).

Papulosquamous syphilids
●● Syphilis is a chronic infection caused by Treponema

pallidum and characterized by florid clinical

Figure 8.15  Annular erythematous plaques with fine trail- manifestations interspersed with periods of
ing scales in erythema annulare centrifugum. (Courtesy: asymptomatic latency; it has primary, secondary, and
Dr. Piyush Kumar, Katihar, India.) tertiary stages as well as a latent period of variable length.
194  Plaques: Generalized

●● Papulosquamous syphilids are the most common finger web, wrist, axilla, umbilical, and genital regions.
clinical presentation of secondary syphilis. Typically Severe eczematous changes are common and may
they do not itch, are coppery-red in color, and are be widespread, with severe and extensive secondary
symmetric in distribution. infection.
●● Generalized, nonpruritic, papulosquamous eruptions ●● Crusted scabies (Norwegian or hyperkeratotic scabies)
range in size from 1–2 mm to 15–20 mm, and they is found in immunocompromised or debilitated
vary in color from pink to violaceous to red-brown. patients.
Psoriasiform plaques of the palms and soles are ●● In these patients, the infestation assumes a heavily
common in black people, as are annular and circinate scaling and crusted appearance (Figure 8.17a–c).
papular rashes (Figure 8.16). ●● DD: Atopic dermatitis (chronic, history of atopy,
●● Mucosal lesions range from small, superficial ulcers that white dermatographism, seasonal variation, raised
resemble painless aphthae to large gray plaques. immunoglobulin E level), allergic contact dermatitis
●● DD: Pityriasis rosea (pruritus, lesions along the cleavage (confined to area of contact, erythematous plaque
line, herald patch, collarette of scale, hanging curtain with oozing and crusting, patch test), dermatitis
sign), scaly varieties of tinea corporis, parapsoriasis herpetiformis (papulovesicles, extensor site, gluten
(elderly, necrosis absent, no lymphadenopathy, absent sensitivity), psoriasis (micaceous scales, Auspitz sign,
prodromal symptoms), drug eruptions (scarlatiniform extensors).
or morbilliform, pruritic), psoriasis (thick scale, absence
of lymphadenopathy and mucous patches). Sulzberger-Garbe disease
●● This is also known as exudative discoid and lichenoid
Norwegian scabies chronic dermatitis.
●● Human scabies is a pruritic condition caused by ●● This is an extremely pruritic condition characterized by

infestation by the host specific mite Sarcoptes scabiei discoid lesions with “lichenoid” and exudative phases,
var. hominis. which either coexist or alternate rapidly with each other.
●● Typically, small erythematous papules with a variable ●● There may be showers of round, erythematous, oozy

degree of excoriation present symmetrically on the plaques.

Figure 8.16  Scaly papules and plaques of secondary

syphilis. (Courtesy: Dr. Santoshdev Rathod, SVPIMSR,
Smt. NHL Municipal Medical College, V.S. Hospital, Figure 8.17  (a) Crusted, scaly plaque in Norwegian
Ahmedabad, India.) scabies. (Continued)
 Plaques: Generalized  195

●● Penile and scrotal lesions are common and are almost

pathognomonic.
●● DD: Mycosis fungoides (mildly itchy, generalized,
epidermotropism, atypical cells), contact dermatitis
(localized, asymmetrical, patch test), dermatitis
herpetiformis (papulovesicles, extensor site, gluten
sensitivity).

Generalized seborrheic keratosis

●● Generalized seborrheic keratosis presents as oval,

slightly raised, tan/light-brown to black, sharply

demarcated papules or plaques, rarely more than 3 cm
in diameter (Figure 8.18).
●● Mostly present on the trunk, face, and extremities.

●● Palms and soles are usually spared.

●● The sudden appearance of numerous seborrheic

keratosis may be a cutaneous sign of internal

malignancy (Leser-Trelat sign).
●● DD: Verruca vulgaris (pseudo-Koebnerization, warty

plaque, and papules), post-pemphigus acanthoma.

Pruritic urticarial papules and plaques of pregnancy

(PUPPP)
●● This eruption is characterized by erythematous papules

and plaques that begin as 1- or 2-mm lesions within the

abdominal striae.
●● It spreads within a day to involve the abdomen, thighs,

buttocks, and extremities.

Figure 8.18  Multiple hyperpigmented papules and

Figure 8.17 (Continued)  (b) Palm involvement in Norwegian plaques on chest in seborrheic keratosis. Central larger
scabies. (c) Resolution of lesions with anti-scabies treatment. plaques show hyperkeratotic surface. (Courtesy:
(a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.) Dr. Piyush Kumar, Katihar, India.)
196  Plaques: Generalized

●● It spares the periumbilical area, face, palm, soles, and ●● Face, head, palms, and soles are spared.
mucosa. ●● The edges of the lesions are not completely sharp.
●● It is most common in primigravida in the later part of ●● They become edematous within a few minutes of gentle
pregnancy. rubbing (Darier sign).
●● DD: Pemphigoid gestationis (early part of pregnancy, ●● DD: Urticaria (transient, evanescent, edematous
involves umbilical area, no relation with striae), contact plaque), juvenile xanthogranuloma (asymptomatic,
dermatitis (scaly, weeping plaque, hyperkeratotic), rubbery in consistency, head and neck are is the most
urticaria (transient, evanescent, edematous plaque). common site).

Urticaria pigmentosa Kaposi sarcoma (KS)

●● This is the most common pattern of cutaneous mastocytosis. ●● Kaposi sarcoma is formed by the proliferation of

●● Urticaria pigmentosa develops in the first year of life. abnormal vascular endothelial cells.
●● Adult onset urticaria pigmentosa is present most ●● It is A multifocal systemic disease with four principal

commonly at 20 to 40 years of age. clinical variants:

●● Numerous reddish-brown or pale, pruritic, ●● Classic Kaposi sarcoma
monomorphic maculopapules, plaques or nodules ●● African endemic KS
appear in a symmetrical distribution, mainly on the ●● KS due to iatrogenic immunosuppression
trunk and thighs (Figure 8.19a,b). ●● AIDS-related epidemic KS
●● Cutaneous lesions can vary from pink patches to dark

violet plaques, nodules, or polyps.

●● The process usually begins on the extremities, most

commonly on (Figure 8.20) the feet and occasionally on

the hands, ears, or nose.
●● The lesions may involute to leave pigmented scars or

may become eroded, ulcerated, or fungating.

●● Different stages of lesions are found in the same patient.

●● There is predilection for the head, neck, trunk, and

mucous membrane.
●● DD: Pseudo-Kaposi’s sarcoma (non-progressive,

spindle-cell proliferation in histopathology),

angiosarcoma (localized, lymphedema).

Erythema nodosum leprosum (ENL)

●● ENL is encountered during therapy of patients with

lepromatous leprosy or borderline leprosy.

●● Classic ENL is characterized by the appearance of

crops of tender, painful subcutaneous nodules with

associated constitutional symptoms (Figure 8.21a–c).
Acute lesions appear in crops and heal over several
days. Commonly affected sites are the face and extensor
surfaces of the limbs.

Figure 8.20  Numerous violaceous plaques and nod-

Figure 8.19  (a) Pigmented plaques of urticaria pigmen- ules in Kaposi sarcoma. (Courtesy: Prof. Reza Yaghoobi,
tosa on the back. (b) Another case of urticaria pigmen- Department of Dermatology, Ahvaz Jundishupor
tosa. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.) University of Medical Sciences, Iran.)
 Plaques: Generalized  197

Figure 8.21  (a) Multiple erythematous edematous plaques and nodules of erythema nodosum leprosum. (b)
Erythematous, edematous plaques of erythema nodosum leprosum (ENL) on the face. Prior lesions have healed with
atrophic scarring. (c) Erythematous, edematous plaque and nodules in ENL. (d) Pustular ENL. (e) Erythema multiforme like
lesions in ENL. (f) Eschar covering an ulcerated plaque in ENL. (a–f – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Ulceration may occur that heals with scarring. are slow to heal. It is due to infarction consequent upon
●● The Lucio reaction occurs in patients with lucio leprosy. deep cutaneous vasculitis.
Clinically, it appears as irregularly shaped erythematous ●● Other clinical variants of ENL are vesicobullous,
patches that become dark, sometimes forming bullae pustular, hemorrhagic, and erythema multiforme-like
and becoming necrotic, leaving deep painful ulcers that (Figure 8.21d–f).
198  Plaques: Generalized

●● DD: Cutaneous necrotizing vasculitis (mucous Diffuse acanthosis nigricans

membrane involvement, no sensory complaints, ●● Acanthosis nigricans (AN) is characterized by
no nerve thickening, negative slit skin smear), hyperpigmentation and velvet-textured plaques,
Lucio leprosy (extremities, erythematous patch, symmetrically distributed.
superficial scar).

Disseminated cutaneous leishmaniasis

●● Disseminated cutaneous leishmaniasis may be seen in

both New and Old World diseases.

●● Multiple non-ulcerated papules and plaques, chiefly on

exposed surfaces, characterize this type.

●● The disease begins with a single ulcer, nodule,

or plaque from which satellite lesions may

develop and disseminate to cover the entire body
(Figure 8.22).
●● DD: Lepromatous leprosy (gloves-and-stockings

anaesthesia, thick peripheral nerve, AFB positive),

malignant lymphoma.

Post-kala azar dermal leishmaniasis (PKDL)

●● Patients with PKDL may present with widespread

papules, plaques, and nodules in addition to

hypopigmented patches.
●● These plaques and nodules usually develop over existing

hypopigmented patches, and the lesions are usually

distributed over the face, trunk, and upper extremities
(Figure 8.23a,b).
●● Sometimes, plaques and nodules develop de novo,

and such lesions show a predilection for acral areas,

especially over the joints (Figure 8.23c).

Figure 8.22  Multiple crusted plaques and ulcers in Figure 8.23  (a) Erythematous, smooth-surfaced papules
cutaneous leishmaniasis. (Courtesy: Prof. Reza Yaghoobi, and plaques on the face in post-kala azar dermal leish-
Department of Dermatology, Ahvaz Jundishupor maniasis (PKDL). (b) Typical erythematous, succulent
University of Medical Sciences, Iran.) papules and plaques of PKDL. (Continued)
 Plaques: Generalized  199

●● Palms and soles may show thickening of the palmar

skin, with exaggeration of the dermatoglyphics (tripe
palms).
●● DD: Addison’s disease (pigmentary change, mucosal
involvement), epidermolytic hyperkeratosis (blisters,
spine like scales).

Epidermodysplasia verruciformis
●● Epidermodysplasia verruciformis (EV) is a rare,

inherited disorder characterized by widespread HPV

infection and cutaneous SCCs.
●● EV usually manifests in childhood with persistent and

often widespread warts.

●● Individual lesions typically have either the

appearance of f lat warts or f lat scaly red-brown

macules that resemble pityriasis versicolor
(Figure 8.25a,b).
●● On the trunk are lesions that are red, tan, or

brown patches/plaques or hypopigmented, very

slightly scaly plaques resembling tinea versicolor.
Plaques on the elbows may resemble psoriasis
(Figure 8.25c).
●● Later Bowen disease and squamous cell carcinoma

develop, primarily in sun-exposed skin.

Figure 8.23 (Continued)  (c) Erythematous and skin-colored
infiltrative plaques and nodules in post-kala azar dermal leish-
maniasis. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Clinically, it presents as brown to gray-black

papillomatous cutaneous thickening in the flexural
areas, including the postero-lateral neck, axillae, groin,
and abdominal folds (Figure 8.24a,b).

Figure 8.24  (a) Hyperpigmented, grayish-black velvety

patch in generalized acanthosis nigricans. (b) Generalized Figure 8.25  (a) Numerous pigmented and verrucous
acanthosis nigricans. (a,b – Courtesy: Dr. Piyush Kumar, plaques in a middle-aged lady with epidermodysplasia
Katihar, India.) verruciformis. (Continued)
200  Plaques: Generalized

●● DD: Widespread warts (pseudo-Koebner,

immunocompromised patient), pityriasis versicolor
(scaly, perifollicular, spaghetti-and-meatball
appearance).

Systematized verrucous epidermal nevus

●● Verrucous epidermal nevi are congenital, non-

inflammatory cutaneous hamartomas composed of

keratinocytes following Blaschko’s lines.
●● When two or more linear lesions are present, the term

“systematized” is used.
●● At birth they have a whitish or macerated appearance

that becomes velvety pigmented streaks or plaques

within few days (Figure 8.26a,b).
●● They may involve adjacent mucosal surfaces and nail

folds.

Figure 8.25 (Continued)  (b) Plaques may coalesce

to form larger lesions. (c) Hyperpigmented, verru- Figure 8.26  (a) Hyperpigmented, mildly scaly papules
cous, keratotic plaques in epidermodysplasia verru- coalescing to form plaques distributed linearly along
ciformis. (a,b – Courtesy: Dr. PC Das and Dr. Piyush Blaschko’s lines in systemized verrucous epidermal
Kumar, Katihar, India; c – Courtesy: Dr. Piyush Kumar, nevus. (b) Systemized verrucous epidermal nevus. (a,b –
Katihar, India.) Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Plaques: Generalized  201

●● DD: Incontinentia pigmenti (mostly in female, four

distinct clinical stages), ichthyosis bullosa of Siemens
(non-Blaschkoid pattern, Mauserung phenomenon).

Incontinentia pigmenti (verrucous stage)

●● This is an X-linked dominant condition caused by

NEMO gene mutation and clinically characterized

by spattered pigmentation on the trunk, preceded by
vesicular and verrucous changes.
●● There are four phases of lesions, all following a peculiar

linear and streaked pattern, starting with the vesicular

phase and gradually changing into the verrucous and
pigmentary phase.
●● The verrucous phase has smooth red nodules or plaques,

often irregularly linear, along Blaschko’s lines on the

limbs and trunk. The plaques, bluish-purple in color,
may be extensive and precede the bullae and may Figure 8.27  Generalized plaques in erythema multiforme
ulcerate to heal with characteristic, bizarre “Chinese characterized by central vesiculation, zone of edematous
letter pattern” pigmentation. plaque and erythema at periphery. (Courtesy: Dr. Piyush
●● The most commonly involved extracutaneous organ is Kumar, Katihar, India.)
the teeth followed by bone and CNS.
●● DD: Verrucous epidermal nevi (no vesicular phase,
erythematosus (Rowell syndrome), secondary syphilis
males can be affected).
(scaly plaques, rebound tenderness, VDRL positivity),
Erythema multiforme (EM) Sweet syndrome (true target lesion absent, papillary
●● EM is an acute, self-limited skin disease characterized
dermal edema, response to steroid).
by the abrupt onset of symmetrical fixed red papules
and plaques. REFERENCES
●● Erythematous plaques, typically 1–3 cm in diameter,
1. Lipsker D. Clinical Examination and Differential Diagnosis of Skin
evolve into pathognomonic “target lesions” or “iris Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 35.
lesions” consisting of a central zone of dusky erythema Palpable Erythematous Lesions. P. 195–200.
or vesicle, surrounded by a rim of pallor and a 2. Lipsker D. Clinical Examination and Differential Diagnosis of Skin
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 31.
third zone of erythema commonly seen over distal
Skin-Colored Palpable Lesions. P. 185–188.
extremities (Figure 8.27). 3. Lipsker D. Clinical Examination and Differential Diagnosis of Skin
●● It rarely becomes bullous or urticarial. It heals
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 32.
spontaneously over weeks, sometimes with residual Brown, Black, Blue, or Gray Palpable Lesions. P. 189–190.
hyperpigmentation. 4. Lipsker D. Clinical Examination and Differential Diagnosis of Skin
●● Sites of predilection are the palms, dorsa of hands, soles, Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 33.
White Palpable Lesions. P. 191–192.
peri-oral area and face, and genital areas. 5. Lipsker D. Clinical Examination and Differential Diagnosis of Skin
●● It is accompanied by febrile prodrome and arthralgias.
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 34.
●● Variants are EM minor and EM major, mostly EM Yellow Palpable Lesions. P. 193–194.
minor because of recurrent HSV infection, while EM 6. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
major is associated with mycoplasma.
Chapter 8. Plaques with Scale. P. 113–122.
●● DD: Stevens-Johnson syndrome (10–30% skin
7. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
involvement with epidermal loss and mucosal erosions, Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
drug causation), erythema multiforme-like lupus Chapter 28. Papillomatous and Verrucous Lesions. P. 461–476.
 9
Nodules: Localized

ASEEM SHARMA, TRUPTI AGARWAL, MANISH KHANDARE, MADHULIKA MHATRE

INTRODUCTION ●● They are soft, compressible blue masses that enlarge

when the affected area is in a dependent position or
Localized nodules pose a diagnostic dilemma owing to the with physical activity.
sheer number of differentials encountered. The clinical fea- ●● The blue color is pathognomonic and caused by the
tures that help in evaluating a nodular growth are listed presence of ectatic anomalous venous channels within
in Table 9.1. The authors have, with the view to reduce the the dermis.
diagnostic challenge, adopted a color-based classification ●● There is no increase in local skin temperatures or thrill
(Table 9.2). Needless to say, the entities under each color on palpation of the lesion.
code should be considered as differential diagnoses for each
other. Other useful clinical clues are listed in Table 9.3, and Blue rubber bleb nevus syndrome
their salient features are discussed below.1–5 While evaluating ●● It is a rare vascular anomaly syndrome consisting

a nodule, one must look for ominous signs. The presence of of multifocal venous malformations (VM). The
nodules in the pediatric age group and indurated nodules in malformations are most prominent in the skin, soft tissues,
an adult, especially progressive or exhibiting surface changes, and gastrointestinal (GI) tract but may occur in any tissue.
is ominous, and warrants a biopsy to rule out malignancy or ●● They are characterized by soft, elevated lesions on

metastasis. Other ominous presentations include a nodule the skin or just under the skin that are dark blue, red,
developing in an existing mole, an elderly patient with a lesion purple-red, or black (Figure 9.1).
in a sun-exposed area, and a middle-aged or elderly patient
who develops widespread skin nodules over a period of a few
weeks especially if they are unwell and losing weight, etc.

Cavernous hemangioma
●● It is a slow-flowing venous malformation present at
birth, though it may not always be evident.
●● It presents as non-proliferating vascular birthmarks

composed of anomalous ectatic venous channels.

Table 9.1  Clinical characteristics of a nodule

• Age of onset
• Duration
• Site
• Distribution
• Color
• Surface anatomy
• Fixity to underlying skin
• Texture
• Symptomatology – pruritus, tenderness Figure 9.1  Multiple, clustered blue-black nodules of blue
rubber bleb nevus syndrome. The background skin is ery-
• Evolution – tumor (size, rate of growth), surface
thematous. (Courtesy: Dr. Anup Kumar Tiwary, Consultant
changes (pigmentation, excoriation, necrosis, Dermatologist, Yashoda Hospital and Research Center,
ulceration) Ghaziabad, India.)

202 DOI: 10.1201/9781351054225-17

 Nodules: Localized  203

Table 9.2  Clinical approach to localized nodules

Color Diseases
Blue • Compressible – cavernous hemangioma, blue rubber bleb nevus
• Firm – pigmented histiocytoma/dermatofibroma, blue nevus, pilomatricoma, glomus tumor
Red • Painful:
• Fingers: glomus tumor, Osler nodes
• Face and trunk: acne conglobata
• Face/extremity: sporotrichosis
• Ear: chondrodermatitis nodularis helicis
• Axilla: hidradenitis suppurativa
• Anywhere: furunculosis, leiomyoma
• With systemic features: erythema nodosum, erythema nodosum leprosum, localized Sweet’s
syndrome, cutaneous lymphoma, leukemia cutis
• Vascular lesions
• Infant: hemangioma
• Adult near ear: angiolymphoid hyperplasia with eosinophilia
• Anywhere: pyogenic granuloma
• Itchy: prurigo nodularis, nodular scabies
• Associated with edema
• eosinophilic cellulitis
• acroangiodermatitis of Mali
• Ulcerated nodules: scrofuloderma, atypical mycobacterial infections, nodular vasculitis,
cutaneous polyarteritis nodosa
• Occupational contact with cattle: orf, milker’s nodule
• Surface telangiectasia: keloid
• Others: Giant molluscum contagiosum, amelanotic melanoma, clear cell acanthoma, eccrine
poroma, Kaposi sarcoma, Merkel cell tumor, cylindroma, Spitz nevus, granuloma faciale (lever
type), Spitz nevus, cutaneous leishmaniasis, schwannoma
Yellowish • Children – juvenile xanthogranuloma
• Head and neck region – sebaceous hyperplasia (yellow-white), necrobiotic xanthogranuloma
(around eyes), chondroid syringoma, steatocystoma multiplex
• Around joints – xanthoma (tendinous, tuberous), tophi
• Central punctum – dilated pore of Winer
• Ulcerative: tertiary syphilis (gumma)
• Soft, cerebriform surface: nevus lipomatosus
Brown • Children – mastocytoma, melanocytic nevi (compound)
• Elderly – malignant melanoma, dermatofibrosarcoma protuberans
• Immunosuppressed/ ill patients – deep mycoses
Black • Smooth surface – melanocytic nevi (compound), pigmented dermatofibroma
• Ulcerated surface – malignant melanoma
• Keratotic surface – cutaneous horn
Skin-colored • Soft, anywhere – neurofibroma, macro acrochordon
• Scalp: cylindroma
• Over bony prominences: rheumatoid nodules
• Face: sebaceous hyperplasia
• Mobile: lipoma
• Miscellaneous: subcutaneous granuloma annulare, melanocytic nevi (intradermal), histoid leprosy
White and Translucent • Hard – cutaneous calcinosis, tophus, osteoma cutis
nodules • Multiple, elderly population – sebaceous hyperplasia (yellow-white)

●● They may be tender, contain blood and be easily ●● DD: Kaposi sarcoma, angiosarcoma, vascular anomalies
compressed, and are usually located on the upper associated with congenital or systemic diseases (Klippel-
limbs, trunk, and soles of the feet but can occur Trenaunay-Weber, Ehlers-Danlos, the CREST variant of
anywhere. scleroderma, and Osler-Weber-Rendu syndrome).
204  Nodules: Localized

Table 9.3  Clinical clues to diagnose localized nodules Blue nevus

●● This is also known as benign mesenchymal melanoma,
Clue Dermatoses
blue neuronevus, chromatophoroma, melanofibroma,
Pruritic Prurigo nodularis, nodular scabies, Tieche-Jadassohn nevus, and dermal melanocytoma.
nodules pseudolymphoma (arthropod-bite), ●● The etiology is unclear; it is believed to originate from
mastocytosis, lymphoma cutis, leukemia latent dermal melanocytes, gestational remnants of
cutis melanocyte migration.
Painful “Blend an egg”
nodules • Blue rubber bleb nevus
• Leiomyoma
• Eccrine spiradenoma
• Neurofibroma
• Dercum disease
• Chondrodermatitis nodularis helicis
• Angiolipoma
• Neuroma
• Neurilemmoma
• Endometriosis
• Erythema nodosum
• Glomus tumor
• Granular cell tumor
• Foreign body Granuloma
Consistency • Hard – osteoma cutis, calcinosis cutis,
pilomatricoma
• Firm – dermatofibroma, chondroid
syringoma, keloid, prurigo nodularis,
trichoepithelioma, rheumatoid nodule,
juvenile xanthogranuloma, leukemia
cutis, connective tissue nevi
• Soft – neurofibroma, angiolipoma,
lipoma
Rapidly Hemangioma, Kaposi sarcoma,
growing keratoacanthoma, malignant granular
nodules cell tumor, Merkel cell tumor, cutaneous
metastases, nodular melanoma,
pilomatricoma, pyogenic granuloma,
subcutaneous granuloma annulare

Dermatofibroma
●● Dermatofibroma is a benign, dermal, and superficial

subcutaneous myofibroblastic proliferation

microscopically mimicking fibromatosis.
●● It is more commonly seen in young adults, and females

are more commonly affected.

●● It is commonly seen on the lower extremities.

●● It presents as an asymptomatic, solitary, variably

pigmented, firm to hard nodule – the color may

vary from light brown to brown-black (Figure 9.2a).
Sometimes, the center may be pale.
●● When the skin is lightly squeezed from the sides of the

lesion, a “dimple” or Fitzpatrick sign appears, owing to

tethering to the skin (Figure 9.2b). The lesion moves
freely over subcutaneous tissue.
●● Hemosiderotic dermatofibroma presents as a blue or
Figure 9.2  (a) Solitary, brown nodule on arm in dermatofi-
blue-red colored nodule. broma. (b) Pressing the nodule from both sides demonstrated
●● DD: Malignant melanoma, blue nevus, Spitz nevus. central dimple known as Fitzpatrick sign. (Continued)
 Nodules: Localized  205

Figure 9.2 (Continued)  (c) Pigmented nodule of blue nevus.

(a – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy:
Dr. Deverashetti Srinivas, Nizamabad, India; c – Courtesy:
Dr. Balkrishna Nikam, Krishna Institute of Medical Sciences,
Deemed University, Karad, India.)

●● It is common in children and shows female predilection.

●● It presents as a solitary asymptomatic, blue-black,
dome-shaped nodule of diameter < 1–2 cm. The lesion
can appear anywhere on the body (Figure 9.2c).
●● The blue color is due to the “Tyndall effect”: a scattering
of blue light as dermal pigment absorbs the longer
visible light wavelengths.
●● DD: Malignant melanoma, pigmented dermatofibroma,
metastasis, thrombosed plantar wart, or a tattooing effect.

Pilomatricoma
●● Also known as calcifying epithelioma of Malherbe,

pilomatricoma is an asymptomatic slow-growing

benign cutaneous tumor, differentiating towards the
hair matrix of the hair follicle.
●● It occurs on the head and neck region in over 50% of cases.

●● It is clinically characterized by a deep-seated, firm, non-

tender subcutaneous mass adherent to the skin but not

fixed to the underlying tissue; blue-red discoloration of
overlying skin is characteristic (Figure 9.3a,b).
●● A pathognomonic sign is that the stretching of the skin

over the tumor shows the “tent sign” with multiple

facets and angles.
●● DD: Inclusion cyst, dermoid cyst.
Figure 9.3  (a) Subcutaneous nodule of pilomatricoma.
Glomus tumor The surface changes are secondary to electrocautery
●● Glomus tumors are rare but benign tumors originating
done earlier. (b) Pilomatricoma presenting as erythema-
tous nodule with crusted surface. (a – Courtesy: Dr. Piyush
from glomus bodies and are mostly seen over the fingertips. Kumar, Katihar, India; b – Courtesy: Dr. Esther Nimisha,
●● Subungual glomus tumors are frequently observed in
Jamshedpur, India.)
middle-aged women as oval, bluish, or reddish tumors
visible through the nail plate (Figure 9.4).
●● They are also reported to be associated with a history of ●● They are associated with various conditions, including
trauma in the affected area and neurofibromatosis. infective endocarditis, and are brought about by
●● The classic triad symptoms of glomus tumors are immune complex deposition.
pinpoint pain, severe pain, and cold hypersensitivity. ●● They occur mainly after the fourth decade of life and
●● DD: Neuroma. more common in males.
●● Common sites are fingers, toes, palms, and soles.
Osler nodes ●● Osler nodes are red-purple, marginally raised, tender
●● Osler nodes are painful, red, raised lesions found on the lumps, regularly with a pale focus. Pain usually appears
hands and feet. 24 hours before lesions.
206  Nodules: Localized

Figure 9.4  Skin-colored, painful nodule (arrow) of glomus

tumor. (Courtesy: Prof RC Gharami, Medical College and
Hospital, Kolkata, India.)

●● They can occur any time on the fingers or toes in

endocarditis (generally subacute) and last from hours to
a few days.
●● Osler nodes are also found in systemic lupus erythematosus,
marantic endocarditis, disseminated gonococcal infection,
and distal to infected arterial catheter.
●● DD: Janeway lesions, cutaneous vasculitis,
meningococcemia, gonococcemia, disseminated
intravascular coagulation, thrombocytopenia,
cholesterol embolization.
Figure 9.5  Pustules mixed with inflammatory nodules on
Nodulocystic acne cheek in nodulocystic acne. (Courtesy: Dr. Piyush Kumar,
●● It is characterized by development of inflammatory Katihar, India.)
nodules that sometimes soften and become fluctuant
(pseudo cysts). They may further evolve into abscesses, or papulopustules, nodules, or verrucous plaques and
sinus tracts, covered by hemorrhagic crusts (Figure 9.5). occasionally non-healing ulcers or small abscesses.
●● These lesions carry high potential for scarring. Facial involvement is common, but it can also occur
on fingers, arms, or hands.
Sporotrichosis (rose gardener’s disease) ●● Lymphocutaneous sporotrichosis: A
●● Sporotrichosis is a subcutaneous fungal infection of noduloulcerative lesion (sporotrichotic chancre)
the skin presenting after a thorn injury caused by the at the inoculation site and a string of similar
fungus Sporothrix schenckii. The fungus is commonly nodules along the proximal lymphatic, with or
found on decaying vegetation, rose bushes, twigs, hay, without transient satellite adenopathy, characterizes
sphagnum moss, and mulch-rich soil. this form (Figure 9.6). These secondary lesions
●● It can occur in any age group but is more common in appearing along lymphatic have varied morphology
male adults. of erythematous papules, nodules, or plaques,
●● Acral, trauma-prone sites such as fingers, hand, having smooth or warty surface, and may soften
forearm, etc., are commonly affected. and ulcerate discharging seropurulent material.
●● Cutaneous sporotrichosis can present in three forms: 1) ●● Disseminated sporotrichosis: This affects people
fixed cutaneous 2) lymphocutaneous 3) disseminated.6 with immunodeficiency, and symptoms depend on
●● Fixed cutaneous sporotrichosis: The lesions the body part affected. Joint pain, headache, and
are asymptomatic, erythematous, papules, seizures are common symptoms.
 Nodules: Localized  207

Figure 9.6  Multiple, erythematous, firm nodules with

crusted surface in lymphocutaneous sporotrichosis.
(Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● DD: Cutaneous leishmaniasis, lupus vulgaris,

furunculosis.
Chondrodermatitis nodularis helicis
●● Also known as Winkler’s disease, nodular

chondrodermatitis, ear pressure sores,

chondrodermatitis nodularis chronica helicis, and
weathering nodules.
●● It is seen in association with age and outdoor activities

leading to “weathering” of the ear due to chronic UV

exposure or cold damage.
●● It is common in middle aged men but can occur in any
Figure 9.7  Inflammatory nodules and discharging sinuses
age group and in females. in axilla in hidradenitis suppurativa. (Courtesy: Dr. Piyush
●● The site of affection is the helix or antihelix of the ear.
Kumar, Katihar, India.)
●● It presents as a single or multiple, ovoid or round

painful nodule with raised, rolled edges and an ulcer ●● It leads to abscess formation with accumulation of pus
or a crust in its center, seen unilaterally on the helix or and necrotic tissue.
antihelix of the pinna. ●● Commonly affected sites are hair-bearing areas, mainly
●● On palpation it is firm and usually fixed to the auricular
thighs, buttocks, armpits, shoulders, infra-mammary
cartilage. area, face and neck.
●● DD: Warts, actinic keratosis, keratoacanthoma, basal
●● It starts as a hard, red, painful follicular nodule usually
cell carcinoma. about half an inch in size. Over the next few days, the
Hidradenitis suppurativa lesion becomes softer, larger, and more painful. Soon a
●● Hidradenitis suppurativa (acne inversa, Verneuil’s pocket of pus forms on the top of the boil (Figure 9.8).
disease) is a chronic inflammatory condition affecting ●● In cases of severe infection the surrounding skin turns
the skin region bearing apocrine glands. red, swollen, painful, and warm.
●● It is characterized by inflamed or non-inflamed, deep-
●● DD: Folliculitis, carbuncle, hidradenitis suppurativa.
seated nodules that may progress to discharging/non-
Leiomyoma
discharging sinus tracts (Figure 9.7).
●● It is a rare, benign, smooth-muscle tumor originating
●● Commonly affected sites include the axillary, inguinal,

and anogenital regions. from the arrector pili muscle.

●● It is more commonly seen in female adults.
Furuncle (boils) ●● It is commonly seen on the extremities, trunk, genitals,

●● Furunculosis (singular: furuncle) is a bacterial infection and nipples.

of the hair follicle and the perifollicular soft tissue ●● The different subtypes of cutaneous leiomyomas

caused by Staphylococcus aureus. are angioleiomyoma, piloleiomyoma, and genital

●● It can affect any age group. Risk factors include diabetes, leiomyoma.
immunodeficiency, poor nutrition and hygiene, and ●● Lesions may be single or multiple nodules seen in a

disruption in the epidermal barrier. bunch, linear, dermatomal, or scattered distribution.

208  Nodules: Localized

Figure 9.8  Furuncle seen as erythematous nodule

topped by a pustule. (Courtesy Dr. Niharika Ranjan Lal,
ESIPGIMSR, Kolkata, India.)

The nodules can be skin-colored, pink, red, or brown

and vary in size from 2 mm to 20 mm (Figure 9.9a,b).
●● The presenting complaint is sharp, shooting or aching
pain that may occur spontaneously or be associated with
pressure, cold temperatures, strong emotion, or light touch.
●● Genital leiomyoma are asymptomatic, solitary nodules
found on the scrotum, penis, and vulva or on the
nipple-areola complex.
●● Angioleiomyoma presents as a firm, often painful,
subcutaneous nodule on the lower extremity and is
most commonly seen in females.
●● DD: Keloid, blue rubber bleb nevus, angiolipoma,
neuromas, glomus tumors, neurilemmoma, endometrioma,
granular cell tumor, eccrine spiradenoma, dermatofibroma.

Erythema nodosum (subacute migratory panniculitis

of Vilanova and Pinol)
●● Erythema nodosum (EN) is a non-specific type IV

delayed-hypersensitivity cutaneous reaction to various

antigens. It may occur in association with several systemic
diseases or drug therapies, or it may be idiopathic.
●● It is more common in the 25- to 40-year age group and

in females.
●● Classically, extensor surfaces of lower legs (shins,

ankles, or knees) are affected.

●● It is clinically characterized by red nodules of different sizes

seen on the front and sides of the legs and knees. The thighs,
external parts of the arms, face, and neck are less frequently
involved, and the size of the lesions there is smaller.
Figure 9.9  (a) Grouped erythematous nodules in leiomy-
●● The nodules are bright-red, hot, painful, and slightly
oma. (b) Zosteriform nodules of leiomyoma. (a – Courtesy:
raised above the surrounding skin when they first appear. Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. Santoshdev
As the disease progresses they become purple then fade Rathod, Smt. NHL Municipal Medical College, SCL
through the color changes of a bruise (Figure 9.10a–c). General Hospital, Ahmedabad, India.)
 Nodules: Localized  209

●● They continue to erupt in crops for about 10 days. The

“bruising” color change starts in the second week, becomes
most prominent in the third week, then regresses at any
time from the end of the third week to the sixth week.
●● Constitutional symptoms include fever, general body
ache, and malaise and arthralgias.
●● DD: Acute urticaria, erythema induratum (nodular
vasculitis), familial Mediterranean fever, insect
bites, sarcoidosis, superficial thrombophlebitis,
thrombophlebitis, erysipelas.

Erythema nodosum leprosum (ENL)

●● It is the cutaneous manifestation of type 2 lepra reaction

characterized by the presence of multiple inflammatory

cutaneous nodules and systemic symptoms such as fever,
malaise, arthritis, iritis, neuritis, and lymphadenitis.
●● More common in 25 to 40 year age group but can occur

at any age; it is more common in females.

●● Common sites include the face and extensor surfaces of

arms and legs.

●● It presents as multiple red, firm, painful, blanchable,

delicate, cutaneous and subcutaneous nodules or

plaques distributed bilaterally and symmetrically. They
occur in crops, with lesions found on the face and outer
surface of limbs, rarely on the trunk (Figure 9.11).

Figure 9.10  (a) Erythematous, subcutaneous nodules

bilaterally symmetrically distributed on shins in erythema
nodosum. (b) Erythema nodosum presenting as large
nodule with erythematous surface. (c) Multiple bilateral Figure 9.11  Erythematous, shiny subcutaneous nodules and
erythematous nodules of erythema nodosum. plaque on face in erythema nodosum leprosum. (Courtesy:
(a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.) Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
210  Nodules: Localized

●● At times they can ulcerate or suppurate – erythema

nodosum necroticans (ulcerative ENL).
●● These nodule crops are transient, resolving in several
days. After regression they leave behind bluish/
brownish marks followed by brownish hue in the skin.
●● Fever, generalized malaise, pain, conjunctivitis and
other constitutional symptoms are also seen.
●● DD: Erythema nodosum, erythema induratum (nodular
vasculitis), Sweet syndrome, sarcoidosis.

Localized Sweet’s syndrome (SS)

●● Also known as acute febrile neutrophilic dermatosis and

neutrophilic dermatosis of dorsal hands (NDDH).

●● It is an inflammatory condition associated with many

systemic diseases.
●● Localized SS is an uncommon variant, and there has

been a special interest in describing its occurrence on

the hands as neutrophilic dermatosis of dorsal hands
(NDDH).
●● It is mostly seen in middle-aged woman between 30 and

50 years but can also occur in the elderly and in children.

●● Commonly affected sites are the forearms, hands,

fingers, and palms.

●● It presents as erythematous to purplish plaques and

nodules that heal without scarring. Fever often precedes

the occurrence of nodules (Figure 9.12).
●● Constitutional symptoms, such as fever, malaise, and

arthralgias, can be seen.

●● DD: Cellulitis, atypical mycobacterial infection, atypical

pyoderma gangrenosum, bullous erythema multiforme.

Leukaemia cutis
●● Leukaemia cutis is the penetration of neoplastic

leukocytes or their antecedents into the epidermis,

the dermis, or the subcutis, bringing about clinically
recognizable cutaneous lesions.
●● It can occur in any age group but is more common in

children and has a male predilection.

●● It can occur anywhere on the body.

●● It presents as solitary or numerous violaceous to red-

dark papules, plaques, or nodules. Nodules may develop

central ulceration (Figure 9.13a).
●● Atypical presentations include indurated plaques,

hemorrhagic plaques, perifollicular acneiform papules,

macules, ulcers, bullae, and purpuric patches.
●● DD: Sarcoidosis, drug eruptions, cutaneous B-cell
Figure 9.12  Erythematous, shiny, edematous-looking nod-
lymphoma (Figure 9.13b), large-cell lymphoma ules on forearm in localized Sweet syndrome. (Courtesy:
(Figure 9.13c), cutaneous pseudolymphoma, erythema Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
nodosum, pseudolymphoma.
●● It has also been associated with certain medications,
Pyogenic granuloma (PG) such as oral contraceptives, retinoids, gefitinib,
●● Also known as lobular capillary hemangioma, pyogenic capecitabine, and afatinib.
granuloma is a benign vascular tumor that occurs on ●● Clinical features include small or large, smooth or
the skin and mucous membranes; occasionally it can be lobulated, reddish exophytic vascular nodules that
found subcutaneously or intravascularly. can grow rapidly. Larger lesions become lobulated and
●● It can arise spontaneously in sites of injury or within sometimes develop into mushroom-like, pediculated
capillary malformations. tumors.
 Nodules: Localized  211

Figure 9.13  (a) Erythematous nodules on scalp in leukemia cutis.

(b) Erythematous nodules of B-cell lymphoma. (c) Ulcerated
nodules on the face in large cell lymphoma. (a – Courtesy:
Dr. Balakrishna Nikam, Krishna Institute of Medical Sciences,
Deemed University, Karad, India; b,c – Courtesy: Dr Tanumay
Raychaudhury, The Skin Hospital, Westmead Skin and Cancer
Foundation, Australia.)

●● Common sites are the hands, lower lips and gingiva Prurigo nodularis
(Figure 9.14a,b). ●● Prurigo nodularis is an intensely pruritic, chronic skin
●● PGs have a tendency to bleed profusely. condition characterized by localized or generalized
●● DD: Kaposiform hemangioendothelioma, infantile hyperkeratotic papules and nodules, typically in a
hemangioma, vascular malformations, and Kaposi sarcoma. symmetrical distribution.

Figure 9.14  (a) Lobular capillary hemangioma (pyogenic granuloma) seen as erythematous nodule covered by hemorrhagic
crust on the lip. (b) Lobular capillary hemangioma on the index finger. (a – Courtesy: Dr. Piyush Kumar, Katihar, India;
b – Courtesy: Dr. PC Das and Dr. Piyush Kumar, Katihar, India.)
212  Nodules: Localized

●● They arise in the setting of chronic prurigo and the distribution over the extensor surfaces, trunk, and lower
induction of an uncontrollable itch–scratch cycle. extremities (Figure 9.15a–d).
●● They may be associated with underlying dermatoses such ●● Repeated scratching can lead to excoriation, further
as scabies, stasis dermatitis, allergic contact dermatitis, lichenification, or crusting, often resulting in a
lichen planus, and bullous pemphigoid, and certain hyperpigmented border.
internal diseases such as chronic renal failure, diabetes ●● DD: Kyrle’s disease, acquired perforating disorders.
mellitus, cholestatic jaundice. In addition, anxiety disorders,
depression, and tactile hallucinations can also lead Nodular scabies
to chronic pruritus and thereby to prurigo nodularis as well. ●● Nodular scabies is a well-known clinical variant of

●● Lesions are distributed in areas accessible to chronic scabies, characterized by pruritic nodule that persists
scratching and are often found in a symmetrical even after the specific treatment of scabies (Figure 9.16).

Figure 9.15  (a) Hyperpigmented nodules on the dorsum of the foot in prurigo nodularis. (b) Hyperpigmented nodules with exco-
riated top and hemorrhagic crust in prurigo nodularis. (c) Excoriated papules and nodules of prurigo nodularis on the lower back.
(d) Prurigo nodularis presenting as pigmented nodules with eroded tops. (a–d – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Nodules: Localized  213

of two to eight weeks and can result in morphea-like

residual skin atrophy and hyperpigmentation.
●● Patients may complain of asthma, joint pain, fever, or
fatigue.
●● DD: Allergic contact dermatitis, urticaria, insect bites,
erysipelas, bacterial cellulitis, Churg-Strauss syndrome.

Acroangiodermatitis
●● Also known as acroangiodermatitis of Mali-Kuiper, pseudo-

Kaposi sarcoma, gravitational purpura, stasis purpura.

●● Acroangiodermatitis is a reactive angiodysplasia of

cutaneous veins related with venous inadequacy or with

vascular pathologies, for example, Klippel-Trenaunay
syndrome or stump dermatosis in amputees.
●● It starts as violaceous macules and patches that

progressively form into papules, nodules, or indurated

plaques, often bilateral; it is normally situated on the
lower edematous extremities.
●● Adults are more commonly affected; the condition is

more common in males.

●● The usual sites of affection are dorsal feet and the

extensor surface of lower extremities.

●● It presents as confluent, violaceous or dark-colored

coalescing papules or nodules covering vast regions of

the distal legs. Ulceration and draining may be noted
(Figure 9.17).
●● Bilateral lesions are usually seen in venous inadequacy, while

unilateral lesions suggest a basic vascular abnormality.

●● DD: Kaposi sarcoma, pigmented purpuric dermatosis,

vasculitis, stasis dermatitis.

Scrofuloderma
●● It is a manifestation of endogenous tuberculosis that can

present in an isolated form or coexist with pulmonary

Figure 9.16  Erythematous, shiny papules and nodules on and disseminated forms of tuberculosis.
●● The clinical picture is characterized by the presence
scrotum in nodular scabies. (Courtesy: Dr. Piyush Kumar,
Katihar, India.) of subcutaneous, painless, slow-growing nodules that

●● It probably represents the hypersensitivity reaction to

retained mite parts or antigens. Genital skin and scrotal
skin are the most common sites for such lesions.
●● It can also occur in the axilla and over the breast.

Wells syndrome (Eosinophilic cellulitis)7

●● Wells syndrome is an uncommon eosinophilic disorder

affecting the skin. Affected individuals develop a skin rash

that is preceded by a tingling, itching, or burning sensation.
●● It can occur in any age group, but is more common in

adults; males are commonly affected.

●● It is commonly seen on the arms, legs, trunk, etc.

●● Initially, it presents as burning or pruritus, as well

as localized or diffuse cutaneous erythematous

plaques. These lesions are mildly tender, with patients
subsequently developing cutaneous edema. In addition to
erythema, papules, nodules, blisters, or bullae may occur. Figure 9.17  Pigmented scaly plaque studded with a
●● The second stage is characterized by a progressive
few violaceous nodules in acroangiodermatitis of Mali.
involution of the lesions, which occurs over a period (Courtesy: Dr. Sunil Kumar Gupta, AIIMS, Gorakhpur, India.)
214  Nodules: Localized

evolve to ulcers and fistulous tracts with drainage of ●● DD: Bacterial abscesses, hidradenitis suppurativa,
serous, purulent, or caseous content. atypical mycobacteriosis, sporotrichosis, gummatous
●● The evolution is insidious and can evolve with persistent syphilis, and actinomycosis.
purulent discharge, chronic ulcers, atrophic sequelae, or
spontaneous cure. Atypical mycobacteria infection
●● Cervical lymph nodes are the most frequently ●● Atypical mycobacteria are also known as non-

compromised, but there may be involvement of the axillary, tuberculous mycobacteria (NTM), environmental
inguinal, and pre- and post-auricular, submandibular, mycobacteria, and mycobacteria other than
epitrochlear, and occipital lymph nodes (Figure 9.18a,b). tuberculosis (MOTT). They are small rod-shaped bacilli,
and the disease is caused due to environmental exposure.
●● It can occur at any age group but is more common in

male adults.
●● The usual sites of affection are exposed parts of the body.

●● The morphology of the lesion varies depending upon

the causative organism.

●● Mycobacterium marinum causes fish tank
granuloma or swimming pool granuloma. The
lesion presents as single papule, nodule, or pustule
that breaks open to form ulcers, crusty sores, or
abscesses on the elbows, knees, knuckles, or fingers.
●● Mycobacterium ulcerans causes buruli ulcer
(Bairnsdale ulcer). The lesion starts as a single,
painless, pruritic nodule that ulcerates.
●● M. fortuitum complex (Mycobacterium
fortuitum, M.chelonae, and M. abscessus)
infection leads to a subcutaneous nodule, non-
healing ulcer, abscess, or cellulitis (Figure 9.19).
●● Constitutional symptoms, such as fever,

lymphadenopathy, weight loss, easy fatigability,

shortness of breath, chronic diarrhea, and recurrent
abdominal pain, may be seen in some cases.
●● DD: Bacterial adenitis, lymphoma.

Nodular vasculitis (erythema induratum of Bazin)

●● Erythema induratum of Bazin is a chronic, nodular

eruption considered to be a manifestation of tuberculin

Figure 9.18  (a) Scrofuloderma of inguinal region seen as

nodules and discharging sinuses. (b) Scrofuloderma of Figure 9.19  Subcutaneous nodules, sinuses, and puckered
axillary region. (a–b – Courtesy: Dr. Panchami Debbarman, scars on the back due to M. fortuitum complex infection.
Mumbai, India.) (Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Nodules: Localized  215

hypersensitivity. Pulmonary and lymph node and connective-tissue diseases (systemic lupus
tuberculosis are the most frequent types of underlying erythematosus, rheumatoid arthritis) may be associated
tuberculosis. Other than tuberculosis, associated with CPAN.
conditions include Nocardia and Pseudomonas ●● It is clinically characterized by tender, erythematous,
infection. subcutaneous nodules usually 0.5–3 cm in diameter),
●● It is commonly seen in females of 20 to 30 years of age. mostly on the lower extremities; they may disappear
●● The lower legs are the most commonly involved site. spontaneously or undergo ulceration.
Rarely, thighs, and arms may be involved. ●● Other features include livedo reticularis,
●● It presents as multiple unilateral or bilateral, petechiae, purpura, cutaneous necrosis, and auto
erythematous, tender nodules of varying size. Lesions amputations.
appear in crops and preferentially involve posterior and ●● Other extracutaneous manifestations include arthralgia,
lateral parts of lower legs. They may ulcerate and heal myalgia, constitutional symptoms (such as fever and
over in several weeks, with scarring and pigmentation malaise), and peripheral neuropathy (mononeuropathy
(Figure 9.20). and mononeuritis multiplex).
●● DD: Erythema nodosum leprosum, erythema nodosum, ●● DD: Erythema nodosum, subcutaneous granuloma
cold panniculitis, subcutaneous panniculitic T-cell annulare, nodular vasculitis.
lymphoma.
Orf
Cutaneous polyarteritis nodosa ●● Orf is also known as ecthyma contagiosum,
●● Cutaneous polyarteritis nodosa (CPAN) is an contagious pustular dermatitis, scabby mouth, and
uncommon and rare form of cutaneous vasculitis. It sore mouth.
involves small and medium-sized arteries of the dermis ●● It is caused by a large DNA parapox virus contracted by

and subcutaneous tissue. humans from sheep and goats.

●● Infections (streptococcal, parvovirus B19, ●● Can occur in any age group but is more common in

mycobacterium, hepatitis viruses B and C) adult males.

●● The lesion usually affects fingers, hands, forearms, or

face.
●● After an incubation period of three to five days, a small,

red, itchy, or painful nodule appears. Lesions may be

multiple.
●● The lesion is usually firm, red or blue in color, and varies

from 2–5 cm in diameter. Over the period of three to six

weeks the condition progresses to a vesicle or pustule
that gets crusted.
●● Constitutional symptoms such as fever and

lymphadenopathy may be present.

●● DD: cowpox, milker’s nodule, anthrax, herpetic

whitlow.

Milker’s nodule
●● This is also known as milkmaid blisters, paravaccinia,

and pseudocowpox.
●● It is caused by a double-stranded DNA virus of the

genus Parapoxvirus in people whose occupation

involves close contact with dairy cattle.
●● It is commonly seen in adolescents and adults and is

more common in males.

●● Fingers, hands, or forearms are the most commonly

affected sites.
●● After an incubation period of 5 to 14 days, lesions of

milker’s nodules develop as 0.5–1.5 cm, single, pruritic

or painful, firm, red or purplish red, mobile dome-
shaped papules or nodules. They may evolve to develop
targetoid appearance. Focal ulceration or crusting may
Figure 9.20  Erythematous nodules localized to posterior happen.
legs in erythema induratum. (Courtesy: Dr. Swetha Jain, ●● They have a grayish covering in the target stage and a

Mumbai, India.) verrucous surface in the papillomatous stage.

216  Nodules: Localized

●● Other variants may incorporate vesicles, flaky patches, ●● There is no specific predilection; palms and soles are spared.
or erosions. Local lymphadenopathy, fever, diarrhea, or ●● It clinically presents as clusters of whitish-pink, small,
abdominal cramping might be present. dome-shaped, umbilicated papules, that may coalesce
●● DD: Orf, pyogenic granuloma, anthrax, erythema and/or progress to form giant molluscum (>10 mm)
multiforme, staphylococcal abscess. (Figure 9.22a,b).
●● It may develop eczematous reaction – “eczema
Keloid molluscatum” – in atopic children.
●● A keloid is an abnormal proliferation of scar tissue that ●● DD: Calcinosis cutis, tuberous xanthoma, gouty tophi.
forms at the site of a cutaneous injury (e.g., on the site
of a surgical incision or trauma); it grows beyond the
original margins of the scar and does not regress.
●● It is usually seen between 10 to 30 years of age with a

slight female preponderance. People with pigmented

skin and Asians show a greater tendency to develop
keloids.
●● It can affect any part of the body but is mainly seen on

the chest, shoulders, earlobes, and cheeks.

●● It is usually asymptomatic but may be tender, painful, or

pruritic. It presents as a localized raised lumpy or ridged

area that is flesh-colored, pink, or red with claw-like
projections (Figure 9.21).
●● Keloids tend to be larger than the original wound itself.

They may take weeks or months to develop fully.

●● DD: Hypertrophic scar, dermatofibroma, lobomycosis.

Giant molluscum contagiosum

●● It is a viral infection caused by molluscipox viruses 1–4,

communicated via direct and indirect cutaneous contact,

auto-inoculation, and in adults via sexual contact.
●● Clusters of cases are seen in swimming pools, play

fields, school compounds, and overcrowded houses.

Figure 9.22  (a) Erythematous nodules with central

umbilication seen in giant molluscum. (b) Giant molluscum
contagiosum in a seropositive male. (Courtesy: Dr. Piyush
Figure 9.21  Flesh-colored, shiny nodule in earlobe keloid. Kumar, Katihar, India.)
 Nodules: Localized  217

Figure 9.23  (a) Amelanotic melanoma presenting as erythematous noduloulcerative lesion covered by crust and eschar.
(b) Poroma presenting as crusted nodule on the palm. (a – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr.
Balakrishna Nikam, Krishna Institute of Medical Sciences, Deemed University, Karad, India.)

Amelanotic melanoma ●● DD: Pyogenic granuloma, benign lichenoid keratosis,

●● Cutaneous amelanotic melanoma (AM) is an amelanotic inflamed seborrhoeic keratosis, eccrine poroma, basal
or a hypomelanotic subtype of cutaneous melanoma that cell carcinoma, squamous cell carcinoma, amelanotic
shows no or little pigment on macroscopic inspection or melanoma, and psoriasis.
dermoscopic evaluation or lacks melanin pigmentation
Eccrine poroma
on histological examination.
●● Eccrine poroma is a benign adnexal neoplasm
●● There are three main clinical forms of AM: (a)

erythematous macule with epidermal changes on sun- originating from eccrine epithelial cells that show
exposed skin, (b) skin-colored dermal plaque without tubular (generally distal ductal) differentiation.
●● It commonly presents in fourth to sixth decade of life.
epidermal changes, and (c) papulonodular form.
●● It usually occurs on palms and soles.
●● The papulonodular form accounts for 58% cases of AM
●● It presents as asymptomatic or painful, slow-growing, or
and may present as an ulcerated nodule or a vascular
lesion (Figure 9.23a). stable nodular lesions. Clinically, the lesion is noted as
●● Despite the absence of melanin, AMs can be skin- solitary skin-colored or pink papule, plaque, or nodule
colored, red, pink, or erythematous, among which red less than 2 cm in diameter. The surface may be ulcerated
AMs account for nearly 70% of AM. and sometimes pigmented (Figure 9.23b).
●● Some patients may develop multiple poromas
●● It often occurs in acral sites, with the highest incidence

of 20–28%. (poromatosis) simultaneously.

●● DD: Granuloma pyogenicum, cutaneous squamous cell
●● DD: Pyogenic granuloma, hemangioma.
carcinoma, melanoma.
Clear cell acanthoma
●● It is a rare, benign tumor of unknown etiology. Kaposi sarcoma
●● Most commonly located on the lower extremities, clear ●● Kaposi sarcoma (KS) is the second most common tumor

cell acanthomas can also appear on the trunk, forearm, in HIV patients with CD4 counts less than 200 cells/
face, and inguinal area, and a few cases of nipple and mm3 and is an AIDs-defining illness.
areola lesions have been reported.  ●● Types of KS:

●● Lesions are usually solitary; however, there are reports ●● Classic type: occurs primarily in patients
of rare cases of multiple disseminated clear cell over 50 years old of Eastern European and
acanthomas. These tumors clinically present in middle- Mediterranean descent. These patients are at greater
aged to elderly individuals. risk for secondary malignancies.
●● Clinically it presents as a solitary red or red-brown, ●● Endemic type: unusual predilection for the pediatric
dome-shaped papule or nodule. A peripheral, wafer-like population and mirrors HHV-8 seropositivity.
scale (collarette) is classically described in a majority of ●● AIDS related: second most common tumor in HIV
lesions but may not always be present. The surface may patients with CD4 counts less than 200 cells/mm3
also have a crusted or moist appearance and may bleed and is an AIDs-defining illness.
with minor trauma. ●● Iatrogenic: seen in transplant recipients.
218  Nodules: Localized

●● Clinically all forms of KS are characterized by multiple cylindroma affects middle-aged and elderly people.
pigmented, painless, non-blanchable, pale-pink to Multiple cylindromas are inherited and highly
vivid-purple macules or papules. associated with Brooke-Spiegler syndrome.
●● Larger plaques on the trunk often follow the skin ●● Clinically lesions are firm, rubbery nodules with
creases as oblong lesions. red, pink, or sometimes blue stain that range in
●● Occasionally, lesions form exophytic, ulcerated, and size from a few millimeters to several centimeters
bleeding nodules that can be associated with painful edema. (Figure 9.24a).
●● Oral and visceral lesions are common with AIDS-related ●● The most frequent location for the solitary form is
KS; lymphedema is frequently seen in endemic KS. the head and neck. The multiple form usually occurs
●● DD: Spindle cell hemangioma, acquired tufted angioma, on the head and neck followed by the trunk and the
kaposiform hemangioendothelioma, cutaneous extremities and the pubic area. When multiple lesions
angiosarcoma, dermatofibrosarcoma protuberans, cover the scalp, the neoplasm is referred to as “turban
aneurysmal dermatofibroma, acroangiodermatitis. tumors.”
●● They are usually painless, but pain or paraesthesia may
Merkel cell tumor occur due to nerve compression.
●● Merkel cell carcinoma (MCC) is a rare, clinically ●● DD: Trichoepithelioma, spiradenoma, angiolymphoid
aggressive cutaneous neuroendocrine neoplasm with a hyperplasia with eosinophilia (Figure 9.24b).
high mortality rate.
●● Clinically it presents as painless subcutaneous mass Granuloma faciale (Lever type)
with a cystic, nodular, or plaque-like appearance. ●● Granuloma faciale is an uncommon and benign skin

●● Lesions can vastly range in color, most often presenting disease portrayed by chronic leukocytoclastic vasculitis
as red/pink, blue/violaceous, or skin-colored. with a neutrophilic predominance.
●● They can exhibit overlying telangiectasia or a shiny ●● It is mostly seen in the third to fifth decade of life, with

surface, and may be confused with basal cell carcinoma. a male predilection.
●● Lesions occur in head and neck region in more than ●● It classically affects the face (nose, preauricular area,

50% cases and have a slighter greater predilection for cheeks, forehead, helix of the ear).
sun-exposed areas. ●● A typical lesion is an asymptomatic solitary,

●● DD: Epidermoid cyst, lipoma, dermatofibroma, erythematous nodule, in the case of the Lever subtype.
amelanotic melanoma, basal cell carcinoma, squamous It may also present as multiple soft-brown to red
cell carcinoma, lymphoma, sarcoma. papules, nodules, or plaques.
●● The lesions are smooth surfaced with prominent
Cylindroma follicular orifices and telangiectasia.
●● Cylindromas are benign adnexal tumors showing ●● DD: Erythema elevatum diutinum, sarcoidosis,

an eccrine and apocrine differentiation. Solitary cutaneous lymphomas, lymphocytoma cutis, discoid

Figure 9.24  (a) Multiple erythematous nodules of cylindroma in a case of Brooke-Spiegler syndrome. (b) Multiple grouped
erythematous nodules of angiolymphoid hyperplasia with eosinophilia. (a – Courtesy: Dr. Hiral Shah, Baroda Medical
College, Vadodara, India; b – Courtesy: Dr Piyush Kumar, Katihar, India.)
 Nodules: Localized  219

lupus, basal cell carcinoma, lymphocytic infiltrate of ●● DD: Melanoma, Reed nevus, vascular tumors,
Jessner, fixed drug eruption, lupus vulgaris, and fungal basal cell carcinoma, juvenile xanthogranuloma,
and mycobacterial infections. pyogenic granuloma (lobular capillary hemangioma),
melanocytic nevi, non-genital warts.
Spitz nevi
●● Spitz nevi is also known as epithelioid and spindle-cell Cutaneous leishmaniasis
nevus, benign juvenile melanoma, and Spitz’s juvenile ●● Leishmaniasis is a parasitic disease with multiple

melanoma. clinical forms and is transmitted by sand flies

●● A spitz nevus is slow- or fast-growing tumor that may infected with the protozoa Leishmania. The clinical
arise de novo or within a melanocytic nevi. The exact manifestation of the infection depends on the species
etiology of growth remains unknown. causing infection and the host’s immune response.
●● It occurs mainly before the second decade of life; ●● It can occur at any age group but is more common in adults.

females are more commonly affected. ●● It is more common in males.

●● Commonly affected sites include face, neck, legs, or oral ●● Usually affected sites are exposed areas of the body,

cavity. mainly the face and extremities.

●● The most typical lesion is single dome-shaped, red or ●● Skin lesions develop at the site of inoculation and start as

pigmented papule or nodule (Figure 9.25a,b). single or multiple small red papules. The lesion gradually
●● The color may vary from pink to orange-red to enlarges in size and may develop central ulceration. The
pigmented. Pigmented spitz moles have an irregular ulcer is typically painless and may exude pus or may
border and are black, blue, or dark tan. be dry with a crusted scab. The lesions may heal over
several months with atrophic scarring (Figure 9.26a,b).

Figure 9.26  (a) Cutaneous leishmaniasis presenting

as erythematous nodule with central crust on the
face. (b) Cutaneous leishmaniasis seen as erythema-
tous noduloulcerative lesion on the nose. The surface
is notable for eschar. (a,b – Courtesy: Dr. Ennakshee,
Figure 9.25  (a) Spitz nevus. (b) Close-up view of spitz nevus. Government Medical College, Jammu, India.)
220  Nodules: Localized

●● Sporotrichosis spread and chronic disease can occur. ●● Although the head, neck, and trunk are the most
●● Mucocutaneous lesions are painful and present after common sites for JXG, it can appear anywhere on the
resolution or treatment of primary lesions. They body, including the groin, scrotum, penis, clitoris,
generally affect the mucous membranes of the eyes and eyelids, toenails, palms, soles, and lips.
genital tracts and may cause extensive disfigurement. ●● Extracutaneous involvement is usually restricted to the
●● DD: Chromoblastomycosis, sporotrichosis, Wegener’s eye, specifically the iris, but may also occur in bone,
granulomatosis, lymphomas. lung, and liver.
●● DD: Dermatofibroma, Langerhans cell histiocytosis,
Schwannoma mastocytosis, Spitz nevus, xanthomas.
●● Schwannomas or neurilemmoma are benign

encapsulated tumors of nerve sheath origin. Necrobiotic xanthogranuloma

●● Although they commonly occur as solitary lesions ●● Necrobiotic xanthogranuloma, a non-Langerhans

(90%), they can be associated with several central histiocytosis, is a subset of inflammatory form of
neurological tumors (usually meningiomas, 5%), normolipemic xanthoma.
neurofibromatosis type 2 (3%), or appear as multiple ●● The age of onset ranges from 17 to 85 years, without any

lesions (schwannomatosis, 2%). sex predilection.

●● Clinically, cutaneous schwannomas (CS) usually range ●● The periorbital region is the most frequent site of

in size from 0.25–3.00 cm and generally occur in the involvement, followed by the trunk, face, and extremities.
head and neck region. ●● The most frequent skin lesion is an indurated papule,

●● CS are generally asymptomatic; however, when pain is nodule or plaque. The color varies from violaceous to
present, it is usually associated with compression the red-orange, often with a yellowish hue (Figure 9.28).
adjacent structures of nerve and the paraesthesia restricted
on the tumor site or radiating along nerve of origin.
●● They most often occurs in the fourth and fifth decades

of life, without significant evidence of sex predilection.

●● DD: Pilomatricoma, epithelial cysts, lipoma, desmoid

tumor, and rheumatoid nodule.

Juvenile xanthogranuloma (JXG)

●● JXG is a member of the non-Langerhans cell group of

histiocytic proliferative disorders and is a relatively

uncommon benign cutaneous fibrohistiocytic lesion.
●● JXG is a disease of the young child. Median age of onset

is two years, but lesions may be present at birth. Male

preponderance is seen.
●● Clinically it starts as reddish-yellow macules/papules,

which may enlarge and evolve into yellow-brown

patches/plaques or nodules with surface telangiectasia.
The consistency is generally firm and rubbery. Giant
JXG, defined as a lesion greater than 2 cm in diameter,
has been reported (Figure 9.27).

Figure 9.27  Yellow-orange firm nodule of juvenile xan-

thogranuloma on the foot. (Courtesy: Dr. Anup Kumar Figure 9.28  Yellow-orange and brownish nodules of
Tiwary, Consultant Dermatologist, Yashoda Hospital and necrobiotic xanthogranuloma. (Courtesy: Dr. Hiral Shah,
Research Center, Ghaziabad, India.) Baroda Medical College, Vadodara, India.)
 Nodules: Localized  221

●● There is a characteristic absence of generalized ●● The lesions are usually devoid of a central punctum,
lymphadenopathy. and yellowish, cheesy, semisolid or oily secretion can be
●● Ophthalmic features of necrobiotic xanthogranuloma expressed through the same.
include eyelid lesions, orbital masses, conjunctival ●● Areas of involvement include the trunk, neck, groin,
involvement, keratitis and scleritis, episcleritis or scalp, and proximal extremities. However, uncommon
anterior uveitis. sites, including face, glutei, breasts, or flexures, may be
●● Other systemic findings include hepatosplenomegaly, affected.
arthralgia/arthritis, hypertension, generalized ●● DD: Epidermoid cysts, neurofibromatosis, lipomatosis.
sensorineural polyneuropathy, cardiomyopathy, and
pulmonary fibrosis. Xanthoma (tendinous, tuberous, eruptive,
●● DD: Diffuse normolipemic plane xanthoma, normolipemic)
granuloma annulare, necrobiosis lipoidica, juvenile ●● Xanthomas are a common manifestation of lipid

xanthogranuloma, and deep xanthomas. metabolism disorders.

●● These may occur in persons of any age.
Chondroid syringoma ●● Tuberous xanthoma are the nodules that are frequently

●● Chondroid syringoma is a benign, skin appendageal localized to extensor surface of elbows, knees, knuckles,
tumor. and gluteal region (Figure 9.30).
●● It is also known as mixed tumor of the skin as it contains ●● Tendinous xanthoma are firm subcutaneous nodules

epithelial and mesenchymal stromal components. found in facia, ligaments, and Achilles tendon or
●● Is usually occurs in middle-aged males. extensor tendons of the hands, knees, and elbows.
●● The lesions are typically located in the head and neck ●● DD: Nodular amyloidosis, erythema elevatum

region. diutinum, histoid leprosy.

●● They are slow-growing, subcutaneous or dermal

nodules. Rarely, rapid growth, ulceration, and necrosis Tophus (podagra)

may be noted. ●● It is a disorder of hyperuricemia; elevated uric acid

●● The malignant form occurs predominantly in females, levels lead to precipitation of monosodium urate
has no age-related predilection, and is observed more (MSU) crystals in skin, soft tissue, joints, and
commonly on the extremities. kidneys.
●● DD: Epidermal cyst, pilar cyst, calcifying epithelioma, ●● It is considered a lifestyle disease; risk factors include

solitary trichoepithelioma. high protein and alcohol intake and metabolic

syndrome.
Steatocystoma multiplex8 ●● Usually occurs in the fourth sixth decade of life, males

●● Steatocystoma multiplex (SCM) is a rare, commonly more affected than females.

hamartomatous disorder of the pilosebaceous unit ●● Acral involvement is most common in distal to

characterized by sebum-containing cysts in the dermis. proximal joints.

●● Clinically, it manifests as skin-colored to yellowish,

soft or firm, cystic or dome-shaped papules or nodules

(Figure 9.29).

Figure 9.29  Multiple, skin-colored to yellowish nodules Figure 9.30  Tuberous xanthomas seen as shiny, yellow
in steatocystoma multiplex. (Courtesy: Dr. Piyush Kumar, nodules and plaques on the gluteal region. (Courtesy Dr.
Katihar, India.) Deverashetti Srinivas, Nizamabad, India.)
222  Nodules: Localized

●● Cutaneous lesions are known as tophi and are characterized ●● A clinical variant is miliarial gout.
as firm, skin-colored papules and nodules (Figure 9.31a,b). ●● Joint involvement may present in an acute manner, with
●● Gout nodulosis is a rare presentation of the disease in excruciating pain and swelling in one joint, commonly
which multiple nodular tophi develops in the absence of the great toe (podagra), and acute episode usually
gouty arthritis (Figure 9.31c).9 lasts for 7 to 14 days. Multiple episodes result in joint
destruction and mutilation.
●● DD: Calcinosis cutis, tuberous xanthoma, giant
molluscum.

Dilated pore of Winer

●● Dilated pore of Winer results from an obstruction

of the follicle ostium in a process similar to that of

inflammatory cystic acne.
●● Most cases of dilated pore of Winer are diagnosed in

individuals older than 40 years.

●● A male preponderance is seen.

●● A dilated pore of Winer usually appears as a solitary

large comedone on the face, predominantly on the

upper lip, cheek, or forehead. The lesion can also be
found on the trunk, most commonly the back. A rare
incidence involving the external ear canal has also been
reported.
●● The skin surrounding the pore appears to be

unchanged, with no inf lammation or

induration.
●● DD: Epidermal inclusion cyst, Favre-Racouchot

syndrome, trichilemmal cyst (pilar cyst),

trichoepithelioma.

Tertiary syphilis (gumma)

●● Gummas are the characteristic lesions of tertiary

syphilis and occur three to ten years after primary

infection.
●● The condition is more common in males.

●● Commonly involved sites are thighs, buttocks,

shoulders, forehead, and scalp.

Figure 9.31  (a) Multiple tophi on the toes in a patient of gout. (b) Bilateral nodules of tophi on the planatar aspect of the
feet. (c) Multiple tophi on the hand and foot in a case of gout nodulosis. (a,c – Courtesy Dr. Piyush Kumar, Katihar, India;
b – Courtesy: Dr Hiral Shah, Baroda Medical College, Vadodara, India.)
 Nodules: Localized  223

Figure 9.32  Erythematous nodule with crusted surface

in tertiary syphilis (gumma). (Courtesy: Dr. Hiral Shah,
Baroda Medical College, Vadodara, India.)

●● The gumma appear as cutaneous plaques or nodules of

irregular shape and outline and are often single lesions
on the arms, back, and face. The size of lesions may vary
from 2–10 cm (Figure 9.32). Figure 9.33  Skin-colored, smooth-surfaced, cerebriform
nodule of nevus lipomatosus.
●● Gummas are usually painless even when they ulcerate.
Their central necrotic tissue may turn into a slimy,
stringy mass, and it is this that gives rise to the name
“gumma.” Multiple gummas tend to coalesce, the or nodules in unilateral, band-like, linear, or
bridges of skin between them gradually undergoing zosteriform distribution (Figure 9.33).
necrosis. They have a tendency for peripheral healing ●● The solitary form usually occurs after the age of 20
with tissue-paper scarring. and presents with a single nodular lesion with no
●● Gummas that start in bone or muscle also tend to particular predilection sites.
ulcerate the skin; this form is difficult to differentiate ●● DD: Nevus sebaceous, skin tags, neurofibroma,
from a nodular syphilide. lymphangioma, hemangioma, and focal dermal
●● DD: Sarcoidosis, xanthogranulomas, xanthomata. hypoplasia (Goltz syndrome).

Nevus lipomatosus Melanocytic nevus

●● Also known as nevus lipomatosus cutaneous ●● Melanocytic nevi are a localized proliferation of nests of

superficialis, it is an uncommon benign hamartomatous nevus cells (melanocytes).

skin lesion defined by the presence of aggregates of ●● Most cases are sporadic, but familial occurrence

mature adipose tissue among the collagen bundles of is not uncommon. It may also appear in patients
the dermis. receiving chemotherapeutic agents (BRAF-inhibitor
●● It is more common in children and young adults drugs).
(first two decade of life); a male preponderance is ●● Most frequently seen in the Caucasian race and fair-

seen. skinned people, global incidence is 1–1.5%.

●● The gluteal region, upper posterior thigh, and lumbar ●● It is classified into

back are most commonly affected. ●● Congenital – small, medium, giant

●● Two clinical types are distinguished: the classical type ●● Acquired – junctional, intradermal, compound
and the solitary type.10 ●● Special types dermal melanocytosis – speckled
●● The classical type, Hoffman and Zurhelle type, (lentiginous), nevus of Ota/Hori, café-au-lait
consists of multiple soft, non-tender, pedunculated, macules, Mongolian spots, hairy nevi, halo nevus,
cerebriform, yellowish or skin-colored papules perinevoid dermatitis (Meyerson’s phenomenon)
224  Nodules: Localized

●● DD: Mastocytoma, melanoma, verruca plana,

acrochordons.

Mastocytoma
●● A mastocytoma is a tumor of mast cells, which

are derived from myeloid stem cells and located in

connective tissues, predominantly in the skin and
mucosal linings.
●● C-kit gene mutation causes increased concentration

of growth factors and interleukins that induce

proliferation of mast cells is responsible.
●● This usually affects children.

●● Lesions are noted as single or a few yellowish-brown

pigmented macules, papules, or nodules of variable

size. The lesions are generally non-tender but can be
associated with intermittent itching and erythema
(Figure 9.35a,b).
●● Rubbing or stroking the lesion can degranulate the

mast cells and cause itching, and edema of the skin,

Figure 9.34  Brownish, dome-shaped nodule bearing hair known as Darier sign, which is pathognomonic of
on the face in compound nevus. (Courtesy: Dr. Piyush the disease.
Kumar, Katihar, India.) ●● The blistering and bullous presentation is common in

children less than three years of age and can also be

present in diffuse and severe forms of the disease.
●● The diffuse disease presents with thickened, leathery
●● Melanocytic nevi may present as solitary or multiple skin without individual lesions.
pigmented nodules to plaques. The color of the lesion ●● DD: Melanocytic nevus, melanoma, dermatofibroma,
varies and gives clues to the type of nevi – skin-colored insect-bite reaction.
(intradermal), dark-brown to black (compound)
(Figure 9.34), black (congenital), and blue (deep Nodular melanoma
dermal). The surface may show hairs. ●● Nodular melanoma is a common subtype of melanoma
●● Color of the nevus becomes darker with progression in and shows aggressive vertical growth phase from the
the Fitzpatrick skin type. early stage.

Figure 9.35  (a) Solitary nodule of mastocytoma on the leg. (b) Flesh-colored nodule of mastocytoma on the upper lip
(arrow) of a child. (a – Courtesy: Dr. Sunil Kumar Gupta, AIIMS, Gorakhpur, India; b – Courtesy:
Dr. Mandeep Bhukar, Rohtak, India.)
 Nodules: Localized  225

●● It can appear de novo in normal-appearing skin or

in existing melanoma of other types or dysplastic
nevi.
●● It is more commonly seen in fair-skinned individuals
and is common in elderly men.
●● It presents as a solitary rapidly progressing,
dome-shaped nodule of black or variegated color
(black, red, etc.). The surface shows variable
ulceration, bleeding, crusting, or warty surface
(Figure 9.36a).
●● Regional lymph nodes and cutaneous metastases may
occur (Figure 9.36b). The prognosis is poor.
●● In amelanotic melanoma up to one-third of
nodular melanomas may not be pigmented and
appear red or skin-colored. They carry an even
worse prognosis.

Dermatofibrosarcoma protuberans11
●● Dermatofibrosarcoma protuberans (DFSP) is a rare soft-

tissue tumor that involves the dermis, subcutaneous fat,

and, in rare cases, muscle and fascia.
●● It typically presents as a slow-growing, firm plaque on

the trunk of young adults.

    

Figure 9.36  (a) Erythematous noduloulcerative lesions and multiple black nodules in a case of nodular melanoma with
cutaneous metastasis. (b) Ulcerated, pigmented nodule of malignant melanoma on the palm, with pigmented nodules of
metastatic lesions on the forearm and arm. (Courtesy: Dr. PC Das and Dr. Piyush Kumar, Katihar, India.)
226  Nodules: Localized

Figure 9.37  Dermatofibrosarcoma protuberans pre-

senting as subcutaneous nodule with flesh-colored and
pigmented nodules on its surface. (Courtesy: Dr. Piyush
Kumar, Katihar, India.)

●● It is clinically characterized by asymptomatic, skin-

colored to red-brown indurated plaque which eventually
develops multiple raised violaceus to red-brown nodules
(Figure 9.37).
●● As they grow larger, some can ulcerate and become
painful. The majority of DFSPs occur on the trunk
(50%), followed by the extremities (35%), and then head
and neck (15%).
●● DD: Cutaneous melanoma, dermatofibroma.

Mycetoma
●● Mycetoma is caused by fungi as well as filamentous

bacteria. Lesions are more common on the feet, shins,

and hands.
●● The earliest clinical manifestation is a hard painless

nodule that spreads slowly to produce papules and

sinuses that discharge fluid containing granules onto
the skin surface (Figure 9.38a,b).
●● The original site of infection is distorted by local tissue

swelling, formation of chronic sinuses, and late bone

involvement.
●● DD: Bacterial osteomyelitis, tuberculous osteomyelitis,

hidradenitis suppurativa, Kaposi sarcoma, and

cutaneous tuberculosis.

Phaeohyphomycosis
●● Phaeohyphomycosis is caused by dark-walled

(dematiaceous) fungi. These fungi are found in all

climates, although they are more common in tropical
climates. Immunosuppressed patients with HIV
infection or AIDS, transplant recipients, and diabetic Figure 9.38  (a) Nodule and discharging sinuses on the heel
patients are more susceptible. in mycetoma. (b) Localized swelling and discharging nod-
●● In subcutaneous phaeohyphomycosis following local ules in actinomycetoma. (a – Courtesy: Dr. Piyush Kumar,
trauma or inoculation with foreign material, patients Katihar, India; b – Courtesy: Dr Rajeev Ranjan, Ara, India.)
 Nodules: Localized  227

develop a slow-growing solitary lesion (generally a cyst

or a nodule, or possibly a plaque or abscess) normally
located on the extremities.
●● DD: Lipoma, epidermal or synovial cysts, fibromas,
foreign body cysts, and bacterial abscesses.

Histoplasmosis
●● This is a chronic granulomatous disease caused by the

dimorphic fungus Histoplasma capsulatum.

●● Skin lesions are not specific and are characterized by

their polymorphism, ranging from papules, plaques

with or without crusts, pustules, and nodules to
mucosal ulcers, erosions, and lesions resembling
molluscum contagiosum.
●● The most commonly affected sites are the face, trunk,

extremities, oral, perianal and genital mucosa.

Oropharyngeal ulcers present as painful nodules on the
tongue, gums, and larynx.

Cryptococcosis
●● It is an opportunistic fungal infection caused by

Cryptococcus neoformans.
●● Skin lesions are frequently a sentinel for disseminated

disease; however, primary cutaneous lesions do

occur in immunocompetent persons. The primary
cutaneous lesion may be a papule, maculopapular
lesion with an ulcerated center or a violaceous
nodular lesion. Often there is a discharging sinus
that connects to the underlying deep abscess or the
underlying bone.

Neurofibroma
●● Neurofibromas are the most prevalent benign peripheral

nerve sheath tumor.

●● They are comprised of Schwann cells, fibroblasts,

perineural cells, and mast cells in a variably myxoid

background.
●● Patients are often asymptomatic; however, irritation,

mild pruritus, pain, or paresthesia, can occur; the most

common chief complaint is cosmetic appearance.
●● Often appearing as a soft, skin-colored papule or small

subcutaneous nodule, they arise from endoneurium

and the connective tissues of peripheral nerve sheaths Figure 9.39  Nodule of neurofibroma in a patient of neuro-
(Figure 9.39). fibromatosis type 1. (Courtesy: Dr. PC Das, Katihar, India.)
●● A characteristic “buttonhole sign” where, on palpation,

the lesion retracts into the subcutis and reappears on

the release of pressure, is seen. ●● Frequent irritation seems to be an important
●● Localized neurofibromas can occur anywhere on the causative factor, especially in persons who are obese.
body, with a predilection for the trunk, head/neck, and Hormone imbalances may facilitate the development
extremities. of acrochordons (e.g., high levels of estrogen and
●● Neurofibromas may appear without any known cause or progesterone during pregnancy, high levels of growth
may be associated with neurofibromatosis. hormone in acromegaly). Epidermal growth factor
●● DD: Acrochordons, leiomyoma, schwannoma. (EGF) and alpha tissue growth factor (TGF) have also
been implicated in the development of these lesions.
Macro acrochordon ●● The onset of acrochordon increases in frequency up
●● This is also known as fibroepithelial polyp, skin tag, soft through the fifth decade. As many as 59% of persons
fibroma, and giant acrochordon. may have acrochordons by the time they are age 70.
228  Nodules: Localized

●● Commonly, lesions are found on the groin, upper thigh, ●● Nodules are skin-colored, can be solitary or multiple,
and back. and their size ranges from 2 mm to more than 5 cm in
●● It presents as large, pedunculated, bag-like, soft diameter.
fibromas (Figure 9.40a,b). ●● They are firm, non-tender, and movable within
●● It can cause considerable discomfort for patients when the subcutaneous tissue; however, they could also
located in the axilla and genital regions. be attached to underlying structures such as the
●● DD: Neurofibromatosis Type 1, genital warts, periosteum, tendons, or bursae.
melanocytic nevi, premalignant fibroepithelial tumor ●● Most rheumatoid nodules are found on areas prone
(Pinkus tumor), seborrheic keratosis. to mild repetitive trauma, such as bony prominences,
extensor surfaces, or adjacent to joints.
Rheumatoid nodules12 ●● They have a predilection for the elbows and fingers.
●● Classic rheumatoid nodules occur in approximately ●● They are most frequently found on extensor surfaces
20% to 25% of patients with seropositive rheumatoid of the proximal forearm, metacarpophalangeal, and
arthritis (RA) and are the most common extra-articular proximal interphalangeal joints, occiput, back, and
manifestation of RA. heel.
●● DD: gouty tophi, subcutaneous granuloma annulare,
tumoral calcinosis, fibromas, xanthomas, subcutaneous
sarcoidosis.

Sebaceous hyperplasia
●● Sebaceous hyperplasia is a benign dermatosis

characterized by asymptomatic solitary or multiple

lesions on the face, with a predilection for the forehead.
●● Lesions are common in middle-aged persons and occur

rarely in early adult life.

●● Clinically they are characterized by single or multiple

yellow papules or skin nodules, usually 0.2–0.3 cm in

diameter with a central area of umbilication.
●● DD: Basal cell carcinoma, sebaceous adenoma.

Lipoma
●● It is usually seen in people between 25 and 50 years of

age.
●● It is more common in males.

●● The trunk and upper limbs are the common sites but it

can occur on other parts of the body too.

●● It is clinically characterized by soft, fluctuant, freely

mobile nodules with normal-appearing overlying skin

(Figure 9.41a–c).
●● A characteristic “slippage sign” may be elicited by

gently sliding the fingers off the edge of the tumor. The
tumor will be felt to slip out from under, as opposed
to a sebaceous cyst or an abscess that is tethered by
surrounding induration. The overlying skin is typically
normal.
●● DD: Epidermoid cyst, abscess, liposarcomas.

Subcutaneous granuloma annulare

●● Subcutaneous granuloma annulare (SGA) is a rare type

of granuloma annulare (GA), a benign granulomatous

inflammatory disease that usually involves the skin or
deeper tissues.
●● Etiology is unknown; it may be associated with systemic

Figure 9.40  (a) Skin-colored, soft, pedunculated nod- diseases such as diabetes, rheumatoid arthritis, and
ule seen in macro acrochordon. (b) Giant acrochordon malignancy.
showing surface necrosis due to a thread tied around its ●● It is most frequently seen in children and young adults;

peduncle. (Courtesy: Dr. Piyush Kumar, Katihar, India.) males are more commonly affected.
 Nodules: Localized  229

Figure 9.41  (a) Subcutaneous nodules of lipoma on the back. (b) Lipoma causing pale colored swelling of the pulp of
index finger. (c) Intraoperative visualization of lipoma. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● It is most commonly located on the anterior aspects nodules are currently considered within the spectrum
of the lower legs, hands, head, and gluteal of subcutaneous granuloma annulare.
region. ●● DD: Rheumatoid nodule, necrobiosis lipoidica,
●● It is clinically characterized by subcutaneous nodules epithelioid sarcoma.
with no inflammatory appearance at the skin surface
(Figure 9.42). Histoid leprosy13
●● In rare cases, subcutaneous granuloma annulare may ●● Also known as histoid Hansen’s disease,

extend, involving deeper soft tissues, and produce a histoid nodules, mycobacterium leprae histiocytoma
destructive arthritis and limb deformity. (cutis), and leprous histiocytoma.
●● The lesions described in children under the names ●● Histoid leprosy is considered to be a variant of

pseudo rheumatoid nodules and benign rheumatoid lepromatous leprosy caused by Mycobacterium leprae.
230  Nodules: Localized

Figure 9.43  Skin-colored and white, shiny nodules on

scrotum in scrotal calcinosis. (Courtesy: Dr. Anup Kumar
Tiwary, Consultant Dermatologist, Yashoda Hospital and
Research Center, Ghaziabad, India.)

nodules (Figure 9.43). The lesions may become tender

and ulcerate spontaneously to reveal a chalky white
discharge, rich in calcium carbonate/phosphate.
●● DD: Tophus, mycetoma, giant molluscum, osteoma cutis.

Osteoma cutis
●● Osteoma cutis or cutaneous ossification is a rare and

Figure 9.42  Multiple grouped, skin-colored nodules and benign dermatological disease characterized by bone
plaques in subcutaneous granuloma annulare. (Courtesy: formation in the dermis or subcutaneous tissue.
Dr. Shahid Hassan, Purnea, India.) ●● Primary osteoma cutis (without any pre-existing lesion)

can occur in isolation or in association with metabolic

●● It may be seen in all ages (most commonly 20–39 years) syndrome. Secondary osteoma cutis is associated
with male preponderance. with cutaneous inflammatory processes, scars,
●● The lesions are located on the back, buttocks, face, dysembryoplasia, or neoplasia.
extremities, or over bony prominences. Oral mucosal ●● The most common site is the face in females and the

lesions are rarely seen; palms, sole, and nasal mucosa scalp in males. Other sites of involvement include the
are not affected commonly. breasts, buttocks, and extremities.
●● The nodules of histoid leprosy may be soft to firm; ●● Clinical features can range from asymptomatic single

superficially placed, deeply fixed, or subcutaneous; to multiple lesions. They range in size from 0.1–5.0 cm.
reddish or skin-colored; and dome-shaped, oval, or These lesions may present as papules, plaques, nodules,
regular in contour, with overlying shiny or stretched or as miliary lesions. On palpation, they are hard and
skin. The lesions may ulcerate at times. The number of can sometimes be responsible for skin discoloration that
lesions may vary from 3 to 50. becomes white or yellowish (Figure 9.44).
●● Other lesions are neurofibroid or “molluscum- ●● In rare cases, the overlying epidermis may be ulcerated

contagiosum-like” umbilicated papules. with the release of bony spicules known as perforating
●● DD: Lepromatous nodules, ENL, Von Recklinghausen’s osteoma cutis.
disease/NF-1, molluscum contagiosum, keloids, post ●● DD: Pilomatricoma, osteochondroma, ossified hair

kala-azar dermal leishmaniasis. follicle, cutaneous calcinosis.

Cutaneous calcinosis (calcinosis cutis) Furuncular myiasis

●● It may be iatrogenic, idiopathic, metastatic (increased ●● This is also known as blowfly strike and fly-blown

calcium and phosphate as in renal failure, milk-alkali maggot infestation.

syndrome, calciphylaxis), or dystrophic (calcification ●● Myiasis is a skin disease caused by the larvae (maggots) of

occurs in damaged tissue as in collagen vascular disease, the fly Dermatobia hominis and Cordylobia anthropophaga.
infections, trauma, chronic acne, panniculitis) in origin. ●● It can occur in any age group, and appears more in males.

●● It is more common in young adults; but the occurrence ●● Predisposing factors include poor socioeconomic status,

may vary with type of calcification. poor hygiene, advanced or very young age, psychiatric
●● Acral involvement is common – distal phalanges, tips, illness, alcoholism, diabetes, peripheral vascular
etc.; it may be distributed over other areas as well. disease, and physical disabilities.
●● It is clinically characterized by solitary to multiple ●● Exposed sites most commonly include the fingers,

asymptomatic, flesh-colored to yellow or whitish, hard hands, forearms or face, but it can occur anywhere.
 Nodules: Localized  231

●● It occurs more frequently between ages 20 and 40. There

is a second peak of incidence around the age of 60. It is
less common in the elderly and rare in children.
●● Males are more commonly affected.
●● Lesions mostly occur on the face, scalp, back, buttocks
and extremities.
●● It usually presents as multiple round or oval, infiltrated
reddish-brown plaques. They are larger than 10 mm in
diameter, tend to be thicker and more indurated and
persistent, and are sometimes mammillated.
●● Plaques can adopt an annular appearance by means of
peripheral extension and central clearing, especially
on the forehead and neck. Plaques can simulate lupus
vulgaris, necrobiosis lipoidica, morphea, leprosy,
leishmaniasis, discoid lupus erythematosus, and
granuloma annulare.
●● Plaques can be associated with nodular dermal lesions.
They can be located on the face, scalp, back, buttocks,
and extremities (Figure 9.45).
●● An unusual manifestation of sarcoidosis, known as
Darier-Roussy sarcoidosis, is characterized by insidious,
multiple, firm, asymptomatic to slightly tender, mobile,
round to oval, skin-colored nodules. Commonly, they
Figure 9.44  Secondary osteoma cutis presenting as hard
involve the extremities, but the trunk and face can also
papulonodules on the face. (Courtesy: Dr Ganesh Avhad,
Mumbai, India.)
be affected.

●● The typical lesion presents as an itchy, painful

erythematous papule or a nodule with a central
punctum. A serosanguinous or purulent discharge from
the punctum and end of the larvae is visible.
●● The pain associated is sharp and stabbing, with
sensation of movement.
●● At times it heals, leaving behind slight
hyperpigmentation and scarring.
●● DD: Furunculosis, cellulitis, abscess, insect-bite
reaction, cutaneous larva migrans, leishmaniasis.

Nodular amyloidosis
●● The cause of nodular localized cutaneous amyloidosis

is not known, although the amyloid protein is derived

from a localized infiltrate of plasma cells.
●● It is more common in the fifth to sixth decade and

equally seen in males and females.

●● It clinically presents as firm nodules on the face, scalp,

extremities, trunk, and genitalia; however, acral areas

are preferentially involved. Nodules vary from a few
millimeters to a few centimeters and may coalesce to
form larger plaques. Nodules appear pink to brown
or red. Underlying epidermal atrophy has been
described.
●● Disseminated cases have been described.

●● DD: Colloid milium, cutaneous pseudolymphoma,

leiomyoma.

Sarcoidosis Figure 9.45  Scar sarcoidosis in an elderly lady presenting

●● Sarcoidosis is a multisystem granulomatous disease of as dermal nodule with surface mamillations. (Courtesy:
unknown etiology. Soumyajit Roychoudhury, Berhampore, India.)
232  Nodules: Localized

Figure 9.46  Red-brown nodule of eccrine angioma-

tous hamartoma presenting with pain and hyperhidro-
sis. Lesional hypertrichosis is appreciable. (Courtesy:
Soumyajit Roychoudhury, Berhampore, India.)

Figure 9.47  Shiny, confluent, smooth-surfaced, pearly

papules and nodules of colloid milia. (Courtesy: Dr. Piyush
Kumar, Katihar, India.)
Figure 9.48  Superficial acral fibromyxoma presenting as
slow-growing, smooth-surfaced, shiny nodule in the periun-
●● DD: Deep mycoses, cutaneous metastases of visceral gual area. (Courtesy: Dr. Tanumay Raychaudhury, Skin and
neoplasms and melanoma, epidermoid cysts, lipomas, Cancer Foundation, The University of Sydney, Australia.)
rheumatoid nodules, and erythema induratum.
Additional images – Figures 9.46, 9.47, and 9.48 7. Sinno H, Lacroix JP, Lee J, et al. Diagnosis and management of
eosinophilic cellulitis (Wells’ syndrome): A case series and literature
REFERENCES review. Can J Plast Surg 2012;20(2):91–97.
8. Sharma A, Agrawal S, Dhurat R, Shukla D, Vishwanath T. An unusual
1. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: case of facial steatocystoma multiplex: A clinicopathologic and
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. dermoscopic report. Dermatopathology (Basel) 2018;5(2):58–63.
Chapter 15. Subcutaneous Diseases. P. 253–268. 9. Kumar P, Das A, Savant SS, Mandal RK, Hassan S. Gout nodu-
2. Lipsker D. Clinical Examination and Differential Diagnosis of Skin losis: Report of a rare case and brief review. Dermatol Online J
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 4. 2015;21(1):13030/qt7x98t2jt.
Palpable and Solid Lesions. P. 19–28. 10. Pujani M, Choudhury M, Garg T, Madan NK. Nevus lipomatosus
3. Higgins JC, Maher MH, Douglas MS. Diagnosing common benign superficialis: A rare cutaneous hamartoma. Indian Dermatol Online
skin tumors.Am Fam Physician 2015;92(7):601–607. J 2014;5:109–110.
4. Nguyen T, Zuniga R. Skin conditions: Benign nodular skin lesions. 11. Das A, Kumar P. Dermatofibrosarcoma protuberans. J Pak Assoc
FP Essent 2013;407:24–30. Dermatol 2016;26(2):166–170.
5. Cohen BA. Pediatric Dermatology. 4th ed. Oxford: Saunders 12. García-Patos V. Rheumatoid nodule. Semin Cutan Med Surg
Elsevier; 2013. Chapter 5. Nodules and Tumors. P. 126–147. 2007;26(2):100–107.
6. Mahajan VK. Sporotrichosis: An overview and therapeutic options. 13. Gupta SK. Histoid leprosy: Review of the literature. Int J Dermatol
Dermatol Res Pract 2014;2014:272376. 2015;54(11):1283–1288.
 E7
Nodules: Generalized

SWETALINA PRADHAN, KANANBALA SAHU

ABSTRACT
Generalized or multiple nodules are the primary lesions in various skin conditions. The characteristic morphology, distribu-
tion, and duration of these lesions help in diagnosing a variety of diseases such as infective and neoplastic conditions. This
chapter will provide a basic idea in diagnosing skin diseases that present with multiple/generalized nodules.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-18 233

 E8
Tumors

PC DAS

ABSTRACT
The term tumor refers to solid elevated skin lesions of more than two centimeters diameter. Papules, nodules, and tumors are
skin lesion morphologies usually considered together because they belong to the same type, the difference being that of size
only. The same dermatosis may give rise to all three morphologies in the same patient at a particular time in the course of
disease progression. In this chapter, the word “tumor” is used in reference to skin lesion morphology rather than neoplasia,
which it is often used to mean.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

234 DOI: 10.1201/9781351054225-19

 10
Vesicobullous lesions: Localized

NIHARIKA RANJAN LAL

INTRODUCTION ●● There are four types: EB simplex, junctional, dystrophic,

Kindler syndrome.
Vesicles and/or bullae may be the primary morphologi- ●● Electron microscopy, immunofluorescence mapping
cal manifestation of many skin disorders of localized or help in diagnosis.
systemic pathology. These conditions traditionally come ●● Acral blisters heal without scarring; there is mild or
under the purview of vesiculobullous disorders. Approach no mucosal involvement seen in localized EB simplex
to vesicobullous disorders may be discussed in more than (Figure 10.3a,b).
one ways- etiology wise (genetic, autoimmune, infective ●● There are herpetiform blisters and mucosal and
etc.), presenting age groups (adults or children), course nail involvement in EBS-DM (Dowling-Meara
of the disease (short duration or recurrent and chronic), type).
etc. In this book, author attempts to discuss vesicobul- ●● In junctional EB, Herlitz type, there is non-healing
lous diseases based on the extent of involvement. In this granulation tissue on areas of non-healing and
chapter, blistering diseases expressing on limited skin area dystrophic teeth and nails.
of the body are considered. It is important to remember ●● Blisters/erosion with abdominal distension
that systemic pathology may present on localized area of and non-bilious vomiting/and or ureteral and
the skin initially, so one should keep them in mind when renal abnormalities occur with JEB with pyloric
dealing with localized vesicobullous conditions. The clini- atresia.
cal approach to diagnose vesicobullous conditions usu- ●● Dystrophic EB is the most severe form and may result in
ally involving the limited area of skin has been depicted pseudosyndactyly (thin membranous scarring joining
in Figure 10.1 and the salient features of conditions are two adjacent digits, also called mitten hand deformity)
described below.1–4 (Figure 10.3c,d).
●● Lesions in EB heal with varying scarring, milia, and
Pompholyx/dyshidrosiform eczema nail loss, most noticeable in dystrophic EB.
●● The cause of this condition is unknown; it is ●● DD: Epidermolysis bullosa acquisita (manifests in
usually associated with atopy, contact allergy, adulthood).
psychological stress, hot climate, and palmoplantar
hyperhidrosis.
●● Clinical features include deep-seated vesicles (sago-like) Erythema multiforme
●● Infections (Herpes simplex virus, Mycoplasma) and
on the lateral aspect of fingers (Figure 10.2a,b) and on
palms and soles (Figure 10.2c). drugs are the most important causes.
●● It is extremely pruritic. ●● Target/iris lesions with three concentric zones

●● DD: Acute palmoplantar dermatitis, id reaction. develop from inside out: a central dusky area covered
with a blister, middle edematous zone, peripheral
Epidermolysis bullosa (EB) erythematous (Figure 10.4a–c).
●● This is an inherited mechanobullous disorder. ●● Lesions heal over weeks with hyperpigmentation.

●● The cause is mutations of genes coding for components ●● Mucosal blisters easily rupture into erosions.

of the basement membrane zone. ●● Lesions are usually symmetrical and acral.

●● There is skin fragility and blister formation on minor ●● DD: Fixed drug reaction; secondary syphilis; hand, foot,

trauma/friction. and mouth disease.

DOI: 10.1201/9781351054225-20 235

236  Vesicobullous lesions: Localized

Localized
vesicobullous
Diseases

Clear/turbid fluid Hemorrhagic fluid

Bullous cellulitis
Localized Linear/Grouped Erysipelas
Ecthyma
gangrenosum
Erysipeloid
Orf
Grouped Cutaneous anthrax
umbilicated Vibrio vulnificus
Hands & feet Legs Others vesicles: Herpes infection
simplex
Dermatomal:
Herpes zoster
Blaschkoid:
Sides of fingers: Children – Bullous Incontinentia
Pompholyx – On – Noninflammatory impetigo pigmenti
friction or minor tense blisters, – Exposed area:
trauma: Localized EB Drug history,
diabetes patient: Phytophoto
– Targetoid recurrence at same
Bullous dermatitis,
appearance: EM – site– FDE – At
diabeticorum Paederus
Oval blisters with exposed part:
Underlying purpuric dermatitis
red halo: HFMD Bullous arthropod
lesions: Bullous LCV
Webspace bite
– At pressure sites in
predilection: Scabies comatose patients: Trauma prone sites –
Inner aspect of foot: Coma blisters – friction blister
Bullous tinea pedis Underlying edema: Itching/burning –
Single, infant – Edema blisters ACD, ICD
Sucking blister

Figure 10.1  Clinical approach to localized vesicobullous diseases (LCV: leukocytoclastic vasculitis, FDE: fixed drug
eruption, ACD: allergic contact dermatitis, ICD: irritant contact dermatitis).

Figure 10.2  (a) Deep-seated vesicles on the lateral aspect of fingers in pompholyx. (b) Pompholyx over palm. (Continued)
 Vesicobullous lesions: Localized  237

Hand, foot, and mouth disease

●● With this condition, blisters occur on the hands and

feet, and sometimes buttocks (Figure 10.5a–c).

●● Typically, blisters are oval shaped and have an

erythematous halo (Figure 10.5d).

●● Sometimes, they can be more widespread.

●● They may have oral aphthae-like ulcers.

●● It is associated with a low-grade fever, sore throat, and

loss of appetite.
●● Usually occurs in children <5 years of age.

●● DD: Acropustulosis of infancy, scabies.

Bullous scabies
●● Bulla in scabies is rare.

●● They may form because of secondary bacterial infection

or autoantibody-mediated or id reaction.

Figure 10.2 (Continued)  (c) Pompholyx affecting the palm

and foot. (b,c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 10.3  (a) Flaccid blisters and erosions on the back in epidermolysis bullosa simplex. (b) Blister on the sole in epi-
dermolysis bullosa simplex. (c) Blister and scars on the dorsa of both hands in dystrophic epidermolysis bullosa.
(d) Dystrophic epidermolysis bulla resulting in atrophic scarring, pigmentary loss, and loss of nails. Crusted erosions are
noted over digits. (a,b,d – Courtesy: Dr. Piyush Kumar, Katihar, India; c – Courtesy: Dr. Vishal Gupta, AIIMS, New Delhi.)
238  Vesicobullous lesions: Localized

Figure 10.4  (a) Erythema multiforme with three

concentric zones visible. (b) Erythema multiforme
with target lesions on upper extremity. (c) Erythema
multiforme lesions on palms and soles. (a – Courtesy:
Dr. Vishal Gupta, AIIMS, New Delhi, India; b –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 10.5  (a) Oval vesicle surrounded by erythema on

the sole in hand, foot, and mouth disease. (b) Hand, foot,
and mouth disease with blisters on knees. (Continued)
 Vesicobullous lesions: Localized  239

Figure 10.6  A patient with scabies having vesicles and

excoriations on the dorsum of the foot. (Courtesy:
Dr. Vishal Gupta, AIIMS, New Delhi, India.)

●● Clinical features include painful/pruritic vesicobullous

lesions on the instep and adjacent plantar surfaces of the
feet (Figure 10.7a,b).
●● It may be associated with “id reaction.”
●● DD: Pompholyx, allergic contact dermatitis, bullous
impetigo.

Figure 10.5 (Continued)  (c) Vesicles on buttocks in hand,

foot, and mouth disease. (d) Characteristic oblong vesicle
on sole in hand, foot, and mouth disease. (a – Courtesy:
Dr. Vishal Gupta, AIIMS, New Delhi, India.)

●● Tense/flaccid bullae appear on a scabies-prone site with/

without classic lesion of scabies (Figure 10.6).
●● They are associated with excoriated papules elsewhere
(circle of Hebra) and may burrow.
●● DD: Bullous pemphigoid (more on extremities, no
response to anti scabietic treatment).
Vesicobullous tinea pedis
Figure 10.7  (a) Plantar surface showing bullous tinea with
●● Vesicobullous tinea pedis is a dermatophyte infection of
deroofed lesion and moist floor. (b) Same patient with
the soles of the feet and interdigital spaces. interdigital tinea lesion. (a,b – Courtesy: Dr. Piyush Kumar,
●● The most common cause is Trichophyton rubrum. Katihar, India.)
240  Vesicobullous lesions: Localized

Sucking blister
●● A sucking blister is caused by intense suction by the

intrauterine fetus.
●● It consists of flaccid, serous, blisters of 5–20 mm

surrounded by normal skin (Figure 10.8).

●● The location is the radial border of the forearm, wrist,

hand, dorsal part of the thumb and forefinger.

●● It is diagnosed as a congenital blister in typical site with

good general condition.

●● DD: Blistering distal dactylitis.

Diabetic bulla/bullous diabeticorum

●● The cause is spontaneous, non-inflammatory blistering

in a diabetes setting.
●● Clinical features include asymptomatic, large, asymmetrical

tense bulla, usually on acral sites (Figure 10.9a,b).

●● Signs of inflammation are typically absent.

●● DD: Friction bulla, bullous fixed drug eruption, bullous

pemphigoid.

Figure 10.9  (a) Non-inflammatory bulla on the leg in diabetic

patient. (b) Large diabetic bulla. (a – Courtesy: Dr. Anil Patki,
Pune, India; b – Courtesy: Dr. Ganesh Avhad, Mumbai, India.)

Bullous leukocytoclastic vasculitis

●● This presents with blisters overlying purpuric macules

and papules and with necrotic or hemorrhagic blisters

(Figure 10.10).
●● Bulla formation occurs due to necrosis of the skin

overlying areas of small vessel vasculitis.

●● Common locations are the lower extremities.

●● Bullae appear in crops and may be associated with

constitutional features such as fever, malaise, and joint pain.

●● DD: Erythema multiforme, septic emboli.

Coma blister
●● Coma blisters are self-limiting blisters occurring in the

setting of loss of consciousness (within 48–72 hours).

●● Risk factors include neurological diseases,

immobilization, metabolic conditions, substance

poisoning, and drug overdoses primarily involving
barbiturates, opiates, benzodiazepine, alcohol, and
other neuropsychiatric medications.
●● Blisters develop as a result of tissue hypoxia, ischemia,

and local pressure effects.

●● Clinical features include tense blisters on erythematous

base, usually on pressure-dependent sites such as the

fingers, forearms, malleoli, heels, buttocks, medial
aspect of the knees, and other bony prominences.
●● DD: Bullous cellulitis, friction blister.

Blistering distal dactylitis

Figure 10.8  Oval flaccid, ducking blister. (Courtesy: ●● Blistering distal dactylitis is a localized infection of the

Dr. Tanumay Raychaudhury, Sydney, Australia.) anterior pad of the distal phalanx of the digits.
 Vesicobullous lesions: Localized  241

Bullous impetigo
●● This condition consists of flaccid transparent or

pus-filled bullae, usually in neonates or children

(Figure 10.11a,b).
●● It is not associated with systemic symptoms.

●● Common sites are the face, trunk, perineum, and

extremities.
●● Sometimes, it can have an annular arrangement of

blisters with a central honey-colored crust (impetigo

circinata) (Figure 10.11c,d); it can resemble chronic
bullous disease of childhood (periorificial location) and
vesicular/pustular tinea infection.
●● DD: Neonatal candidiasis, erythema toxicum

neonatorum, miliaria pustulosa.

Figure 10.10  Necrotic blisters over purpuric papules in

leukocytoclastic vasculitis. (Courtesy: Dr. Vishal Gupta,
AIIMS, New Delhi, India.)

●● Less common locations of infection include the proximal

or lateral nail folds, palmar or dorsal hand, toes, and feet.
●● Children are most commonly affected, but cases in
adults are occasionally reported as well.
●● The most common etiologic pathogen is group A
beta-hemolytic Streptococci and, rarely, Staphylococcus
aureus. Multiple bullae tend to occur more commonly
with S. aureus infection.
●● Autoinoculation of the digits from nose picking is
proposed to play a role in causing blistering distal
dactylitis.
●● They present as tense, tender, oval bullae that are filled
Figure 10.11  (a) Bullous impetigo – the lesion starts as a
with thin, seropurulent fluid. These bullae eventually
vesicle over an erythematous skin (1) and enlarges to form
progress to bullae with central erosions or simple flaccid blisters with pus in their bottom part (2). Soon blis-
erosions with adherent layers of skin. ters rupture to leave erosions covered with crust (3) and
●● DD: Herpetic whitlow, epidermolysis bullosa, bullous ultimately heal with epithelization (4). (b) Bullous impetigo
impetigo, friction blisters. over neck. (Continued)
242  Vesicobullous lesions: Localized

Figure 10.11 (Continued)  (c) Honey-colored annular crust

in impetigo circinata. (d) Annular lesions of impetigo cir-
cinata. (a,b,d – Courtesy: Dr. Piyush Kumar, Katihar, India;
c – Courtesy: Dr. Vishal Gupta, AIIMS, New Delhi, India.)

Bullous fixed drug eruption

●● Bullae occur over the central area of dusky

erythematous or violaceous round-to-oval macules and

Figure 10.12  (a) Bullous fixed drug reaction.
plaques (Figure 10.12a–c). Hyperpigmented, oval patch surrounded by erythematous
●● It is associated with pain and a burning sensation.
border with vesiculation. (b) Bulla over round, erythematous
●● Common locations are lips, genitals, and acral sites. area of skin. (c) Bullous fixed drug eruption over the dorsum
●● They heal with significant post-inflammatory of the foot. (a – Courtesy: Dr. Vishal Gupta, AIIMS, New
hyperpigmentation. Delhi, India; b,c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Vesicobullous lesions: Localized  243

Figure 10.13  (a) Tense blisters on the dorsa of both feet

due to insect bites. (b) Tense blisters of insect bite reaction
in a child. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Recurrence at the same site with subsequent implicated

drug exposure is typical.
Bullous insect-bite reaction
●● Bullous lesions in reaction to insect bites are common

with mosquitoes and bedbugs (Figure 10.13a,b).

●● Skeeter syndrome is a mosquito saliva-induced large local

inflammatory reaction clinically resembling cellulitis.

●● Bedbug bites are known as cimicosis.

●● Common sites are the arms, shoulders, and legs.

Friction blister
●● Friction blisters result from pressure/shear forces, causing

delamination at the level of the stratum spinosum.

●● Risk factors include outdoor pursuits (hiking, marathon

races).
●● The most common sites are the foot and areas with

thick stratum corneum. Figure 10.14  (a) Friction blisters on the pressure areas
●● Clinical features include distinct bulla on trauma site of the foot in leprosy. (b) Thermal damage resulting in
(Figure 10.14a–c). blister on anesthetic hand in borderline tuberculoid
●● DD: Acquired/hereditary epidermolysis bullosa (other
leprosy. (c) Friction blisters on hand and feet in leprosy.
(a – Courtesy: Dr. Piyush Kumar, Katihar, India.)
trauma-prone areas also involved).
Thermal burns
●● Burn blisters are a common wound finding for health ●● In the inflammatory phase, blisters can occur as a
care providers treating burn patients in burn centers, physiological response to burn damage if the epidermis
emergency rooms, and other medical facilities. is separated from the underlying dermis.
●● They are unique in that they incorporate several zones ●● Deep partial-thickness blisters are thick-walled and
of tissue damage due to differences in heat transfer. contain white skin; sensation is either impaired or intact.
244  Vesicobullous lesions: Localized

●● Superficial partial-thickness blisters contain weeping

skin and are typically thin-walled, with intact sensation
(Figure 10.15a–c).

Allergic contact dermatitis

●● This is delayed hypersensitivity reaction of skin to

contact with foreign substances; on re-exposure,

sensitized T cells initiate an inflammatory cascade.
●● Causative allergens include nickel, fragrances,

neomycin, parabens.
●● Clinical features are erythema, edema, vesicles, oozing,

and intense pruritus (Figure 10.16a–c).

●● It is not sharply limited to area of contact; it may get

generalized on subsequent exposures.

Figure 10.15  (a) Clear fluid containing tense blisters on dusky

erythematous skin. (b) Thermal burn – blister has ruptured to
leave erosion. (c) Oblong tense blister due to thermal injury.
(a,c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Figure 10.16  (a) Allergic contact dermatitis manifest-

ing as erythematous papules and vesicles on hands.
(b) Grouped vesiculopustular lesions in allergic contact
dermatitis. (Continued)
 Vesicobullous lesions: Localized  245

●● DD: Pompholyx, nummular dermatitis, irritant contact

dermatitis.

Acute irritant contact dermatitis (ICD)

●● This is a result of inflammatory and cytotoxic effects

caused by exposure to various irritant stimuli, which

activate the innate immune system.
●● Irritants induce skin barrier disruption and direct

cellular damage.
●● Symptoms include burning, pain, stinging, pruritus,

and soreness.
●● Signs are erythema, edema, oozing/exudation, bullae,

and erosions (Figure 10.17a–c).

Figure 10.16 (Continued)  (c) Severe allergic contact

dermatitis to hair dye manifesting as blisters over eyelids,
facial edema and erythema, and oozing. (a–c – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

Figure 10.17  (a) Flaccid blister and dusky erythematous skin in irritant contact dermatitis to an antiseptic solution contain-
ing chlorhexidine gluconate and cetrimide among others. (b) Patient applied an antiseptic solution (containing chlorhexi-
dine gluconate and cetrimide among others) over abrasions and developed irritant contact dermatitis. (Continued)
246  Vesicobullous lesions: Localized

Figure 10.17 (Continued)  (c) Irritant contact dermatitis

over medial aspect of knee.

●● The severity of the condition and hence, clinical feature

depends on the nature, concentration, duration of
contact with the irritant chemical, pre-existing skin
conditions, mechanical, thermal, or climatic effects.

Herpes simplex
●● This is caused by herpes simplex virus (HSV) types 1

and 2.
●● HSV-1 affects above the waist while HSV-2 causes

infections below the waist; overlap may occur.

●● Infection may be primary or following reactivation.

●● HSV-1 mostly causes orolabial infections; it may infect

other cutaneous sites also (herpetic whitlow, herpes

gladiatorum).
●● Grouped vesico-ulcerative lesions occur the on

erythematous base palate, gingiva, tongue, lips, and

perioral area (Figure 10.18a–c).
●● Pharyngitis, fever, tender cervical lymphadenopathy

may be present.
●● HSV2 lesions are seen on the labia majora, minora,

cervix, vagina, and mons pubis in females and on the

penile shaft in males.
●● Perianal lesions may occur, depending on sexual

practice.
●● Pain, dysuria, fever, tender inguinal lymphadenopathy

may be present.
●● DD: Paederus dermatitis, bullous fixed drug eruption,
Figure 10.18  (a) Herpes labialis. Grouped vesicles on an
aphthous ulcer.
inflammatory base. (b) Multiloculated blisters in herpes
simplex on hand. (c) Herpes simplex with gingivos-­
Herpes zoster (HZ)
stomatitis. Oral blisters rupture rapidly, leaving erosions
●● Herpes zoster is caused by reactivation of a latent
covered with whitish slough. (a – Courtesy: Dr. Vishal
varicella zoster virus infection. Gupta, AIIMS, New Delhi.)
 Vesicobullous lesions: Localized  247

Figure 10.19  (a) Herpes zoster presenting as multiple groups of blisters on an inflammatory base along T3–T4 dermatome.
(b) Herpes zoster along L2–L3 dermatome in an infant. (c) Bullae in herpes zoster. (a – Courtesy: Dr. Vishal Gupta, AIIMS,
New Delhi; c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Prodromal symptoms that herald HZ include pruritus, ●● Stage 2 occurs in about 70% of patients. Eruption of
dysesthesia, and pain along the distribution of the hyperkeratotic verrucous papules and plaques develop
involved dermatome. over the healing blisters.
●● The rash consists of grouped vesicles on a red base in a
unilateral, dermatomal distribution (Figure 10.19a–c).
●● Most commonly affected area is the thoracic dermatome.
●● Herpes zoster ophthalmicus may cause keratitis,
scarring, and vision loss.
●● DD: ICD, herpes simplex.

Incontinentia pigmenti
●● Incontinentia pigmenti (IP), a rare X-linked dominant

genodermatosis, is a multisystem, ectodermal and

mesodermal disorder accompanied by dermatologic,
dental and ocular features. In a minority of cases,
neurologic deficits may occur.
●● Pathogenesis is mutation of the NEMO gene.

●● Stage 1 (vesicular stage) is present at birth or within the

first two weeks in 90% of patients; it is characterized by

a rash of erythematous blisters, often appearing along
the lines of Blaschko (Figure 10.20).
●● Stage 1 is short lived and may be completed in utero.

It is followed by stage 2 (verrucous stage), stage Figure 10.20  Vesicles and crusted erosions along
3 (hyperpigmented stage), and stage 4 (atrophic/ Blaschko’s lines in incontinentia pigmenti. (Courtesy:
hypopigmented stage). Dr. Sweta Rambhia, Mumbai, India.)
248  Vesicobullous lesions: Localized

●● Stage 3 is seen in 98% of patients as pigmentation

(blue-gray or slate to brown) in streaks or whorls.
●● Stage 4 is observed in adolescence and persists into
adulthood.
●● Involvement of appendageal structures may result in
vertex alopecia, ridged, pitted, or dystrophic nails.
●● Dental abnormalities include delayed dentition, partial
anodontia, conical or peg-shaped teeth, or absence of
teeth.
●● Neurologic manifestations include seizures, mental
retardation, developmental delays, spastic paralysis,
ataxia, and motor dysfunction.
●● Ocular abnormalities include strabismus, cataracts,
optic atrophy, retinal dysfunction, uveitis, nystagmus,
and blindness.
●● Occasionally there may be skeletal and structural
anomalies, such as somatic asymmetry, skull
deformities, spina bifida, dwarfism, syndactyly,
extra ribs, primary pulmonary hypertension, and
cardiopulmonary failure.

Lymphangioma circumscriptum
●● Lymphangioma circumscriptum (LC) is a benign

lymphatic malformation characterized by dilation of

lymphatic vessels in the skin and subcutaneous tissue.
These abnormal lymphatic malformations do not
communicate to the normal lymphatics.
●● The exact cause is unknown. It may be congenital or

acquired due to damage of lymphatic vessels from

various etiologies.
●● However, various growth factors, such as vascular

endothelial growth factor-C (VEGF-C) and VEGF-D

and their receptors on the lymphatic endothelial cells,
may have a role in the mechanism.
●● It is commonly found in the chest, mouth, axilla, and

tongue.
●● It is characterized by translucent, pink-red vesicle

bundles with thin membranes, sometimes filled with

blood; the vesicles have clear edges and can be scattered
or in groups; hyperkeratosis may give rise to a warty
appearance (Figure 10.21a,b).
●● DD: Lymphangiectasia, hemangioma, verruca,

molluscum contagiosum, angiokeratoma, and

lymphangioendothelioma.

Paederus dermatitis
●● Paederus dermatitis is an irritant dermatitis caused by

pederin, a toxin produced by the rove beetle (Paederus). Figure 10.21  (a) Lymphangioma circumscriptum present-
●● The hemolymph of the Paederus beetle contains this ing as grouped translucent, pink vesicles on the trunk.
pederin, which is released when crushing the insect (b) Lymphangioma circumscriptum over the gluteal area.
onto the skin by the reflex of brushing away the insect. (a – Courtesy: Dr. Piyush Kumar, Katihar, India.)
●● Symptoms typically begin between 24 and 48 hours

after contact with the insect, the most common being ●● Morphological patterns include linear dermatitis
itching and burning or smarting sensation. (Figure 10.22a), kissing lesions (Figure 10.22b),
●● Lesions evolve through an initial erythematous phase localized pustules (Figure 10.22c), and, rarely, extensive
followed by vesiculation and subsequent crusting and skin involvement with systemic manifestations such as
desquamation. fever, neuralgia, arthralgia.
 Vesicobullous lesions: Localized  249

Figure 10.22  (a) Paederus dermatitis as vesicopustular

lesions on erythematous base, arranged in a linear fashion
on the arm. (b) “Kissing lesions” in paederus dermatitis
– blisters on an erythematous base. (c) Irritant contact
dermatitis with necrotic skin surrounded by erythema. The
epidermal damage is maximum in the center of the lesion.
(b – Courtesy: Dr. PC Das, Katihar, India.)

●● Moderately severe cases with significant vesiculation ●● Streptococcus pyogenes is the most common.
may dry out and exfoliate with hyperpigmentation that ●● Clinical features include acute, spreading, poorly
may last up to a month. Cutaneous necrosis may occur demarcated area of erythema, blister formation, and
occasionally. peau d’orange appearance of skin; fever may be present
●● DD: Herpes zoster, herpes simplex, irritant contact (Figure 10.23a).
dermatitis, blister beetle dermatitis. ●● DD: Erysipelas (presence of sharp border)
(Figure 10.23b), necrotizing fasciitis (rapidly spreading,
Bullous cellulitis very painful), Sweet syndrome.
●● Cellulitis is a bacterial infection of the skin, presenting

with poorly demarcated erythema, edema, warmth, and Erysipelas

tenderness. ●● Erysipelas is a streptococcal infection involving the

●● Portals of entry include toe web-space infections and upper dermis and extending into the superficial
venous ulcers. cutaneous lymphatics.
250  Vesicobullous lesions: Localized

●● The cutaneous finding will show fiery red, indurated,

shiny plaque with a sharp raised border; lower limbs are
involved in 80% of cases (Figure 10.24a–c).
●● DD: Cellulitis, allergic contact dermatitis, erysipeloid.

Ecthyma gangrenosum
●● This is a condition caused by Pseudomonas

aeruginosa.
●● Risk factors include immunosuppression with severe

neutropenia.

Figure 10.23  (a) Swelling, erythema, and erosion in bul-

lous cellulitis. (b) Well-defined, erythematous, edematous
plaque of erysipelas with tense bullae on its surface.
(b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Risk factors include lymphatic obstruction,

immunosuppression, venous insufficiency,
dermatophyte infection.
●● Clinical features include high fever, chills, and Figure 10.24  (a) Well-defined, erythematous, indurated
malaise. lesion with surface vesiculation in erysipelas. (Continued)
 Vesicobullous lesions: Localized  251

Figure 10.24 (Continued)  (b) Well-defined, erythematous, edematous plaque with surface vesiculation in erysip-
elas. (c) Erysipelas with surface vesiculation. (a – Courtesy: Dr. PC Das, Katihar, India; b,c – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

●● Erythematous/purpuric macules appear on the skin. ●● A purpuric macule evolves into a vesicle surrounded by
●● They evolve into hemorrhagic vesicles and blisters and non-pitting edema (Figure 10.25).
rupture to form necrotic ulcers with black eschar over ●● The vesicle ruptures into a hemorrhagic ulcer with black
extremities and anogenital regions. eschar.
●● DD: Cutaneous anthrax. ●● DD: Ecthyma gangrenosum, cellulitis, spider bite.

Erysipeloid Vibrio vulnificus infection

●● Erysipeloid is caused by gram positive bacilli ●● This is caused be gram negative anaerobic bacteria.

Erysiplothrix rhusiopathiae. ●● Risk factors include diabetes and chronic liver

●● It occurs through traumatic inoculation in fishermen/ disease.

meat handlers. ●● There is often a history of exposure to warm sea water

●● Clinically it manifests as a violaceous to erythematous and raw or undercooked seafood consumption.

area of non-suppurative cellulitis. ●● Erythematous macules progress to vesicles and

●● Hemorrhagic vesicles may appear. hemorrhagic bullae.

●● DD: Erysipelas, cellulitis, FDE. ●● Necrotizing fasciitis may ensue.

●● Other features include fever, chills, nausea, vomiting,

Cutaneous anthrax diarrhea, abdominal cramps, and hypotension.
●● This is caused by occupational exposure to gram

positive bacilli Bacillus anthracis. Orf

●● It affects exposed body parts after contact with infected ●● Orf is an infection caused by Parapoxvirus and mainly

animals/their hides or carcasses. affects goats and sheep.

252  Vesicobullous lesions: Localized

Hidrocystoma
●● Also known as cystadenomas, sudoriferous cysts, and

Moll’s gland cysts, hidrocystomas are benign cystic

tumors derived from either the eccrine or apocrine
sweat gland.
●● The difference between eccrine hidrocystoma (EH)

and apocrine hidrocystoma (AH) can be tabulated as

in Table 10.1.

Figure 10.25  Cutaneous anthrax presenting as vesico-

pustular lesion on an erythematous, edematous base.
(Courtesy: Dr. Rajesh Kumar Mandal, North Bengal
Medical College, Darjeeling, India.)

●● Humans get infected by direct inoculation through

cuts or abrasions while handling animals, carcasses, or
contaminated equipment.
●● A lesion appears three to seven days after contact with
an infected animal and evolves slowly over the course of
four to eight weeks.
●● It starts as a firm red painful papule that expands into a
broad, thickened 1- to 3-cm lesion.
●● The center of the lesion is red and is surrounded by a
white, raised ring with erythematous periphery. The
crusting occurs and resolves by three to six weeks, with Figure 10.26  (a) Blister of orf. (b) Vesicopustular lesion of
little to no scarring (Figure 10.26a,b). orf on the finger. (a – Courtesy: Dr. Deverashetti Srinivas,
●● DD: Cutaneous anthrax, pyoderma gangrenosum, and Nizamabad, India; b – Courtesy: Dr. Shahid Hassan,
herpetic whitlow. Purnea, India.)
 Vesicobullous lesions: Localized  253

Table 10.1  Differences between eccrine hidrocystoma (EH) and apocrine hidrocystoma (AH)

Eccrine hidrocystoma Apocrine hidrocystoma

Appearance Skin-colored to bluish vesiculopapular lesions Translucent or semi-transparent, round,
(Figure 10.27a) skin-colored or bluish vesicles, cystic lesions
that contain a watery fluid (Figure 10.27b)
Cause Obstruction of the eccrine sweat gland, which An adenoma of the coil structure of the
causes retention of secretions and the apocrine sweat gland
appearance of a dilated cystic structure
Age Usually middle age Middle age
Sex More in women No sex predilection
Site Periorbital area, sparing lid margin Near lid margin
Other sites: penis, axilla, and perianal regions
Number Robinson type: solitary Usually solitary
Smith and Chernosky type: smaller and multiple
Effect of temperature Become larger on sun exposure No change

Figure 10.27  (a) Tiny, translucent lesions of eccrine hidrocystoma. (b) Apocrine hidrocystoma presenting as solitary trans-
lucent cyst over lateral canthus. (a – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. Abhishek De, Calcutta
National Medical College and Hospital, Kolkata, India.)

●● Multiple eyelid AHs are associated with Schöpf-Schulz- 2. Hull C, Zone JJ. Approach to the patient with cutaneous blisters.
In, Stratman E, Ofori AO, editors. https://www.uptodate.com/
Passarge and Goltz-Gorlin syndrome.
contents/approach-to-the-patient-with-cutaneous-blisters
●● DD: Syringoma, trichoepithelioma, and multiple basal 3. Wick MR. Bullous, pseudobullous, & pustular dermatoses. Semin
cell carcinomas. Diagn Pathol 2017;34(3):250–260.
4. Yeh SW, Ahmed B, Sami N, Razzaque Ahmed A. Blistering disor-
ders: diagnosis and treatment. Dermatol Ther 2003;16(3):214–223.
REFERENCES
1. Hill SF, Murrell DF. Differential diagnosis of vesiculobullous lesions.
In, Hoeger P, Kinsler V, Yan A, editors. Harper’s Textbook of
Pediatric Dermatology. 4th edition. New Jersey: Wiley-Blackwell,
2020. P. 859–867.
 11
Vesicobullous lesions: Generalized

RITI BHATIA AND VISHAL GUPTA

INTRODUCTION Grover’s disease

●● This is also known as transient acantholytic
Vesiculobullous diseases are a distinct group of mucocuta- dermatosis.
neous disorders characterized by the formation of vesicles ●● It usually occurs in middle-aged and elderly people.
or bullae. A wide variety of conditions may present with ●● It is characterized by discrete pruritic, papular, or
generalized vesicles and bullae and include viral and bac- papulo-vesicular eruptions favoring the trunk.
terial diseases, autoimmune diseases, and genetic diseases ●● Eruptions may persist in some patients and the term
among others. A detailed history and clinical examination “papular acantholytic dermatosis” is a preferred term to
in most of the cases help us to arrive at a list of clinical dif- describe this entity.
ferentials. However, a clinician must closely examine the ●● DD: Hailey-Hailey disease, Darier disease.
nature of vesicles/bullae (tense, fragile) along with distri-
bution and arrangement (annular, discrete). One should Pemphigus vulgaris
keep in mind that vesicles and bullae, especially flaccid
●● Pemphigus vulgaris is an autoimmune blistering disease
ones, may get ruptured easily, leaving erosions and crusts
only, and vesicobullous lesions may not be observed dur- due to formation of autoantibodies against desmosomal
ing clinical examination. Examination of surrounding skin proteins, desmoglein 1 and 3.
●● Clinical features include flaccid blisters that easily
(urticarial plaques in pemphigoid gestationis and bullous
pemphigoid; lichenoid papules in bullous lichen planus rupture to form erosions (Figure 11.3a,b), initially
and lichen planus pemphigoides) and demonstration of beginning from the head and neck and gradually
clinical signs like Nikolsky sign, Asboe-Hansen sign, etc., extending to the trunk and flexural area; generalized
provide important clues to the diagnosis. However, clinical involvement may occur in severe cases. The erosions
diagnosis has a somewhat limited role in the diagnosis of
autoimmune vesicobullous diseases; biopsy and immuno-
Table 11.1  Vesicobullous lesions in neonates*
fluorescence studies are conclusive. This chapter discusses
the clinical approach to generalized vesicobullous diseases Systemic
(Figure 11.1, Table 11.1), followed by discussion on salient features Diseases
features of these conditions.1–7
Absent • No inflammation – miliaria crystallina
Miliaria crystallina • Generalized erythema – epidermolytic
●● Miliaria crystallina is a self-limiting eccrine gland ichthyosis
disorder due to blockage of sweat glands, presenting as • Trauma prone sites – epidermolysis bullosa
fluid-filled vesicles that easily break. • Blaschkoid distribution – incontinentia
●● It is seen mostly in regions with high degrees of heat pigmenti
and humidity as in tropical regions, and is characterized • Colored urine – congenital porphyria
by high-grade fever, hypernatremia, and neutropenia. Present • Diffuse erythema, Nikolsky sign positive
●● Tiny 1- to 2-mm fragile clear vesicles present on a non- – staphylococcal scalded skin syndrome
inflammatory base (Figure 11.2a,b). • Lesional erythema – varicella
●● Common sites are the face, neck, flexures. • Hemorrhagic bullae, petechiae, scaly
●● It is usually seen in neonates but can also occur in plaques – Congenital syphilis
adults with fever. *Entities having prominent vesiculo-pustular lesions are not
●● DD: Erythema toxicum neonatorum, bullous impetigo. included here.

254 DOI: 10.1201/9781351054225-21

 Vesicobullous lesions: Generalized  255

Generalized vesiculobullous
diseases

Widespread Specific distribution

Systemic features Systemic Seborrheic area predilection Flexures Photodistributed

usually absent features
present

Pemphigus foliaceous Hailey Hailey disease – Sclerodermoid,

Flaccid Tense Langerhans cell histiocytosis Langerhans cell histiocytosis hypertrichosis, milia: PCT
blisters blisters – Infant, fever, – Pseudoporphyria
flaccid blisters: – Photosensitizing drug
SSSS /contact allergen:
– Children, Phototoxic reaction
– Fragile tiny clear – Urticarial plaques: hypotension: – Bullous LE
vesicles: miliaria bullous pemphigoid, bullous
crystallina, Grover’s pemphigoid mastocytosis
disease gestationis – Fever,
– Erosions, mucous – Trauma-prone sites, polymorphic
membrane milia formation: EBA eruption:
involvement: – Annular varicella, Kaposi
pemphigus configuration of varicelliform
vulgaris, blisters: LAD eruption
paraneoplastic – Itchy grouped – Necrotic blisters,
pemphigus, IgA papulovesicles on severe mucosal
pemphigus extensors: DH involvement:
– Lichenoid plaques – SJS/TEN
bullous lichen
planus, lichen
planus
pemphigoides

Figure 11.1  Clinical approach to generalized blisters in children and adults. (EBA – Epidermolysis bullosa acquisita, LAD –
linear IgA dermatosis, DH – dermatitis herpetiformis, SJS – Stevens Johnson syndrome, TEN – toxic epidermal necrolysis,
SSSS – Staphylococcal scalded skin syndrome, PCT – porphyria cutanea tarda).

show a tendency to spread much beyond the size of

bullae.
●● Bullae may contain a clear or turbid fluid; sometimes
turbid fluid may settle in the dependent portion of
blister (“hypopyon sign”) (Figure 11.3c).
●● It shows a positive Nikolsky sign (Figure 11.3d).
●● Mucosal involvement is common: painful oral
erosions on the buccal mucosa, tongue, and gingiva;

Figure 11.2  (a) Tiny, clear vesicles in miliaria crystallina. (b) Close-up of lesions of miliaria crystallina. (Courtesy: Dr. Ganesh
Avhad, Mumbai, India.)
256  Vesicobullous lesions: Generalized

Figure 11.3  (a) Pemphigus vulgaris with extensive erosions on the back. (b) Flaccid blisters, cornflake like scales and ero-
sions in pemphigus vulgaris. (c) Hypopyon sign in pemphigus vulgaris. (d) Pemphigus vulgaris. Positive Nikolsky sign (solid
arrow) has been demonstrated. (e) Oral and perioral erosions and crusting in pemphigus vulgaris. (Continued)
 Vesicobullous lesions: Generalized  257

leukemia, Castleman disease, Waldenstrom

macroglobulinemia, and thymoma – with or without
myasthenia gravis).
●● Clinically, PNP may manifest in following forms:
a. Pemphigus-like: superficial vesicles, flaccid vesi-
cles, erosions, crust, and erythema (Figure 11.4a).
b. Bullous pemphigoid-like: scaly erythematous
papules, which may or may not be associated
with stretched vesicles.

Figure 11.3 (Continued)  (f) Crusted erosions on lip

in pemphigus vulgaris. (g) Erosions on the hard pal-
ate. (a,c,f,g – Courtesy: Dr. Piyush Kumar, Katihar, India
b – Courtesy: Dr. Hiral Shah, Baroda Medical College,
Vadodara, India.)

genital erosions; oral ulcers with irregular margins

(Figure 11.3e–g).
●● The disease is progressive if untreated; death may occur
from sepsis.
●● DD: Pemphigus foliaceus (scaly crusted erosions
seen more commonly than intact blisters, lacks
mucosal involvement), linear IgA pemphigus (annular
arrangement), bullous pemphigoid (tense blisters).

Paraneoplastic pemphigus
●● Paraneoplastic pemphigus (PNP) is a rare and often

fatal autoimmune blistering disease accompanied by

both benign and malignant neoplasms. Skin lesions
are thought to be caused by an autoimmune response
generated by antibodies to tumor antigens that
cross-react with epithelial antigens; autoantibodies
are directed against both desmosomal and
hemidesmosomal antigens. Figure 11.4  (a) Pemphigus vulgaris-like cutaneous and
●● The most frequently reported associated
mucosal erosions in paraneoplastic pemphigus.
malignancies are lymphomatoid and hematologic (b) Hemorrhagic blisters and erythema multiforme-like lesions
(e.g., B-cell lymphoma, chronic lymphocytic on the palm in paraneoplastic pemphigus. (Continued)
258  Vesicobullous lesions: Generalized

c. Erythema multiforme-like: polymorphic alter-

ations, mainly erythematous peeling pellets
with erosions and sometimes even with hard-
to-heal ulcerations (Figure 11.4b,c).
d. Graft-versus-host disease: disseminated dusky-
red scaly papules.
e. Lichen planus-like: small flat scaly papules and
intense mucous membrane involvement.
●● PNP may involve not only the buccal mucosa but also
the mucous membranes of the esophagus, stomach,
duodenum, and intestines and also the pulmonary
epithelium.
●● DD: Pemphigus vulgaris, mucous membrane
pemphigoid, erythema multiforme, Stevens-Johnson
syndrome, lichen planus, graft-versus-host disease, and
herpes simplex virus infection.

IgA Pemphigus
●● IgA pemphigus is common in middle-aged and elderly

people.
●● There are two types: subcorneal pustular dermatosis

(SCPD)-like and intraepidermal neutrophilic type.

●● Patients with both types present with flaccid vesiculo-

pustules on erythematous or normal skin (Figure 11.5a).

●● Pustules tend to coalesce to form an annular or

circinate pattern, with crusts in the central area

Figure 11.4 (Continued)  (c) Erythema multiforme-like

lesions in paraneoplastic pemphigus. (a,b – Courtesy:
Dr. Piyush Kumar, Katihar, India; c – Courtesy: Dr. Rajesh
Kumar Mandal, North Bengal Medical College and
Hospital, India.)
Figure 11.5  (a) Vesicles arranged in circinate pattern in
IgA pemphigus. (Continued)
 Vesicobullous lesions: Generalized  259

●● It is caused by autoantibodies against BPAg1 and 2.

●● Clinical features include the following
●● Non-bullous phase: Mild to severe pruritus
alone or associated with urticarial plaques
(Figure 11.6a,b), eczematous papules/plaques
(Figure 11.6c).
●● Bullous phase: Blisters are tense with straw-
colored fluid (Figure 11.6d,e); they can also
be hemorrhagic (Figure 11.6f,g); the Nikolsky
sign is negative. Bullae persist for a longer time
compared to those in pemphigus vulgaris, and
subsequent erosions do not extend much beyond
the size of bullae.
●● Eruptions typically heal with hypopigmentation and
follicular repigmentation (Figure 11.6h), usually with
no scarring or milia formation.
Figure 11.5 (Continued)  (b) IgA pemphigus. (a,b – Courtesy: ●● Common locations include the extremities (lower and
Dr. Niharika Ranjan Lal. ESIPGIMSR, Kolkata, India.) upper) and the trunk.
●● It may also have mucosal involvement.
(Figure 11.5b). It more frequently involves the trunk, ●● DD: Other subepidermal immunobullous disorders
intertriginous areas, and flexor aspects of the limbs. such as epidermolysis bullosa acquisita, linear IgA
●● Mucous membranes are almost never affected. bullous dermatosis, anti-p200 pemphigoid.
●● DD: Pemphigus vulgaris, bullous pemphigoid,
generalized pustular psoriasis (in case of SCPD). Pemphigoid gestationis
●● Pemphigoid gestationis or gestational pemphigoid is a
Bullous pemphigoid rare pregnancy-associated autoimmune skin disorder
●● Bullous pemphigoid is an autoimmune blistering that is immunologically and clinically similar to the
disease characterized by blisters, usually observed in pemphigoid group of autoimmune blistering skin
elderly patients. disorders.

Figure 11.6  (a) Urticarial plaque with few vesicles in bullous pemphigoid. (b) Urticarial lesion with tense bulla in bullous
pemphigoid in an elderly. (c) Eczematous plaque with tense bulla in bullous pemphigoid. (d) Tense bullae in bullous
pemphigoid. (Continued)
260  Vesicobullous lesions: Generalized

Figure 11.6 (Continued)  (e) Tense blisters in bullous

pemphigoid. (f) Hemorrhagic bulla in bullous pemphi-
goid. (g) Tense blisters with hemorrhagic crust and
erosion. Note the erosions correspond to the size of
blisters. (h) Lesions of bullous pemphigoid healing
with follicular repigmentation. (a,c,d–h – Courtesy:
Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. PC
Das, Katihar, India.)

●● It mainly affects multiparous women in their second or ●● PG initially presents with intense pruritus, progressing
third trimester of pregnancy. to polymorphic skin lesions, which are usually
●● The pathogenesis is similar to that of bullous urticarial papules and annular plaques.
pemphigoid, caused by autoantibodies directed against ●● It is followed by vesicles and finally large tense bullae on
two hemidesmosomal proteins, BP180 and BP230, an erythematous background.
within the dermoepidermal junction, resulting in the ●● It typically develops on the abdomen, characteristically
formation of bullae and skin erosions. involving the umbilical region.
 Vesicobullous lesions: Generalized  261

●● In 90% of the cases, it later spreads to the rest of the

abdomen, involving the thighs palms, and soles.
●● The mucous membranes and face are usually spared.
●● DD: Urticaria, scabies, contact dermatitis, atopic
eruption of pregnancy, polymorphic eruption of
pregnancy, intrahepatic cholestasis of pregnancy.
Epidermolysis bullosa acquisita (EBA)
●● This is an autoimmune, acquired subepidermal

blistering disease.
●● It is caused by autoantibodies to collagen VII.

●● Clinical features include the following

●● Non-inflammatory type: skin fragility and blistering

over trauma-prone areas, healing with scarring,
milia formation, dystrophic nails (Figure 11.7a–c).
●● Inflammatory type: widespread, tense vesicles and
bullae not localized to trauma-prone sites and generally
healing with minimal scarring and milia formation. It
resembles other chronic bullous disorders.
●● DD: Bullous pemphigoid (generalized blister, preceded

with itching,), dystrophic epidermolysis bullosa,

cicatricial pemphigoid.
Linear IgA dermatosis/chronic bullous disease of
childhood
●● Linear IgA dermatosis is a rare autoimmune blistering

disease with two clinical forms: childhood type (chronic

bullous disease of childhood) and adult type

Figure 11.7   (a) Tense blisters, scarring, milia, and anonychia in epidermolysis bullosa acquisita. (b) Tense blisters and scar-
ring of the skin over knee in EBA. (c) Extensive scarring of the skin over hands and anonychia in EBA. (Continued)
262  Vesicobullous lesions: Generalized

Figure 11.7 (Continued)  (d) Tense blisters in chronic bullous

dermatosis of childhood, central blister showing string-of-
pearls appearance. (e) Tense blisters in chronic bullous dis-
ease of childhood. (f) Chronic bullous disease of childhood.
(g) Chronic bullous disease of childhood. Healing stage.
(a–f – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● It is caused by formation of autoantibodies against

basement membrane zone (BMZ) proteins.
●● It presents as pruritic, grouped, annular papules,
vesicles, and bullae in an atypical “string of pearls” or
“cluster of jewels” pattern (Figure 11.7d–g).
●● There is symmetrical distribution on extremities.
●● Mucosa involvement is often seen, with oral and ocular
mucosa most commonly affected.
●● DD: Bullous pemphigoid, dermatitis herpetiformis,
epidermolysis bullosa acquisita.
 Vesicobullous lesions: Generalized  263

Dermatitis herpetiformis ●● Diagnosis is neutrophilic microabscess and IgA deposits

●● Dermatitis herpetiformis presents with intensely on dermal papillary tips.
pruritic and grouped vesicobullous lesions and ●● DD: Scabies, id reaction.
urticarial plaques (Figure 11.8a).
●● It is commonly found on extensor surfaces (elbows,
Bullous lichen planus
●● Bullous lichen planus (BLP) is an uncommon variant of
knees, buttocks, shoulders, sacral areas) (Figure 11.8b).
●● Gluten sensitive enteropathy is seen in most of the lichen planus (LP) and results from severe inflammation
patients. at the dermoepidermal junction (DEJ), causing a split at
the DEJ.
●● It is clinically characterized by vesicular and/or bullous

lesions developing on pre-existing LP lesions or on

perilesional skin (Figure 11.9).
●● There are two variants of BLP: familial and non-familial.

●● Familial variant is more common, occurs at an earlier

age with longer duration of disease, more extensive

eruptions, and increased tendency to involve nails as
compared to non-familial variants. Pathogenesis of
familial variants may be attributable to genetic factors.
●● Lower extremities are most commonly affected. Nail

involvement is characterized by hemorrhagic crusty

lesions resulting in exposure of nail bed and nail atrophy
●● DD: Lichen planus pemphigoides.

Lichen planus pemphigoides

●● Lichen planus pemphigoides (LPP) is a rare disease

usually affecting adults; patients of lichen planus

develop autoantibodies against BPAg2 and BPAg1.
●● The cause is mostly idiopathic, drugs: ACE inhibitors,

PUVA therapy, anti-tuberculosis medications, hepatitis B.

●● In LPP, two discrete primary skin lesions occur:

lichenoid papules/plaques and tense blisters.

Figure 11.8  (a) Excoriated papulovesicles symmetrically

distributed on elbow extensors in dermatitis herpetifor-
mis. (b) Excoriated grouped papulovesicles on sacral area Figure 11.9  Bullae over violaceous papules and plaques
in dermatitis herpetiformis. (a,b – Courtesy: Dr. Piyush in bullous lichen planus. (Courtesy: Dr. Piyush Kumar,
Kumar, Katihar, India.) Katihar, India.)
264  Vesicobullous lesions: Generalized

●● Lichenoid eruptions consist of pruritic violaceous ●● Mucosa and nail involvement may occur at times.
polygonal papules and plaques with a shiny surface. ●● DD: Bullous lichen planus (blisters limited to LP
●● Blisters and erosions typically appear after the lesions and perilesional skin) (Figure 11.10b), bullous
development of the lichenoid skin changes and classically pemphigoid (over urticarial plaques).
on previously unaffected skin (Figure 11.10a).
●● Acral areas are preferred. Staphylococcal scalded skin syndrome
●● Staphylococcal scalded skin (SSSS) syndrome presents with

superficial flaccid bullae that peel off, resulting in sheets of

desquamation, and generalized erythema along with facial
edema; skin tenderness is present (Figure 11.11a,b).

Figure 11.10  (a) Lichen planus pemphigoides with tense

bulla. The patient has developed lesions preferentially
over atrophic scars, an illustration of Ruocco’s immuno-
compromised district. (b) Bullous lichen planus with tense Figure 11.11  (a) Superficial flaccid bulla with peeling
bullae over lichenoid patch and plaques. (a – Courtesy: and erosion in staphylococcal scalded skin syndrome.
Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr Soumyajit (b) Facial edema with scaling in staphylococcal scalded
Roychoudhury, Berhampore, India.) skin syndrome. (Continued)
 Vesicobullous lesions: Generalized  265

blistering and starts with slowly progressive, diffusely

distributed yellow–orange infiltrates that evolve into
nodular lesions.
●● DD: Infections (viral, bacterial, or mycotic infections),
genetic diseases (epidermolysis bullosa, epidermolytic
hyperkeratosis, incontinentia pigmenti), autoimmune
bullous diseases.

Varicella
●● In varicella there are generalized eruption of

lesions in varying stages of evolution: papules,

papulovesicles, vesicles, pustules, and crusted lesions,
(Figure 11.12a–c).
●● Vesicles with surrounding erythematous halo
Figure 11.11 (Continued)  (c) Extensive flaccid blisters on
the trunk in bullous impetigo. (characteristic “dew drops on rose petal” appearance)
are characteristic. Vesicles may have an umbilicated
appearance (Figure 11.12d,e).
●● The Nikolsky sign is positive.
●● There is no mucosal involvement.
●● It is associated with fever, malaise, and irritability.
●● SSSS is usually seen in infants and children; adults with
renal dysfunction are also at risk.
●● Healing occurs within 10 to 14 days without any
residual changes.
●● Common sites start from the perioral area and
perineum, followed by generalized involvement.
●● Search for the primary site of staphylococcal
infection, such as ears, throat, conjunctiva, and
umbilicus.
●● DD: Viral exanthem, drug reactions, Kawasaki disease,
extensive bullous impetigo (Figure 11.11c), toxic
epidermal necrolysis (areas of skin necrosis, severe
mucosal involvement).

Diffuse cutaneous mastocytosis

●● Mastocytosis is a rare, heterogeneous group of

hematopoietic disorders characterized by an abnormal

increase and accumulation of mast cells in one or more
organ systems.
●● Diffuse cutaneous mastocytosis, the rarest and the

most severe form, presents with erythroderma,

hemorrhagic blisters, and in some cases extensive
bullous lesions.
●● Onset is commonly seen in first two years of life.

●● Lesions are triggered by sudden changes in temperature,

irritability, fever, and teething.

●● Systemic symptoms include itching, flushing,

diarrhea, vomiting, hypotension. and even

anaphylactic shock.
●● Two clinical variants of diffuse mastocytosis are

recognized. The first variant is the “initial bullous

presentation,” which presents as diffuse erythema,
with generalized blistering, and appears early in
life. The second variant is the “infiltrative small
vesicular type,” which presents with limited vesicular Figure 11.12 (a) Neonatal varicella. (Continued)
266  Vesicobullous lesions: Generalized

Figure 11.12 (Continued) (b) Multiple vesicles (with tur-

bid fluid) on an erythematous skin in varicella.
(c) Hemorrhagic varicella. (d) Multiple vesicles on erythem-
atous base resembling “dew drops on rose petals.” Note
central umbilication in some lesions. (e) The vesicle of
varicella with central umbilication. (f) Pigmented atrophic
scars after resolution of varicella lesions. (a – Courtesy:
Dr. Hiral Shah, Baroda Medical College, Vadodara, India;
b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata,
India; c–e – Courtesy: Dr. Piyush Kumar, Katihar, India;
f – Courtesy: Dr. PC Das, Katihar, India.)
 Vesicobullous lesions: Generalized  267

●● Lesions heal with variable atrophy and pigmentation reported in association with psoriasis, pityriasis rubra
(Figure 11.12f). pilaris, Darier, Grover’s, and Hailey-Hailey diseases,
●● It is associated with constitutional features such as fever, and contact dermatitis (both irritant and allergic),
sore throat, and malaise. among others.
●● DD: Pityriasis lichenoides et varioliformis acuta, ●● The lesions are painful and often associated with fever,
pustular pyoderma gangrenosum, papulovesicular malaise, and regional lymphadenopathy.
pityriasis rosea, disseminated herpes zoster. ●● DD: Chickenpox, impetigo, contact dermatitis.

Kaposi varicelliform eruption Stevens-Johnson syndrome/toxic epidermal

●● Kaposi varicelliform eruption (KVE), also known as necrolysis
eczema herpeticum, is a rare and potentially fatal viral ●● This presents with dusky erythematous or purpuric

infection. macules with a tendency to coalesce (Figure 11.14a,b);

●● It is caused mainly by the herpes simplex virus (HSV), skin tenderness is present.
predominantly type I. Other viruses, such as coxsackie ●● There is rapid progression, with vesicles, bullae, skin

A 16, vaccinia, and varicella zoster have also been necrosis, and epidermal sloughing in a few days
implicated in its pathogenesis. (Figure 11.14c,d).
●● It is characterized by widespread clusters of ●● It is usually accompanied by constitutional

umbilicated vesicopustules and eroded vesicles, features.

alternating with punched-out ulcers covered by ●● There is severe mucosal involvement: ocular, oral

hemorrhagic crusts usually located over the head, neck, and genital mucosa are involved in almost all cases
and trunk (Figure 11.13). (Figure 11.14e).
●● KVE usually develops in patients with existing chronic ●● DD: Extensive bullous fixed drug eruption,

dermatoses, especially atopic dermatitis; it has also been erythema multiforme, acute cutaneous systemic

Figure 11.13  Kaposi varicelliform eruption seen as vesicles

and pustules in a patient with pre-existing Darier disease. Figure 11.14  (a) Dusky, erythematous macules and bullous
(Courtesy: Dr. Piyush Kumar, Katihar, India.) in a patient of Stevens-Johnson syndrome. (Continued)
268  Vesicobullous lesions: Generalized

Figure 11.14 (Continued)  (b) Confluent lesions in Stevens-Johnson syndrome. (c) Blisters with skin peeling in toxic epider-
mal necrolysis. (d) Extensive epidermal sloughing in toxic epidermal necrolysis. (e) Severe erosive mucositis in Stevens-
Johnson syndrome. (a,b – Courtesy: Dr. Bhushan Madke, Jawaharlal Nehru Medical College, Datta Meghe Institute of
Medical Sciences, India; c–e – Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)
 Vesicobullous lesions: Generalized  269

lupus erythematosus (acute syndrome of apoptotic Hailey-Hailey disease

pan-epidermolysis), paraneoplastic pemphigus. ●● Also known as familial benign chronic pemphigus, Hailey-

Hailey disease is an autosomal dominant genodermatosis.

Pemphigus foliaceus ●● Dysregulation of intracellular calcium homeostasis
●● Pemphigus foliaceus presents with flaccid, superficial
leads to impaired desmosome function and suprabasilar
vesicles, and bulla, mostly in seborrheic distribution acantholysis of the epidermis.
(Figure 11.15a). ●● Clinically, it is characterized by intertriginous
●● There is prominent scaling and crusting
vesicles, erosions, and weeping, fissured plaques; it is
(Figure 11.15b,c) but no tendency of lesions to spread complicated by pain, secondary infection, and malodor
(Figure 11.15d). (Figure 11.16a–d). Sometimes, in chronic cases,
●● Mucosas are usually spared.
patients may develop verrucous papules and plaques
●● The Nikolsky sign is positive.
(Figure 11.16e).
●● DD: Bullous impetigo, pemphigus vulgaris, lupus
●● Sometimes lesions may appear on the scalp, antecubital
erythematosus. or popliteal fossa, and trunk.
●● Conjunctiva, mucosa, and vulva involvement is rarely
Langerhans cell histiocytosis seen.
●● It clinically presents with papules, vesicles, crusts, and ●● Friction, heat, and sweating tend to exacerbate the disease

petechiae over seborrheic and intertriginous areas. ●● DD: Grover’s disease, pemphigus vegetans.
●● Patients may have fever, anemia, thrombocytopenia,

pulmonary infiltrates, hepatosplenomegaly, and Porphyria cutanea tarda

enlargement of lymph nodes. ●● Porphyria cutanea tarda (PCT) is a metabolic

●● DD: Seborrheic dermatitis, acrodermatitis disorder caused by reduction and inhibition of hepatic
enteropathica. uroporphyrinogen decarboxylase (UROD) enzyme activity.

Figure 11.15  (a) Crusting and scaling in pemphigus folia-

ceus in seborrheic distribution. (b) Predominant crusted
and scaly lesions in pemphigus foliaceus. (c) Pemphigus
foliaceus. (d) Self-limited erosions showing no tendency
to spread in pemphigus foliaceous. (a–d – Courtesy:
Dr. Piyush Kumar, Katihar, India.)
270  Vesicobullous lesions: Generalized

Fig 11.16  (a) The groin showing erythema, maceration,

vesiculopustular lesions, and fissures in Hailey-Hailey
disease. (b) Hailey-Hailey disease affecting antecubital
fossa. (c) The lesion of Hailey-Hailey disease at pressure
and friction prone area of the waist. (d) Vesicobullous
lesions in Hailey-Hailey disease are transient, and patients
more commonly present with erythema, fissures, and
whitish macerated skin. (e) Macerated and verrucous
plaques in groin in Hailey-Hailey disease. (a–d – Courtesy:
Dr. Piyush Kumar, Katihar, India; e – Courtesy: Dr. Niharika
Ranjan Lal, ESIPGIMSR, Kolkata, India.)
 Vesicobullous lesions: Generalized  271

●● It is characterized by photosensitivity, increased skin ●● Additional features include hypertrichosis and

fragility, tense blisters and erosions over sun-exposed sclerodermoid features.
areas (Figure 11.17a). ●● Common sites are sun-exposed areas: the face and
●● Blisters heal with scarring and milia formation dorsum of hands.
(Figure 11.17b,c). ●● DD: Pseudoporphyria, epidermolysis bullosa acquisita,
and phototoxic drug eruption.
Pseudoporphyria
●● Pseudoporphyria is a photosensitive bullous condition

resembling porphyria cutanea tarda.

●● Risk factors are chronic renal failure, drugs (naproxen),

ultraviolet exposure, and genetic predisposition.

●● Clinical features include clear fluid filled/hemorrhagic

blisters on sun-exposed parts of the body (face, dorsum

of hands); it heals with scars and milia formation.
●● It resembles PCT clinically and histologically; however,

porphyrin levels are normal.

●● DD: PCT, EBA (on trauma-prone areas), phototoxic

drug reactions.
Acute phototoxic drug reactions
●● These develop in a person exposed to photosensitizing

medication in conjunction with sufficient levels of

radiant energy.
●● They begin within minutes to hours after sun exposure.

●● They resemble exaggerated sunburn (Figure 11.18).

Figure 11.17  (a) Crusted vesicles on sun-exposed pinna

and nose in porphyria cutanea tarda. (b) Blisters healed
with scar formation on sun-exposed dorsa of hands in
porphyria cutanea tarda. (c) Porphyria cutanea tarda. Figure 11.18  Diffuse erythema and vesicobullous lesions
Depigmentation (scalp) and crusted erosion (on pinna). of acute phototoxic reaction in a case of vitiligo receiving
(Courtesy: Dr. Piyush Kumar, Katihar, India.) PUVA therapy. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
272  Vesicobullous lesions: Generalized

●● Features include erythema and edema; in severe cases ●● The face, upper trunk, and proximal extremities are
there may be vesicles or bullae, followed by desquamation. affected.
●● Common sites include sun-exposed skin areas lithe ●● Systemic features include fever, weight loss, and
face, V area of chest, nuchal region, and the dorsum of arthralgia.
the hands. ●● DD: Pemphigus foliaceus, bullous pemphigoid, bullous
●● DD: Photoallergic drug reaction. fixed drug reaction, vesicobullous lesions of acute LE.

Bullous SLE8 Epidermolytic ichthyosis (bullous congenital

●● Bullous lesions in SLE may be of three types: ichthyosiform erythroderma)
●● Bullous lesions as LE-specific lesions represent ●● Epidermolytic ichthyosis is an autosomal dominant

severe inflammation, seen in acute cutaneous genetic bullous disease.

lupus erythematosus (ACLE) and sometimes ●● Erythroderma and epidermal blistering are present at

subacute cutaneous lupus erythematosus (SCLE); birth.

presents like erythema multiforme or toxic ●● Blistering diminishes and resolves with age; there is

epidermal necrolysis. progressive hyperkeratosis.

●● Bullous SLE has autoantibodies against type VII ●● Thickened skin, scaling, palmoplantar keratoderma,

collagen. accentuated creases, generalized ichthyosis, spiny

●● Rarely, SLE may be seen in association with other papules, and malodorous body are prominent at
autoimmune blistering disorders like pemphigus advancing age. (Figure 11.20a–c).
vulgaris, pemphigus foliaceus, etc. ●● Depending upon these clinical grounds, six subtypes of

●● Clinical features include blisters on sun-exposed sites; the disease are recognized.
tense blisters form on an erythematous/urticarial base ●● There is skin fragility, and bouts of blistering are

(Figure 11.19). precipitated by fever, skin infections, and friction.

●● DD: Epidermolysis bullosa, staphylococcal scalded skin

syndrome, bullous pemphigoid.

Figure 11.20  (a) Superficial erosion and oozing in epider-

Figure 11.19  Vesicles and bulla over erythematous molytic ichthyosis. (b) Parallel ridges of hyperkeratosis
base in photoexposed distribution in bullous systemic mimicking corrugated cardboard on the posterior aspect
lupus erythematosus. (Courtesy: Dr. Shvetha Jain, of the ankle joint. Note a small erosion on the medial
Mumbai, India.) aspect of right leg. (Continued)
 Vesicobullous lesions: Generalized  273

Figure 11.20 (Continued)  (c) Classic “corrugated card-

board” like parallel ridges of hyperkeratosis on the knee
joint. (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Inherited epidermolysis bullosa

●● This presents as tense blisters filled with serous

or hemorrhagic fluid on a non-inflammatory base

(Figure 11.21a).
●● Common sites include trauma-prone surfaces: acral

sites, elbows, and knees; sometimes it is generalized

(Figure 11.21b–d).
●● Mucosal involvement is variable with laryngeal and

esophageal involvement in some cases.

●● It heals with atrophic scarring (Figure 11.21e) and

hyper- or hypopigmentation; there is milia formation in

dystrophic forms of epidermolysis bullosa.
●● Onset is from birth, in the neonatal period.

●● There are variable nail (Figure 11.21f) and dental

changes, depending on the epidermolysis bullosa

subtype (Figure 11.21g,h).
●● DD: Other skin fragility disorders like porphyria.

Congenital syphilis
●● Congenital syphilis is a multisystem infection caused by

Treponema pallidum and transmitted to the fetus via the

placenta.
●● It is asymptomatic at birth; symptoms develop within

the first two months of life.

●● Early congenital syphilis (first two years of life)

usually manifests as characteristic skin lesions, such as

vesiculobullous or a macular copper-colored rash on the
palms and soles and papular lesions around the nose and
mouth and in the diaper area, as well as petechial lesions. Figure 11.21  (a) Blister filled with clear fluid on non-
●● Other features include rhinitis, bone abnormalities,
inflammatory base. (b) Front view showing blisters, ero-
chorioretinitis, lymphadenopathy, hepatosplenomegaly, sions, and scarring on trauma-prone areas in a child with
and nephrotic syndrome. dystrophic epidermolysis bullosa. (Continued)
274  Vesicobullous lesions: Generalized

Figure 11.21 (Continued)  (c) Back view of same child. (d) Erosions and skin peeling on back of child in epidermolysis bullosa.
(e) Blisters healed with scarring on hands in dystrophic epidermolysis bullosa. (f) Anonychia in a female with dystrophic epider-
molysis bullosa. (g) Epidermolysis bullosa in a nine-day-old child presenting with erosion with profuse bleeding. (Continued)
 Vesicobullous lesions: Generalized  275

REFERENCES
1. Hill SF, Murrell DF. Differential diagnosis of vesiculobullous lesions.
In, Hoeger P, Kinsler V, Yan A, editors. Harper’s Textbook of
Pediatric Dermatology. 4th edition. New Jersey: Wiley-Blackwell,
2020. P. 859–867.
2. Hull C, Zone JJ. Approach to the patient with cutaneous blisters.
In, Stratman E, Ofori AO, editors. UpToDate, Waltham, MA.https://
www.uptodate.com/contents/approach-to-the-patient-with-­
cutaneous-blisters. Accessed on 07 April, 2020.
3. van Beek N, Zillikens D, Schmidt E. Diagnosis of autoimmune bul-
lous diseases. J Dtsch Dermatol Ges 2018;16(9):1077–1091.
4. Bogdanov I, Darlenski R, Hristakieva E, Manuelyan K. The rash
that presents as a vesiculobullous eruption. Clin Dermatol
2020;38(1):19–34.
5. Jaleel T, Kwak Y, Sami N. Clinical Approach to Diffuse Blisters. Med
Clin North Am 2015;99(6):1243–1267, xii.
6. Oberlin KE. Vesiculobullous and Pustular Diseases in Newborns.
Cutis 2017;100(4):E18–E21.
7. Zhao CY, Murrell DF. Blistering diseases in neonates.
CurrOpinPediatr 2016;28(4):500–506.
8. Ashi, Kumar P. Photosensitivity and Bullous Lesions. In: Rahmani F,
Figure 11.21 (Continued)  (h) Father of the child Rezaei N, editors. Pediatric Autoimmunity and Transplantation.
(Figure 11.21g) has dystrophic epidermolysis bullosa. 1st edition. Cham Switzerland: Springer International Publishing;
2020. P. 337–343.
Note erosions, scarring, and variable degree of nail loss.
(a–h – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Syphilitic pemphigus, a rare bullous variant, most

commonly occurs on palms and soles but may be
generalized.
●● DD: Bullous impetigo, congenital pemphigus,
congenital candidiasis.
 12
Pustules: Localized

MADHULIKA MHATRE, ASEEM SHARMA, TRUPTI AGARWAL

INTRODUCTION ●● DD: Dissecting folliculitis of scalp (male patients,

boggy confluent nodules), acne keloidalis nuchae
Localized pustules can be seen in a variety of infective (firm papules/nodules over occipital scalp with
and inflammatory conditions. Pustular lesions may be hypertrophic scarring), erosive pustular dermatosis
seen as exclusive lesions or may occur along with papules (elderly women, suppurative, necrotic, erosive
(acne vulgaris, rosacea), nodules (nodulocystic acne) or papules and plaques), deep fungal infections,
vesicles (pompholyx). Several defining features, including bacterial folliculitis (early scarring and lack of
patient’s age and general health, distribution of lesions, ostia).
and duration of lesions aid in making a clinical diagnosis.
Follicular localization is usually seen in infective condi-
tions and is another important clinical clue. The clinical Erosive pustular dermatosis of scalp
approach to the diagnosis of localized pustules has been ●● This is a chronic inflammatory disease with an
summarized in Table 12.1. Of these listed conditions, a unknown cause.
few will be omitted from the detailed discussion owing to ●● It is commonly seen in elderly populations and has a
the relative rarity of the same.1–5
female preponderance.
●● Risk factors include cutaneous atrophy from any cause,

Folliculitis decalvans chronic sun exposure, trauma and tissue damage

●● Folliculitis decalvans is a rare, chronic neutrophilic and inflammatory triggers such as HZ, sunburn, hair
cicatricial (scarring) alopecia, speculated to be transplants, topical chemotherapy, cryotherapy, and
an abnormal response to bacteria, particularly photodynamic therapy (PDT).
●● Patients complain of pain, pruritus, or burning at the
Staphylococcus aureus.
●● Predisposing factors are trauma and seborrhea. site.
●● Clinically, it presents as sterile non-follicular pustules,
●● It affects young to middle-aged adults.

●● Males are affected more than females. yellow-brown keratotic crusts, and superficial erosions.
●● It affects African Americans more than The surrounding area shows atrophy of affected skin,
Caucasians. with scarring alopecia.
●● DD: Cicatricial (mucous membrane) pemphigoid,
●● Symptoms include pain, itching, burning sensation, and

spontaneous bleeding. folliculitis decalvans, pemphigus foliaceus,

●● Clinical morphology is as follows: pemphigus vulgaris, Langerhans cell
●● The initial lesion is an erythematous follicular histiocytosis.
pustule.
●● It shows livid, bright erythema with yellow-gray
scales around follicles associated with follicular Bacterial folliculitis and impetigo
hyperkeratosis, erosions. and hemorrhagic crusts ●● Folliculitis is a superficial bacterial infection of the hair

(Figure 12.1). follicles with purulent material.

●● It progresses to irregularly shaped, atrophic ●● It primarily affects children but can present in adults

flesh-colored or ivory/white patches of cicatricial as well.

alopecia. ●● There is no sex predilection.

●● Tufted folliculitis, with multiple hairs (5 to 20) ●● Bacterial folliculitis may occur anywhere on the body,

emerging from a single dilated follicular orifice. including the face.

276 DOI: 10.1201/9781351054225-22
 Pustules: Localized  277

Table 12.1  Clinical approach to dermatoses presenting as

localized pustules

Site Features Diseases

Scalp Children Tinea capitis*

Bacterial folliculitis*
Neonatal Langerhans cell histiocytosis
Adult Folliculitis decalvans*
Erosive pustulosis of scalp
Face Children Impetigo contagiosa and bullous
impetigo
Neonatal cephalic pustulosis
(neonatal acne)*
Perioral dermatitis
Neonatal Langerhans cell
histiocytosis*
Adult Acne vulgaris*
Acneiform eruptions
Rosacea (papulopustular)
Sycosis barbae*
Tinea barbae*
Acne keloidalis nuchae*
Pseudofolliculitis barbae*
Pyoderma faciale
Any age Herpes simplex
group Acute Localized Exanthematous
Pustulosis (ALEP)
Trunk Children Keratosis pilaris*
Adult Truncal acne*
Pityrosporum folliculitis*
Gram negative folliculitis*
Acneiform eruptions
Eosinophilic folliculitis*
Figure 12.1  Pustules with scaling in folliculitis decalvans.
Subcorneal pustulosis of Lesions have healed with scarring. (Courtesy: Dr. Bhushan
Sneddon-Wilkinson Madke, Jawaharlal Nehru Medical College, Datta Meghe
Any age Miliaria pustulosa Institute of Medical Sciences, India.)
group
Extremities/ Children Scabies
acral areas Infantile acropustulosis
Papular urticaria ●● Risk factors include areas of repeated shaving,
Parakeratosis pustulosa diabetes mellitus, and immuno-compromised
Adult Dermatitis cruris pustulosa et individuals.
atrophicans (DCPA)* ●● Clinically, folliculitis manifests as clusters of multiple
Acrodermatitis continua suppurativa small, raised, pruritic, erythematous lesions less
of Hallopeau
Pustular pyoderma gangrenosum
than 5 mm in diameter. Pustules may be present at the
Orf centers of the lesions (Figure 12.2a). Impetigo occurs
Sporotrichosis most commonly on the face and can present with
Palms and Adult Contact dermatitis bullae, honey-colored crusts, erythema, edema, and
soles Palmoplantar pustulosis exudate, commonly in children (Figure 12.2b,c).
Any age Pompholyx ●● DD: Pityrosporum folliculitis, superficial actinic
group folliculitis, folliculitis following application of epilating
Flexures Children Bullous impetigo agents, folliculitis due to exposure to occupational
Adults Amicrobial pustulosis of the folds* agents (chemicals, oils) (Figure 12.2d), perforating
Hidradenitis suppurativa*
folliculitis (Kyrle’s disease).
Subcorneal pustulosis of
Sneddon-Wilkinson
Any age Candidiasis*
group Miliaria pustulosa
Sycosis barbae
●● Sycosis barbae is a staphylococcal infection of the entire
Anywhere/no Any age Herpes Zoster
site group Folliculitis* depth of hair follicle.
restriction Dermatophyte infection ●● It affects post-adolescents.
Insect contact dermatitis ●● Males are affected more than females.

* Follicular pustules ●● The primary site is the beard area.

278  Pustules: Localized

Figure 12.2  (a) Closely set pustules pierced by a hair on the erythematous base on neck in bacterial folliculitis.
(b) Pustules, vesicles, and crusts in a child with bullous impetigo. (c) Crusted vesicles and pustules with surrounding
erythema in bullous impetigo in a child. (d) Follicular pustules due to occlusion by oil. (a,b,d – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

●● Predisposing factors include seborrhea and indoor ●● DD: Pseudofolliculitis (typically non-grouped), tinea
work. barbae (typical plaque, may be annular, history of animal
●● Clinical morphology shows the following: contact, relatively asymptomatic or mildly pruritic).
●● Oedematous erythematous papule or pustule which
is folliculo-centric (Figure 12.3a–c). Tinea barbae
●● Neighboring follicular papules may coalesce to form ●● Three types of dermatophytes account for the majority

a plaque studded with pustules resembling a ripe fig; of infections: epidermophyton, trichophyton, and
hence the term “sycosis.” microsporum.
●● Lesions are grouped. ●● The primary site is the beard area.

●● There is crusting and scaling. ●● It affects adults.

●● The disease runs a chronic relapsing and remitting ●● Dermatophyte infection of the beard area can be

course and heals with scarring. superficial and non-inflammatory or deeper and
 Pustules: Localized  279

Figure 12.3  (a) Erythematous patch studded with

folliculocentric crusted pustules typically affecting
the hair-bearing beard and moustache area in
sycosis barbae. (b) Folliculocentric pustules in hair-
bearing chin and neck regions in sycosis barbae.
(c) Sycosis barbae. (a – Courtesy: Dr. Piyush Kumar,
Katihar, India; c – Courtesy: Dr. Niharika Ranjan Lal,
ESIPGIMSR, Kolkata, India.)

inflammatory (kerion type) and is characterized by

scaly, annular plaques with pustules and broken-hairs
(Figure 12.4) (superficial; most commonly caused by
T. rubrum or T. violaceum) or a deep inflammatory
nodule or plaque with pustules and sinus tracts (deep;
most commonly by T. mentagrophytes or T. verrucosum).
●● DD: Acne vulgaris, bacterial folliculitis, herpetic
sycosis, perioral dermatitis, pseudofolliculitis barbae,
sycosis barbae.

Herpes simplex
●● Caused by herpes simplex virus type 1 (HSV-1), this is also
Figure 12.4  Erythematous swelling studded with pustules
known as herpes labialis. It may be a primary or recurrent and loss of hair in the moustache area in tinea barbae.
infection (commonly in immunosuppressed patients). (Courtesy: Dr. Piyush Kumar, Katihar, India.)
280  Pustules: Localized

●● It affects adults and, rarely, children (oropharyngitis Neonatal acne/infantile acne

more than labialis). ●● Also known as acne neonatorum, acne infantum,
●● There is no sex predilection. and neonatal cephalic pustulosis, this is an acneiform
●● Primary sites are as follows. Labialis: lower vermilion eruption that occurs due to the increased sensitivity
lip margin; Genitalis: labia minora or glans; Whitlow: of the infant’s sebaceous glands to maternal hormones
finger tips. during pregnancy.
●● Herpes simplex eruptions present as grouped pustules ●● It affects newborns or neonates; infantile acne affects
or vesicles on an erythematous base typically on the infants three to six months of age.
lower vermilion lip of the face (Figure 12.5a,b). They ●● There is no sex predilection.
may present similarly in the genital (Figure 12.5c) ●● Common sites are the cheeks, chin, and forehead.
or sacral areas, particularly with recurrent disease. ●● Clinical morphology shows comedones, papules, and
Patients frequently have a history of a prodrome prior pustules and rarely nodules and cysts.
to the onset of lesions. ●● DD: Folliculitis, perioral dermatitis, milia, miliaria.
●● Precipitating factors include fever, windburn or
sunburn, trauma, or stress.
●● DD: Aphthous stomatitis, Behçet’s disease, bullous Acne vulgaris
pemphigoid, candidiasis, erythema multiforme, fixed ●● Acne vulgaris is the most common cutaneous disorder

drug eruption. affecting adolescents and young adults.

Figure 12.5  (a) Grouped pustules on upper lip and

below lower lip in herpes labialis. (b) Grouped and
coalesced pustules near the angle of mouth in herpes
labialis. (c) Few intact pustules (marked by arrow) and
multiple ulcers in anogenital herpes. (a–c – Courtesy:
Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
 
 Pustules: Localized  281

●● Acne vulgaris is a disease of pilosebaceous follicles.

Four factors are involved:
●● Follicular hyperkeratinization
●● Increased sebum production
●● Propionibacterium acnes (P. acnes) within the
follicle
●● Inflammation
●● Risk factors include conditions causing androgen
excess, use of pomades, family history, stress, use of
comedogenic cosmetics.
●● It primarily affects adolescent adults but can be seen
well into adulthood.
●● It affects females more than males.
●● The most common sites are areas of the body that have
the largest hormonally responsive sebaceous glands,
including the face, neck, chest, upper back, and upper
arms.
●● Clinical picture is typically pleomorphic with
pustules along with other characteristic acne
lesions including comedones, papules, nodules,
postinflammatory hyperpigmentation, and scarring
(Figure 12.6).
●● There may be a history of acne-inducing medication
such as glucocorticoids, phenytoin, lithium, isoniazid,
epidermal growth factor inhibitors, iodides, bromides,
androgens, Vitamins B2, B6, and B12.
●● DD: Rosacea (no comedones and presence of
telangiectasia), perioral dermatitis (small, grouped
erythematous papules sparing vermilion border),
pseudofolliculitis barbae and acne keloidalis
nuchae (beard and nuchal area affected), folliculitis,
steatocystoma multiplex, acneiform eruptions.

Perioral dermatitis (periorificial dermatitis,

circumoral dermatitis)
●● Deficiencies in skin barrier function and features of

atopy have been detected at increased frequency in

patients with perioral dermatitis.
●● Recent use of topical, nasal, or inhaled corticosteroids

may be elicited.
●● There is a history of disease flares after topical

corticosteroid withdrawal.
●● Potential contributors include fluorinated toothpaste, Figure 12.6  Pustules mixed with inflammatory papules
skin moisturizers and cosmetic products, fusobacteria, over forehead and nose in acne vulgaris.
Candida albicans, hormonal fluctuations in women, and
oral contraceptive therapy.
●● Ages between 16 and 45 constitute the vast majority of ●● The skin lesions usually resolve without scarring.
cases. ●● Clinical morphology shows multiple 1- to 2-mm
●● It affects females more than males. clustered erythematous papules, papulovesicles, or
●● The most common site is the perioral region, but a papulopustules, with or without mild scaling, and
narrow zone around the vermilion border of the lip is sparing of the skin immediately adjacent to the
usually spared. Less often, the perinasal and periorbital vermilion border of the lip (Figure 12.7a,b).
areas are affected. ●● There may or may not be signs of eczematous
●● It may be asymptomatic or may be accompanied by dermatitis.
mild to moderate stinging or burning sensations in the ●● DD: Rosacea, seborrheic dermatitis, demodex
affected areas. infections, lupus miliaris disseminatus faciei, allergic
282  Pustules: Localized

Acne keloidalis nuchae

●● Acne keloidalis nuchae is a chronic inflammatory

condition.
●● It affects post-adolescent young adults.

●● It affects males 20 times more than females.

●● The most common site is the nape of the neck.

●● Risk factors include skin injuries from irritation,

occlusion, trauma, friction, and hair-cutting

practices.
●● Early lesions of acne keloidalis are inflamed

papules with marked erythema. Pustules may

develop as a result of a sterile, acute inflammatory
response to embedded hair or as a manifestation of
secondary infection. Papules may be skin-colored,
erythematous, or hyperpigmented and coalesce into
large plaques and nodules. Secondary infection
can lead to pustules and abscess formation in some
cases. Over time, continued inflammation leads to
pronounced fibrosis and keloid formation. Late
stages of presentation include chronic scarring
and/or scarring alopecia without active
inflammation.
●● DD: Bacterial folliculitis (acute condition), dissecting

cellulitis, folliculitis decalvans.

Rosacea (papulopustular type)

●● Dysfunction of the innate immune system

may contribute to the development of chronic

inflammation and vascular abnormalities in
rosacea.
●● Microorganisms (Demodex, Bacillus olenorium,

and H. pylori), ultraviolet radiation, and

vascular hyper-reactivity might play a role in
pathogenesis.
●● A positive family history may be noted.

●● Exacerbating factors include exposure to extremes of

temperature, sun exposure, hot beverages, spicy foods,

alcohol, exercise, irritation from topical products,
psychological feelings (especially anger, rage, and
embarrassment), and certain drugs such as nicotinic
acid and vasodilators.
●● It primarily affects adults older than 30.

●● It affects females more than males.

●● Symptoms include persistent centrofacial redness

and pustular eruptions in moderate to severe

Figure 12.7  (a) Perioral pustules in perioral dermatitis. cases.
(b) Perioral dermatitis presenting as erythema studded with ●● Clinical clues include pustular rosacea found
tiny pustules. Note involvement of perioral and periocular frequently on the central face. Look for telangiectasia,
area. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
erythema, papules, nodules, thickening of the soft
tissue, and sebaceous prominence of the central face.
contact dermatitis (unlike POD, intense pruritus is In occasional patients the whole nose may be tender,
usually present, scale is often prominent, and lesions fail swollen, and red.
to improve with antibiotic therapy), impetigo (erosions, ●● DD: Acne vulgaris (comedones do not occur in

vesicles and yellow crusts present; scaling and acne-like rosacea and inflammation may extend outward
papules are uncommon). well beyond the follicular unit to form plaques),
 Pustules: Localized  283

seborrheic dermatitis (a greasy quality to the scale

and involvement of other sites such as the scalp,
retroauricular skin, and brow suggest this diagnosis),
topical steroid-induced acneiform eruptions (topical
corticosteroid use can result in the appearance of
monomorphic inflammatory papules and pustules
on facial skin; the patient history is valuable for
diagnosis), perioral dermatitis (perioral dermatitis
presents with numerous small papules with fine scale
in the perioral area; sparing of the skin adjacent to
the vermillion border of the lip is characteristic),
demodicosis (Demodex folliculitis) (demodicosis can
present with numerous inflammatory papules on the
face and is difficult to distinguish from papulopustular
rosacea on clinical examination).

Pityrosporum folliculitis
●● Malassezia (Pityrosporum) folliculitis (MF) is an
acneiform eruption that results from an overgrowth
of the Malassezia yeast present in the normal
cutaneous flora, secondary to occlusion of the follicle
or disturbance of normal cutaneous flora. The yeast is
primarily found in the infundibulum of the sebaceous
glands, as it thrives on the lipid composition of sebum.
●● Risk factors include humid environment, tight non-

absorbent clothes, excessive sweating, topical or

oral antibiotic use, particularly tetracyclines, oral
corticosteroid use, immunosuppression. Figure 12.8  Monomorphic papules and pustules on upper
●● It primarily affects adolescents, and affects males more
back in pityrosporum folliculitis.
than females.
●● It presents as intensely pruritic, 1- to 2-mm,
●● Clinical morphology shows superficial small pustules
monomorphic follicular papules and pustules. The located in the nasolabial line and on the upper lip and
absence of comedones helps differentiate it from acne chin, associated with inflammatory papulopustular
(Figure 12.8). lesions of the cheeks and perioral region (Figure 12.9a,b).
●● It appears often on the upper back, chest, and shoulders
●● DD: Acne vulgaris.
and to a lesser extent on the neck, face (chin and sides of
face), or upper arms. Scabies
●● It may be associated with seborrheic dermatitis or
●● Scabies affects children more than adults.
pityriasis versicolor.
●● There is no sex predilection.
●● DD: Acne mechanica, acne vulgaris, candidiasis, drug-
●● Risk factors include overcrowding, sharing of linen/
induced acne, eosinophilic folliculitis, steroid acne,
clothes/towels.
gram-negative folliculitis, Pseudomonas folliculitis,
●● There is intense pruritus, more at night; family members
rosacea.
may have similar symptoms.
●● Clinical morphology shows isolated papules, vesicles,

Gram-negative folliculitis and pustules particularly located in the interdigital

●● Gram-negative folliculitis is often seen as a web spaces, volar wrists, axillae, breasts, umbilical, and
generalized pustular eruption that ranges from mildly groin areas (Figure 12.10).
symptomatic to being associated with considerable ●● DD: Eczema, atopic dermatitis, Langerhans cell

pain and pruritus. This may occur on the face as a histiocytosis, papular urticaria, acropustulosis of
sudden pustular flare of acne previously controlled by infancy, pompholyx.
chronic oral antibiotics.
●● Truncal involvement is typically seen in healthy Infantile acropustulosis
patients after use of recreational hot tubs, with ●● Infantile acropustulosis is a chronic, recurrent, self-limited,

Pseudomonas aeruginosa identified as the pathogen pruritic, vesicopustular eruption in infants, postulated to
on culture. be a non-specific hypersensitivity reaction to scabies.
284  Pustules: Localized

Figure 12.10  Pustules seen in web spaces in scabies.

(Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata,
India.)

●● It affects young children during the first two to twelve

months of life. Resolution by age three is the norm.
●● There is no sex predisposition.
●● Symptoms include pruritus and irritability.
●● There are recurrent crops of pruritic erythematous
macules or papules that progress into vesicles and then
pustules.
●● Individual bouts may last three to fifteen days and recur
in two- to four-week intervals. The attacks occur with
progressively diminishing numbers of lesions, and with
decreasing frequency, until they cease altogether.
●● DD: Dyshidrotic eczema, eosinophilic pustulosis,
erythema toxicum neonatorum, fire ant bites, impetigo,
Langerhans cell histiocytosis, pustular psoriasis, scabies.

Parakeratosis pustulosa6
●● Parakeratosis pustulosa is chronic dermatosis with

erythema-squamous lesions with benign clinical

course, spontaneous remissions, and improvement
with emollients alone in most patients and an obscure
etiology.
●● It affects children under five years of age.
Figure 12.9  (a) Pustules in gram negative folliculitis.
●● It affects females more than males.
(b) Sudden appearance of erythematous nodules on the
cheek in gram negative folliculitis. (a,b – Courtesy: ●● The thumb or middle finger is affected more often than

Dr. Piyush Kumar, Katihar, India.) the toes.

 Pustules: Localized  285

Table 12.2  Diagnosis of parakeratosis pustulosa

Major criteria
• Children, particularly young girls <5 years
• Benign course, spontaneous remissions
• Other dermatoses are excluded
Minor Criteria
• Absence of similar disease in family members
• Rare and transient postulation, not extending
beyond the initial phase
• Response to topical emollients
• Non-specific histology

●● Erythema and scaling of periungual skin are the

predominant features, while pustule formation may
occur only in the beginning (Table 12.2).
●● Nail findings include subungual hyperkeratosis and
onycholysis with or without nail pitting or ridging.
●● DD: Pustular psoriasis (Table 12.3), acrodermatitis
continua.
Figure 12.11  Acrodermatitis continua of Hallopeau showing
Acrodermatitis continua (pustular acrodermatitis, frank pustule (blue arrow), old scaly hyperkeratotic patch
(red arrow) with anonychia of affected nail. (Courtesy:
acrodermatitis continua suppurativa Hallopeau ACH,
Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)
dermatitis perstans, and dermatitis repens Crocker)
●● This is a rare and chronic eruption of sterile painful
erythematous pustules that frequently coalesce to form
pustules associated with hyperkeratosis and skin
diminutive lakes of pus.
atrophy on the distal fingers and toes. ●● As individual lesions progress, the affected areas often
●● It is considered by many to be a localized variant
become hyperkeratotic and scaly as new pustules continue
of pustular psoriasis, associated with enhanced
to develop underneath the fissured surface (Figure 12.11).
polymorphonuclear leukocyte (PMNL) chemotaxis. ●● Over time the skin in these areas may become severely
●● It affects adults 40 to 50 years old.
atrophic with shiny erythema.
●● Females and males are affected in a ratio of F:M=3:1.
●● Nail involvement includes characteristic pustulation within
●● ACH most often presents at the nail folds of one or
the nail bed and matrix, leading to onychodystrophy,
two fingers, less frequently on the toes, with small,
onychomadesis, onycholysis, or permanent nail loss.
Table 12.3  Clinical differentials of parakeratosis pustulosa

Parakeratosis Acrodermatitis continua Pustular psoriasis

Features pustulosa of Hallopeau of the nails Nail Psoriasis
Age/sex distribution Young girls Young females, seldom No predilection No predilection
adult onset
Clinical course Chronic, benign, Aggressive, mutilating, can Aggressive, Benign, progressive
painless, recurrent progress to generalized mutilating
pustular psoriasis
Spontaneous remissions Usual Seldom None None
Specific Nail changes Lateral onycholysis, Distal onycholysis, Proximal onycholysis, Proximal onycholysis,
subungual hyperkeratosis, nail dystrophy subungual
nail bed pustules, nail owing to nail bed hyperkeratosis,
dystrophy and loss, distal pustules pitting, oil-drop, and
osteolysis commonly seen salmon-patch signs
Pustular lesions Rare/transient Common (nail bed) Common (nail bed) Never
Scaling Sometimes Tips of digits Not seen Periungual
Cutaneous lesions None None Lesions of pustular Lesions of plaque
psoriasis psoriasis
Histology Non-specific, mimics Pustular psoriasis Pustular psoriasis Plaque psoriasis
eczema
286  Pustules: Localized

●● Recurrent inflammation may induce sclerosis of Orf

underlying soft tissue, muscular atrophy, arthropathy of ●● Orf is a zoonotic disease caused by a parapox virus and
interphalangeal joints, and osteolysis with resorption of occurring primarily in sheep and goats. It is also known
the distal phalanx. as contagious pustular dermatitis, infectious labial
●● DD: Pustular psoriasis, palmoplantar pustulosis dermatitis, ecthyma contagiosum, thistle disease, and
(does not show propensity for pustules to coalesce, scabby mouth.
localization over distal extremity, or atrophy and nail ●● It affects adults more than children.
loss), acute contact dermatitis (vesicular and not usually ●● It affects males more than females, as it is an
pustular, palmoplantar involvement more common, occupational hazard.
margins are less defined), pustular dyshidrotic eczema, ●● Risk factors include occupations like ranchers,
bacterial or viral paronychia, parakeratosis pustulosa veterinarians, butchers, shearers, and shepherds.
(common in children, limited to one finger, resolves ●● Sites are mainly fingers, hands, and forearms; it rarely
spontaneously at puberty). appears on the scalp and face.
●● It is accompanied by a low-grade fever.
Pyoderma gangrenosum (pustular type) ●● There may be pruritus.
●● This inflammatory neutrophilic skin disease is ●● Systemic symptoms such as lymphadenopathy,

often associated with underlying systemic disorders erysipelas-like lesions, and erythema multiforme can
such as inflammatory bowel disease, arthritis, and occur in up to one-third of cases.
lymphoproliferative disorders. ●● Clinical morphology shows solitary small, firm, red-
●● It affects young to middle-aged adults (40 to 60 years). to-blue papules then progresses through a series of six
●● It affects females more than males. stages. The fully developed orf lesion is typically 2–3 cm
●● The eruption may begin as an isolated pustule or in diameter, but it may reach 5 cm.
scattered lesions on the trunk or extremities. There is ●● Stage 1 (maculopapular or pustular) – A red
surrounding edema and purplish induration, with rapid elevated lesion
progression into a large ulcer that heals ultimately with ●● Stage 2 (targetoid) – A bulla with an iris-like
cribriform scars. configuration (nodule with a red center, a white
●● Other types include ulcerative (classic) PG (the middle ring, and a red periphery) (Figure 12.12a,b)
most common variant), bullous (atypical) PG, ●● Stage 3 (acute) – A weeping nodule
vegetative PG. ●● Stage 4 (regenerative) – A firm nodule covered by a
●● DD: Vasculitis. thin crust through which black dots are seen

Figure 12.12  (a) Pustulonodular lesion of Orf in a young male. Note targetoid appearance. (b) Pustular lesion in orf. (a – Courtesy:
Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. Rajesh Kumar Mandal, North Bengal Medical College, Darjeeling, India.)
 Pustules: Localized  287

Figure 12.13  (a) Pustules (blue arrow), oozing, and scaling on lateral aspect of fingers in pompholyx. (b) Pustules and
vesicles have eroded to leave erythematous areas with erosions, fissures, and scaling. (a – Courtesy: Dr. Niharika Ranjan
Lal, ESIPGIMSR, Kolkata, India; b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Stage 5 (papillomatous) – Small papillomas ●● DD: Acrodermatitis continua of Hallopeau, allergic

appearing over the surface contact dermatitis, dyshidrotic eczema, keratoderma
●● Stage 6 (regressive) – A thick dry crust covering the blennorrhagicum, secondary syphilis.
resolving lesion
●● DD: Cowpox, pseudocowpox (milker’s nodule), Candidial folliculitis
furuncle, atypical mycobacteria infections, anthrax.
●● This is a follicular infection caused by the yeast Candida

albicans or other Candida species

Dyshidrotic eczema/pompholyx ●● Risk factors include immunodeficiency,

●● It presents with intensely pruritic, chronic and nutritional deficiencies, endocrine diseases such
recurrent, vesicular dermatitis that typically involves as dermatomyositis (DM), Cushing’s disease, and
the palms and soles and lateral aspects of the fingers hypothyroidism.
(Figure 12.13a). Isolated pustules also may occur but ●● It affects the elderly, but may be seen in infants and

rarely are the predominant primary lesions. The vesicles young adults.
gradually desquamate over one to two weeks, leaving
erosions and fissures (Figure 12.13b) that slowly resolve.
●● DD: Tinea pedis/manuum, contact dermatitis, hand,

foot, and mouth disease.

Palmoplantar pustulosis (PPP)

●● It is considered to be a localized form of pustular

psoriasis. Psoriasis occurs in 24% of PPP patients.

●● It affects people 30 to 50 years of age.

●● It affects females more than males.

●● Risk factors include tobacco smoking, family history

of psoriasis, presence of another autoimmune disorder,

and family history of palmoplantar pustulosis.
●● Triggers include smoking, infections (especially

streptococcal bacterial infections), stress, and

medications (steroids).
●● It is persistent and painful, leading to a considerable

functional impairment. Figure 12.14  Pinpoint sterile pustules with erythema and
●● Clinical morphology shows sterile pustules and scaling on lateral aspect of foot in palmoplantar pustulo-
erythematous, scaly skin (Figure 12.14). sis. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
288  Pustules: Localized

●● There is no sex predilection. ●● Risk factors include obesity, hyperandrogenism in

●● It presents as erythema and maceration, with typical females, genetics, infection, and high-glycemic diet.
satellite pustules and scaling. ●● Immune factors including cell-mediated immunity is
●● DD: Contact dermatitis, inverse psoriasis. thought to play a role.
●● Symptoms include pain and discomfort, purulent
Amicrobial pustulosis of the folds (APF)7 discharge, and impaired quality of life.
●● This is a recently described neutrophilic dermatosis ●● Pustules may be evident in early lesions, followed by
characterized by relapsing pustular lesions usually nodules and discharging abscesses. Follicular rupture
occurring in the context of an autoimmune disease. and involvement of the apocrine gland occur deeply,
●● It occurs predominantly in cutaneous folds on the scalp resulting over time in extensive scarring and sinus tract
and periorificial regions, such as the mouth, external formation and chronicity. Multi-headed tombstone
ear canal, and nostrils. comedones are present.
●● Affects patients aged 20 to 40 years. ●● Complications include fistulae, strictures and
●● It affects females more than males. contractures, arthropathy, malaise, depression and
●● Clinical morphology shows folliculo-centric pustules suicide, lymphatic obstruction, lymphedema of limbs
with erythema. and genitalia, long-term effects of chronic suppuration
●● DD: Extensive folliculitis, pustular psoriasis, subcorneal (anemia, hypoproteinemia and amyloidosis), and
pustular dermatosis, candidal intertrigo, dermatophyte squamous cell carcinoma (rarely).
infections. ●● DD: Follicular pyodermas (superficial, quick-healing,
no widespread scarring/induration, no comedones),
Subcorneal pustular dermatosis (SCPD) granuloma inguinale (genital area involved, red ulcers
●● It is more common in women, with onset usually in with granulation, easy to bleed), Crohn’s disease (peri-
their forties. anal and vulval abscesses, fistulae, sinus tracts and
●● It is clinically characterized by recurrent crops of “knife-cut” ulcers, previous history of gastrointestinal
pustules, mainly affecting the intertriginous and involvement).
flexural areas. The lesions resolve with scaling, but new
lesions keep appearing. Herpes zoster
●● DD: Pustular psoriasis, acute generalized ●● Herpes zoster is an acute eruption caused by the

exanthematous pustulosis. reactivation of latent varicella-zoster virus and is

characterized by painful grouped vesicles and erosions,
Hidradenitis suppurativa usually confined to a single dermatome.
●● Hidradenitis suppurativa is also known as acne inversa, ●● It usually affects adults older than 50.

and in older literature as Verneuil’s disease ●● There is no sex preponderance.

●● The etiology is chronic follicular occlusion. ●● The sites affected are dermatomal, rarely multi-

●● The average age of onset is at puberty – second to third dermatomal (if immunocompromised).
decade. ●● Risk factors include immuno-compromised individuals,

●● Females and males are affected in a ratio of F:M=3:1. old age, stress, and other comorbidities.
●● It affects areas of hormonally influenced apocrine sweat ●● Herpes zoster may present early on as isolated pustules

glands, including the axillae, mammary, and inguinal or vesicles with significant discomfort, typically in a
regions. dermatomal distribution (Figure 12.15a,b).

Figure 12.15  (a) Grouped pustules coalescing to form large pus-filled bulla along L2–L3 dermatomes in herpes zoster.
(b) Grouped pustules on erythematous base along T1–T2 dermatome in herpes zoster. (a,b – Courtesy: Dr. Niharika Ranjan
Lal, ESIPGIMSR, Kolkata, India.)
 Pustules: Localized  289

key primary lesion when follicular units are involved,

particularly on the legs, scalp, or forearms.
●● Risk factors include occlusive clothing, excessive
sweating, immunocompromised, contact with animals,
diabetes mellitus.
●● Clinical picture depends on the site of affection.
●● Body: Annular erythematous plaques with a border
and scaling (Figure 12.17a,b).
●● Palms and soles: Diffuse scaling with vesiculo-
pustular eruptions occasionally.
●● Scalp: Scaly plaques with follicular pustules
(Figure 12.17c).
●● Groins: Erythematous annular plaques with
maceration.
●● Majocchi’s granuloma results from dermal invasion
and presents with perifollicular papules and
pustules.
●● DD: Candidiasis, erythema annulare centrifugum,
pustular psoriasis, pyoderma.

Figure 12.16  Topical steroid abuse on face. Pustules and

erythema with hypertrichosis. (Courtesy: Dr. Niharika
Ranjan Lal, ESIPGIMSR, Kolkata, India.)

●● Symptoms include pain, aching, burning, allodynia,

fever, and malaise.
●● Common complications include postherpetic neuralgia,
bacterial skin infection, and ocular complications,
including uveitis and keratitis.
●● DD: Zosteriform herpes simplex, cellulitis, erysipelas,
folliculitis.
Chronic glucocorticoid use and abuse
●● Chronic topical corticosteroid use can cause pustular

lesions on the face and trunk. These lesions are typically

asymptomatic.
●● It affects young to middle-aged adults.

●● It affects females more than males.

●● Risk factors include use of whitening creams and over-

the-counter medications containing steroids.

●● It is generally asymptomatic, but photosensitivity may

be present.
●● It presents with pleomorphic acneiform lesions, such as

papules and pustules, with erythema and mild scaling.

Associated dryness, perioral dermatitis, telangiectasia,
rosacea-like features, hypopigmentation, and
hyperpigmentation may be seen (Figure 12.16).
●● DD: Acne, rosacea, seborrheic dermatitis, LMDF,

demodex folliculitis, contact allergic dermatitis.

Dermatophyte infections
●● Dermatophyte (superficial fungal) infections, such as Figure 12.17  (a) Annular plaques studded with pustules
tinea pedis or corporis, typically occur with papules, on the peripheral active margin in tinea corporis in an
plaques, and peripheral scale. Pustules may also be a infant. (Continued)
290  Pustules: Localized

Figure 12.17 (Continued)  (b) Pustules mixed with excoriated papules on active margin in tinea corporis. (c) Pustules, crusting,
and scales with hair loss over scalp in tinea capitis. (a,b – Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR, Kolkata, India.)

REFERENCES 4. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
1. Goldstein BG, Goldstein AO. Approach to the patient with pustular Chapter 12. Pustular Eruptions, Nonfollicular. P. 205–216.
lesions. In: Dellavalle RP, Levy ML, Corona R, editors. UpToDate. 5. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology:
2020. Available from: https://www.uptodate.com/contents/ Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018.
approach-to-the-patient-with-pustular-skin-lesions Chapter 23. Follicular Disorders. P. 375–384.
2. Mengesha YM, Bennett ML. Pustular skin disorders: Diagnosis and 6. Mahajan VK, Ranjan N. Parakeratosis pustulosa: A diagnostic
treatment. Am J Clin Dermatol 2002;3(6):389–400. conundrum. Indian J Paediatr Dermatol 2014;15:12–15.
3. Lipsker D. Clinical Examination and Differential Diagnosis of Skin 7. López-Navarro N, Alcaide A, Gallego E, et al. Amicrobial pustulosis
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 29. of the folds associated with Hashimoto’s thyroiditis. Clin Exp
Pustules. P. 181–182. Dermatol 2009;34:e561–e563.
 E9
Pustules: Generalized

ASEEM SHARMA, MADHULIKA MHATRE, TRUPTI AGARWAL

ABSTRACT
Generalized pustules may evolve from papulo-vesicular lesions or, less commonly, may appear as the primary lesion itself. The
latter may be a pointer to serious, life-threatening conditions. This chapter discusses a clinical approach to the diagnosis of
various diseases presenting with generalized pustules.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-23 291

 E10
Clinical approach to ulcers

SOURABH JAIN

ABSTRACT
Cutaneous ulcers can develop in a diverse range of pathological processes. The clinical features in these ulcers may not be
distinctive in many ulcers, but detailed history and thorough clinical examination can provide clues necessary to arrive at
a diagnosis or get a list of differentials. This chapter discusses important features of cutaneous ulcers that can play a role in
clinical diagnosis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

292 DOI: 10.1201/9781351054225-24

 13
Ulcer: Single or few

B SHINY SHULAMITE, CH VENKATA KRISHNA, SRI HARSHA KOLLA,

SAI PRASANTH NETI, VIGNASREE A

INTRODUCTION primary and secondary lesions over which ulceration has

occurred (Table 13.1). For example, most infections start
Ulcers can be congenital or acquired with various eti- with papules/nodules, with secondary ulceration (e.g.,
ologies, such as infective, inflammatory, traumatic, cutaneous leishmaniasis, sporotrichosis). Another diag-
and ischemic. The most common site of skin ulcers is nostic clue is changes in the skin surrounding the ulcer.
the lower extremities, but they can occur on any body For example, the presence of purpura and ecchymoses in
part. Most cutaneous ulcers of the lower extremities are the background of ulcers is suggestive of necrotizing fas-
caused by venous insufficiency, arterial insufficiency, or ciitis, warfarin necrosis, etc. The clinical approach to the
neuropathy (especially of diabetic etiology), and in gen- diagnosis of conditions presenting with solitary or few
eral such ulcers are not difficult to diagnose. However, ulcers are outlined in Table 13.2, and salient features are
many ulcers, especially ulcers associated with or due discussed below.1–8
to systemic inflammatory conditions, may have non-
specific clinical findings and are challenging diagnos-
tically. Features that aid in diagnosis include clinical Aplasia cutis congenita
characteristics of the ulcer (as described in Chapter E10), ●● Aplasia cutis congenita (ACC) is a rare, congenital

discharge, symptoms, associated systemic features, and disorder characterized by the focal or widespread
co-morbidities. An important pointer to diagnosis is the absence of skin.
evolution of ulcer – if there is a presence or absence of ●● It usually affects the scalp at midline.

Table 13.1  Evolution of lesions as clinical clue

Primary/ secondary lesion Site/ additional clues Diseases

Papules/ plaque Exposed parts Cutaneous leishmaniasis
Rapid progression into ulcer Pyoderma gangrenosum
Sun-exposed areas Basal cell carcinoma, squamous cell carcinoma
On legs; diabetic Necrobiosis lipoidica diabeticorum
Nodule Over lymph nodes Scrofuloderma
Lower limbs Tuberculous gumma
Linear arrangement Lymphocutaneous sporotrichosis, Nocardiosis
Hand Fish tank granuloma
Vesicles/pustule Dermatomal Herpes zoster
Extremity Cutaneous anthrax
Purpura/ ecchymosis Calciphylaxis, warfarin necrosis, Nicolau
syndrome, vasculitis
Erythema and edema Acral parts Frostbite, glanders disease
Anywhere Necrotizing fasciitis
Triphasic color change Acral parts Raynaud’s phenomenon

DOI: 10.1201/9781351054225-25 293

294  Ulcer: Single or few

Table 13.2  Clinical approach to diagnosis of solitary (or few) ulcers

Site Additional clue Diseases

Head and neck Neonates and infants Aplasia cutis congenita (scalp), ulcerated hemangioma
Children Scrofuloderma (neck), tubercular chancre (face), noma (face)
Adults Leishmaniasis, granulomatosis with polyangiitis
Elderly Basal cell carcinoma, squamous cell carcinoma, herpes zoster,
angiosarcoma, temporal arteritis
Lower back Elderly Decubitus ulcer
Abdomen Kangri ulcer, warfarin-induced skin necrosis, calciphylaxis
Extremities Children Tubercular chancre, Ecthyma
Adults • Anywhere: Nicolau syndrome (history of intramuscular injections),
tropical ulcer
• Upper extremity: Swimming pool granuloma, sporotrichosis,
cutaneous anthrax
• Thighs: cholesterol emboli, calciphylaxis, kangri ulcer (inner thighs,
history of use of kangri basket)
• Lower leg: Livedoid vasculopathy, cryoglobulinemic vasculitis,
cutaneous polyarteritis nodosa, venous ulcer (mid-calf), Martorell ulcer
• Foot: Trench foot
• Distal toes: Thromboangitis obliterans, Raynaud’s phenomenon,
Frostbite, arterial ulcer (punched out ulcers)
• Sole (pressure areas): Neuropathic ulcer
History of exposure to cold Trench foot, frostbite, Raynaud’s phenomenon
Genitalia Single, painless Syphilis, squamous cell carcinoma (ulcerated tumor)
Single, painful Trauma, fixed drug eruption
Multiple, painful Herpes genitalis, chancroid, aphthous ulcer, cutaneous Crohn’s disease
Variable, granulation tissue Donovanosis
Associated with cutaneous Pemphigus vulgaris
blisters and ulcers
Anywhere Pyoderma gangrenosum, necrotizing fasciitis

●● At birth, 15–30% cases may have superficially eroded to ●● The exact causative organism is not clear, although
a deeply ulcerated scalp (Figure 13.1). spirochetes and fusiform bacillus in symbiosis have been
●● Large defects of the scalp or scalp defects that involve the considered to be the primary cause of the condition.
underlying skull or dura, with exposure of sagittal sinus, ●● Early features include soreness of the mouth,
large veins, or brain, may require surgical intervention due pronounced halitosis, fetid taste, tenderness of the lip
to the high risk of infection, hemorrhage, or thrombosis. or cheek, cervical lymphadenopathy, a foul-smelling
purulent oral discharge, and a blue-black discoloration
Ulcerated hemangioma of the skin in the affected area.
●● Ulceration, the most frequent complication of infantile
●● Without appropriate treatment, the mortality rate is
reported to be 70–90%, and surviving patients suffer
hemangioma (IH), tends to heal poorly and is associated
with functional disturbances and disfigurement.
with pain, bleeding, infection, and scarring.
●● Ulceration is more common in centrofacial and
●● DD: Pseudomonas infection, leishmaniasis.
perineum regions and may be heralded by surfacing of
blackish spots or white discoloration (Figure 13.2). Tuberculous chancre
●● Ulcerated IHs tend to be larger than non-ulcerated ones, ●● Tuberculous chancre, also known as primary
and friction, low birth weight, prematurity, and female tuberculous inoculation chancre, is a rare form of
gender are risk factors associated with ulceration.9 tuberculosis.
●● It develops in individuals not previously sensitized
Noma/cancrum oris10 to mycobacterium, occurring most commonly in
●● Noma is a rapidly spreading mutilating gangrenous children, especially those who have not taken the Bacilli
stomatitis caused by normal oral flora that becomes Calmette-Guérin (BCG) vaccine.
pathogenic during periods of compromised immune ●● It has also been reported in surgical wounds, tattoos

status (measles, AIDS, tuberculosis). and piercing sites, even sexual transmission.
 Ulcer: Single or few  295

●● It is commonly seen in children, the face and

extremities being the most frequently involved sites.
●● It is characterized by the appearance at the first site
of inoculation, two to four weeks post contact, of a
shallow, painless ulcer, with granular base with micro-
abscesses or thick crust and undermined borders.11
●● Painful regional lymphadenopathy is present.
●● Spontaneous regression with scarring and regional lymph
node calcification may occur, or the patient may develop
lupus vulgaris lesions and tuberculosis verrucosa cutis.
●● It needs to be differentiated from tubercular gummas.
●● DD: Sporotrichosis, leishmaniasis, atypical mycobacteriosis,
syphilis, cat scratch disease, and tularemia.
Tuberculous gumma
●● Tuberculous gumma, also known as metastatic

tuberculous abscesses, is an unusual form of cutaneous TB.

●● It has generally been observed in malnourished

children and severely immunosuppressed adults, but its

occurrence in immunocompetent individuals is possible.
●● It results from hematogenous spread of the mycobacterium

from a primary focus during a period of impaired immunity.

●● The lesions of tuberculous gumma are typically firm,

single or multiple non-tender erythematous nodules.

●● Occasionally, the lesions break down and discharge

their contents or persist and form ulcers and sinuses

(Figure 13.3a).
●● Limbs are the most frequent site for gummas, especially

Figure 13.1  Aplasia cutis congenita – large defect in scalp thighs and buttocks.
with visible underlying structures. (Courtesy: Dr Rashid ●● DD: Syphilis gumma, pyoderma gangrenosum, atypical
Shahid, AIIMS Patna, India.) mycobacterial lesions.
Scrofuloderma11
●● Scrofuloderma is a common form of cutaneous

tuberculosis caused by a lesion in the lymph node, bone,

muscle, or tendon that contiguously spreads to the skin.
●● Regions most commonly affected include chest, neck,

and axillary lymph nodes.

●● It begins as painless, subcutaneous nodules, which are

called cold abscesses.

●● The reddish-blue induration suppurates and breaks

down, forming an ulcer with granulation tissue at the

base (Figure 13.3b,c).
●● Sometimes sinuses may develop and communicate

directly with areas of deep infection.

●● Progression and scarring produce irregular adherent

masses, densely fibrous in places and fluctuant or

discharging in others.
●● DD: Atypical mycobacterial infection, sarcoidosis,

blastomycosis, and tertiary syphilis.

Cutaneous leishmaniasis
●● Cutaneous leishmaniasis occurs in the New World and

the Old World. Old World disease primarily is caused

by Leishmania tropica in urban areas and Leishmania
major in dry desert areas.
Figure 13.2  Ulcerated infantile hemangioma. (Courtesy: ●● The disease begins as an erythematous papule at the site of

Dr. Piyush Kumar, Katihar, India.) the sandfly bite on exposed parts of the body, usually the face.
296  Ulcer: Single or few

Figure 13.3  (a) Tubercular gumma presenting as solitary ulcer with undermined edge and violaceous indurated margin.
(b) Multiple ulcers with undermined edges and violaceous margin in scrofuloderma. (c) Scrofuloderma with lupus vulgaris.
(a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● The papule increases in size and becomes a nodule. ●● The hallmarks of GPA are systemic necrotizing
●● It eventually ulcerates and crusts over. The border is vasculitis, necrotizing granulomatous inflammation,
usually raised and distinct (Figure 13.4). and necrotizing glomerulonephritis.
●● There may be multiple lesions, especially when the ●● It can involve virtually any organ in the body as
patient has encountered a nest of sandflies. generalized disease or as a limited/localized disease.
●● The ulcer is typically large but painless unless there is ●● Cutaneous vasculitis secondary to GPA can present as
secondary bacterial or fungal infection, and it heals papules, nodules, palpable purpura, ulcers resembling
with scarring. pyoderma gangrenosum, or necrotizing lesions leading
to gangrene. Cutaneous lesions are found in 50% of
Granulomatosis with polyangiitis patients.
●● Initially known as Wegener’s granulomatosis, ●● They are typically located on the lower extremities, but
granulomatosis with polyangiitis (GPA) is a necrotizing they can also manifest on the face, upper extremities,
granulomatous inflammation usually involving the and the extensor surfaces of the joints. Oral and nasal
upper and lower respiratory tract and necrotizing ulcerations may also occur.
vasculitis affecting predominantly small to medium ●● DD: Pyoderma gangrenosum, mycobacterial infection,
vessels. deep fungal infections.
 Ulcer: Single or few  297

Figure 13.4  Ulcerated plaque covered by crusts in cutane-

ous leishmaniasis. (Courtesy: Dr. Md Irfan Anwar, Pakistan.)

Basal cell carcinoma

●● Basal cell carcinoma (BCC) typically presents as a shiny,

pink or flesh-colored papule or nodule with surface

telangiectasia.
●● The tumor may enlarge and ulcerate, with rolled waxy

borders (Figure 13.5a,b).

●● The most common sites for nodular BCC are the face,

especially the nose, cheeks, forehead, nasolabial folds,

and eyelids.
●● Patients often give a history of crusting and recurrent

bleeding, causing them to seek evaluation.

●● Pigmented nodular BCCs are more common in dark-

skinned individuals.
Squamous cell carcinoma12
●● Cutaneous squamous cell carcinoma (SCC) is a non-

melanoma skin cancer that arises from epithelial

keratinocytes or their appendages.
●● It may arise de novo, as a result of previous exposure to

ultraviolet (UV) ionizing radiation or arsenic; within

chronic wounds or scars; or from pre-existing lesions,
such as actinic keratosis, Bowen’s disease (intraepidermal
SCC), bowenoid papulosis, or erythroplasia of Queyrat.
●● Risk factors include sun exposure, old age, fair skin, and

immunosuppression.
●● It is locally aggressive and, without treatment, has the

potential to metastasize to other parts of the body.

●● Most SCCs arise on the sun-damaged skin of the head

and neck, with fewer lesions arising on the extremities

and occasional tumors occurring on the trunk.
●● Early lesions frequently present as red scaly papules.
●● Later, lesions may form nodules or firm plaques, either

of which can ulcerate (Figure 13.6a,b). Figure 13.5  (a) Basal cell carcinoma causing destruction
●● SCC may also present as a non-healing ulcer of left ala nasi and ulceration. (b) Ulcerated basal cell
(Figure 13.6c–e). carcinoma. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
298  Ulcer: Single or few

Figure 13.6  (a) Ulcerated nodule of squamous cell

carcinoma on the scalp. (b) Squamous cell carcinoma on
the area of previous burn (Marjolin ulcer). (c) Squamous
cell carcinoma presenting as non-healing ulcer on
buccal mucosa. (d) Non-healing ulcer of squamous cell
carcinoma on the neck in a patient with epidermodysplasia
verruciformis. (Continued)
 Ulcer: Single or few  299

Figure 13.7  Ulcer and crusts in dermatomal distribution in

herpes zoster. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● It is more common in older individuals, with a reported

median age between 60 and 71 years; cutaneous
angiosarcomas are more prevalent in males.
●● The cutaneous form is the most common, accounting
Figure 13.6 (Continued)  (e) Squamous cell carcinoma for about half of all tumors, with the head and neck
presenting as non-healing ulcer and large noduloulcer-
being the most frequently involved region. Specifically,
ative lesions in a patient with oculocutaneous albinism.
(a,b – Courtesy: Dr. PC Das, Katihar, India; c,d – Courtesy: the scalp accounts for approximately 50% of all
Dr. Piyush Kumar, Katihar, India; e – Courtesy: Dr Hiral angiosarcoma cases.
Shah, Baroda Medical College, Vadodara, India.) ●● Risk factors include chronic lymphoedema and
radiation therapy.
●● Patients often present with skin lesions such as an
Herpes zoster enlarging bruise, a discolored nodule, or persistent
●● Ulcerations in herpes zoster are small and grouped ulceration.
in a linear fashion along a dermatome. They are ●● In the early stages, these lesions can be misdiagnosed
associated with significant pain and heal with scarring for benign entities caused by cellulitis, infection, or skin
(Figure 13.7). injuries.
●● DD: Hemangioblastoma, Kaposi sarcoma, metastatic
Temporal arteritis cancer from an unknown primary site, sinonasal
●● Temporal arteritis is a chronic granulomatous vasculitis squamous-cell cancer and Merkel cell carcinoma.
affecting medium-large cell vessels (external cranial
branches of the aorta). Decubitus ulcer
●● The incidence of temporal arteritis increases with age, ●● Decubitus ulcers, also termed bedsores or pressure ulcers,

almost exclusively affecting people 50 years of age or are skin and soft-tissue injuries that form as a result of
older. Women account for 65–75% of patients. constant or prolonged pressure exerted on the skin.
●● Typical clinical features include new-onset headache, ●● They occur on bony areas of the body such as the

scalp tenderness, jaw claudication, fever, fatigue, ischium, greater trochanter, sacrum, heel, malleolus
malaise, anorexia, weight loss, and polymyalgia. (lateral than medial), and occiput.
●● Rarely, patients may present with scalp necrosis. ●● Etiology

●● External factors: pressure, friction, shear force, and

Angiosarcoma13 moisture
●● Angiosarcoma is an aggressive, malignant endothelial- ●● Internal factors: fever, malnutrition, anemia, and
cell tumor of lymphatic or vascular origin. It has a high endothelial dysfunction speed up the process of
rate of local recurrence and metastasis. these lesions
300  Ulcer: Single or few

●● Clinical feature depends on stage of ulcer Ecthyma

●● Stage I describes intact skin with non-blanchable ●● Ecthyma is a unique and more invasive form of
erythema. skin group A Streptococcus infection. In contrast
●● Stage II pressure ulcers have partial-thickness skin to impetigo, it involves deeper skin tissues, is more
damage with possible blister formation but no painful, and frequently leaves a prominent scar after
subcutaneous tissues visible. healing.
●● Stage III pressure ulcers have full-thickness skin ●● Ecthyma usually arises on the lower extremities of
loss with subcutaneous fat exposed but no muscles, children, persons with diabetes, and neglected elderly
bones, or tendons visible. patients.
●● Stage IV pressure ulcers have tissue loss with ●● It begins as a vesicle or pustule overlying an inflamed
exposure of muscles, bones, tendons, or vital area of skin that deepens into a dermal ulceration
organs. with an overlying crust. The crust of ecthyma lesions
●● A common hallmark of pressure ulcers is that the area is gray-yellow and is thicker and harder than the crust
of skin affected typically underrepresents the amount of of impetigo. A shallow, punched-out ulceration is
subcutaneous tissue involved. apparent when adherent crust is removed. The deep
●● An unstageable pressure ulcer refers to a wound with an dermal ulcer has a raised and indurated surrounding
undetermined level of tissue injury because the entire margin (Figure 13.8a,b).
base of the wound is covered by slough tissue and/or ●● DD: Ecthyma gangrenosum, pyoderma gangrenosum,
eschar.14 insect bite.
Kangri ulcer15
●● This is a squamous cell carcinoma (SCC) that occurs

due to keeping lighted coal in a kangri basket (used in

remote hilly regions of Kashmir) close to the skin in
winter to keep warm.
●● The kangri basket, which holds burning coal and dried

chinar (Platanus orientalis) leaves, is held between the

thighs or over the lower abdomen for prolonged periods
of 12–18 hours a day.
●● Early changes include dyspigmentation, erythema ab

igne, induration, and thickening over the medial aspect

of upper third of the thighs.
●● Malignant transformation is heralded by the

development of exophytic growths and ulcers, which

often get secondarily infected.

Calciphylaxis
●● Calciphylaxis has been classically described as the

sudden development of tender, violaceous, and

reticulate lesions that progress to necrotic ulcerations
of the lower extremities in patients with renal failure on
chronic hemodialysis.
●● These ulcers heal poorly and are very painful.

●● Areas commonly affected are those with thick

adipose tissue, such as the abdomen, breasts, and

buttocks.
●● Peripheral pulses are preserved, since only the smaller

vessels are affected by calciphylaxis.

●● There is often an elevated parathyroid (PTH) level

(secondary to renal failure), with abnormal calcium and

phosphate metabolism.
●● Other risk factors include obesity, liver disease, systemic

corticosteroid use, and an elevated calcium phosphorus

product. In addition, contributing factors are diabetes
mellitus, warfarin use, decreased functional protein Figure 13.8  (a) Ulcer of ecthyma with indurated margin.
C and S activity, vitamin D supplementation, and low (b) Crusted ulcers in ecthyma. (a,b – Courtesy: Dr. Piyush
albumin levels. Kumar, Katihar, India.)
 Ulcer: Single or few  301

Swimming pool granuloma nodule that develops, often within days, at the site of
●● Also known as fish tank granuloma, this is caused by inoculation (Figure 13.9a).
M. marinum infection. ●● Progressive noduloulcerative lesions following the
●● It is most commonly seen in fishers, oyster workers, underlying lymphatics then arise proximally.
swimmers, and aquarium workers, and usually involves ●● DD: Cutaneous leishmaniasis, nocardiosis.
the dominant hand. Cutaneous anthrax
●● The infection starts as a solitary papule or nodule,
●● Anthrax is a zoonotic disease, and humans are an
which grows slowly over several months. Induration
accidental host. It is caused by Bacillus anthracis, a
and ulceration occur later.
sporulating Gram-positive rod.
●● Sporotrichoid spread and lymphangitis occur
●● The initial manifestation is of a painless pruritic papule
subsequently.
that gradually enlarges over a period of seven to ten
●● DD: Lupus vulgaris, sporotrichosis.
days. A serosanguinous discharge may be present.
Sporotrichosis ●● Smaller vesicles may be present in the surrounding

●● Clinically, sporotrichosis typically manifests as a area, and perilesional edema is characteristic and often
lymphocutaneous eruption, beginning as a papule or disproportionate to the size of the lesion (Figure 13.9b).

   

Figure 13.9  (a) Ulcerated plaque with erythematous nodules appearing proximally along the lymphatics in a case with
ulcerated sporotrichosis. (b) Ulcer surrounded by rim of pus in cutaneous anthrax. (a – Courtesy: Dr. Dependra Kumar
Timshina, Remedy Clinics, Siliguri, India; b – Courtesy: Dr. Rajesh Kumar Mandal, North Bengal Medical College and
Hospital, Darjeeling, India.)
302  Ulcer: Single or few

The lesion develops a central depression followed by Raynaud’s phenomenon

eschar formation and heals within one to three weeks, ●● Raynaud’s phenomenon is classically described with a
with variable scarring. triphasic color change of the digits with initial white
●● More than 90% of the lesions are found on the or pallor (ischemic phase), then blue or cyanosis
exposed parts of the body such as the face, neck, (deoxygenation phase), followed by red or erythema
hands, and feet. (reperfusion phase).
●● DD: Staphylococcal skin abscess, cat scratch ●● Patients with secondary Raynaud’s will describe attacks
disease, tularemia, spider bite, and ecthyma that are more frequent, painful, often asymmetric and
gangrenosum. may lead to digital ulcerations.
●● These ulcerations can resolve to leave scars or digital

Frostbite pits or may progress to more severe digital necrosis,

●● Frostbite is a freezing, cold thermal injury that occurs gangrene, or auto-amputation (Figure 13.10).
●● Digital ulcerations can become secondarily infected,
when tissues are exposed to temperatures below their
freezing point (typically −0.55°C but can occur as high leading to osteomyelitis, and can require surgical
as 2°C) for a sustained period of time. intervention.
●● There should be no evidence of tissue gangrene, digital
●● It is mainly due to the formation of ice in both

intracellular and extracellular compartments. pitting, or tissue injury in primary Raynaud’s.

●● It is associated with the following:
●● It is mostly seen in soldiers, those engaged in winter

sports, homeless people, etc. ●● Connective tissue diseases such as scleroderma,

●● Risk increases with alcohol use, smoking, and the lupus, or mixed connective tissue disease.
presence of peripheral vascular disease. ●● Hematologic causes such as cryoglobulinemia,
●● In 90% of cases, limbs are affected. It can also affect the cryofibrinogenemia, paraproteinemia, or
face (nose, chin, earlobes, cheeks, and lips), buttocks/ polycythemia.
perineum (from sitting on metal seats), and penis ●● Drug-induced conditions (ergot, methysergide,
(joggers and Nordic skiers). beta-blockers, bleomycin, cisplatin, clonidine,
●● Classification of severity cocaine, cyclosporine, interferon-alpha, nicotine,
●● 1st degree: partial skin freezing present, erythema, and vinblastine).
edema, hyperemia, but no blisters or necrosis ●● Other vasospastic causes such as
●● 2nd degree: full thickness freeze, erythema, edema, pheochromocytoma, carcinoid syndrome, or
blistering, black eschar thyroid disease.
●● 3rd degree: requires bone amputation
●● 4th degree: major amputation and complicated by
systemic effects
●● DD: Frostnip, trench foot, pernio.

Trench foot
●● Trench foot is one of three subclasses of immersion foot

and is considered a non-freezing cold injury.

●● It is often differentiated by the temperature of the

exposure and is caused by prolonged exposure to

cold but usually not freezing, damp, and sometimes
unsanitary conditions.
●● Trench foot often begins with a tingling and itching that

can progress to numbness.

●● In the setting of cold exposure, vascular changes

resulting in poor blood flow can result in the feet

becoming erythematous or cyanotic.
●● The disease has been known to commonly affect the

heels or toes but can extend above the foot.

●● The skin can appear blotchy, and as the disease

progresses, blisters and open sores can occur, which can

lead to fungal as well as bacterial infections.
●● As the disease continues to advance, skin and tissue

may slough off. If the condition is left untreated, Figure 13.10  Digital ulcer in systemic sclerosis with
gangrene can set in. Raynaud’s phenomenon. (Courtesy: Dr. Piyush Kumar,
●● DD: Frostbite, cellulitis. Katihar, India.)
 Ulcer: Single or few  303

●● Numerous structural causes that may include thoracic ●● Stasis eczema (erythema, scaling, weeping, and
outlet syndrome, atherosclerosis, brachiocephalic itching) is also common and is distinct from cellulitis.
trunk disease as in Takayasu’s arteritis, Buerger’s (See Chapter E11.)
disease or thromboangiitis obliterans, or use of
Martorell ulcer
vibratory tools such as jackhammers.
●● Martorell ulcer is an uncommon ischemic and
●● DD: Acrocyanosis, erythromelalgia.
extremely painful lesion located in the distal portion
Necrobiosis lipoidica diabeticorum/necrobiosis of the lower limb, resulting from severe systemic and
lipoidica poorly controlled hypertension. It is common in women
●● Necrobiosis lipoidica (NL) is characterized by single or between 50 and 70 years of age.
multiple asymptomatic red to yellow shiny plaques that ●● It is frequently symmetrical and located in the distal

gradually enlarge and within which, due to atrophy, third and anterolateral surfaces of the lower limbs.
dermal blood vessels are readily apparent. ●● Classically, the ulcer has variable depth, necrotic base,

●● Ulceration is a complication of NL and commonly and violaceous edges. The presence of satellite lesions
develops after trauma to the lesions (Figure 13.11). and irregular edges are also features that suggest
●● Majority of patients have diabetes mellitus, or pre-diabetes. additional cutaneous ischemia.
●● Pain in greater intensity than in ulcers of other
Venous ulcer etiologies, referred to as being disproportionate to the
●● Ninety-five per cent of venous ulceration is in the gaiter size of the lesion, is the most relevant symptom.
area of the leg, characteristically around the malleoli. ●● DD: Venous ulcer, traumatic ulcer, pyoderma
●● Ulceration may be discrete or circumferential. The ulcer gangrenosum.
bed is often covered with a fibrinous layer mixed with
granulation tissue, surrounded by an irregular, gently Nicolau syndrome
sloping edge. ●● Nicolau syndrome (embolia cutis medicamentosa) is a

●● Ulcers occurring above the mid-calf or on the foot are rare cutaneous adverse reaction occurring at the site of
likely to have other origins. intramuscular, intra-articular or, rarely, subcutaneous
●● Lipodermatosclerosis often precedes venous ulceration injection of particular drugs.
and is characterized by rigid woody hardness that, at ●● Three factors have been proposed to play a final

its worst, may result in the leg resembling an “inverted role in the pathogenesis: embolism (of the injected
champagne bottle.” material), angiospasm (secondary to needle prick),
and thrombosis. Mechanical pressure exerted by the
material placed around the vessel too might play a role.
●● The typical clinical presentation is pain around the

injection site soon after injection, followed by erythema,

livedoid patch, hemorrhagic patch, and finally necrosis
of skin, subcutaneous fat, and muscle tissue.
Tropical ulcer
●● Tropical ulcers result from diseases transmitted

by mosquitoes and flies in tropical and subtropical

countries where the weather is hot and humid.
●● Predisposing factors include poor hygiene, low

socioeconomic conditions, poor nutrition, poverty, and

lack of protection from insect bites.
●● Superinfection with Fusobacterium species, anaerobic

microorganisms (Bacillus fusiformis) and spirochetes

(Treponema vincenti) aggravates these ulcers and delays
healing.
●● The lesion first appears as a small papule on the lower

leg, usually at the site of trauma.

●● The ulcer then spreads rapidly, forms a blister, and undergoes

necrosis, which is associated with pain, fever, and malaise.

●● Within a few weeks the ulcer enlarges to 10–12 cm,

and a superficial, necrotic, malodorous, purulent,

hemorrhagic black coating develops (Figure 13.12a,b).
●● After three to four weeks, the edge becomes flatter, and

Figure 13.11  Crusted ulcer in necrobiosis lipoidica. the swelling and pain decrease.
(Courtesy: Dr. Sushil S Savant, Mumbai, India.) ●● DD: Buruli ulcer, pyoderma gangrenosum.
304  Ulcer: Single or few

●● As the disease progresses, typical calf claudication

and eventually ischemic pain at rest and ischemic
ulcerations on the toes, feet, or fingers may develop.
●● Raynaud’s phenomenon is present in greater than 40%
of patients with thromboangiitis obliterans.
●● Superficial thrombophlebitis differentiates
thromboangiitis obliterans from other vasculitides and
atherosclerosis.

Arterial ulcer
●● Arterial ulceration typically occurs over the toes, heels,

and bony prominences of the foot.

●● The ulcer appears “punched out,” with well-demarcated

edges and a pale, non-granulating, often necrotic base.

●● The surrounding skin may exhibit dusky erythema and

may be cool to touch, hairless, thin, and brittle, with a

shiny texture.
●● The toenails thicken and become opaque and may be

lost. Gangrene of the extremities may also occur. (See

chapter E11.)

Pyoderma gangrenosum
●● Pyoderma gangrenosum (PG) is a reactive non-

infectious inflammatory dermatosis falling within

the spectrum of the neutrophilic dermatoses and
characterized by chronic and recurrent cutaneous ulcers
with a necrolytic border.
●● It is typically seen in adults, with peak occurrence

between the ages of 30 and 50 years and with slight

predilection for females.
●● Etiology is unknown. Risk factors include female

gender, pregnancy, surgery, and a history of underlying

disease such as inflammatory bowel disease or
hematologic malignancy.
●● The lower legs are most frequently affected, although

PG can affect any site. The lesion may be precipitated by

minor trauma, a phenomenon known as “pathergy.”
●● Lesions in classical PG, also known as ulcerative PG,

present as painful small erythematous or violaceous

papules or pustules, which evolve into violaceous ulcers
or necrotic plaques.
●● One of the hallmark features of classic PG is the presence of
Figure 13.12  (a) Tropical ulcer. (b) Large tropical ulcer. an undermined or raised border with a characteristic gun
(a – Courtesy: Dr. PC Das, Katihar, India; b – Courtesy: metal gray or violaceous to blue color (Figure 13.13a–c).
Dr. Piyush Kumar, Katihar, India.) Lesions heal with cribriform scarring.16
●● DD: Arterial and venous disease, hematological causes
Thromboangiitis obliterans (sickle cell disease, cryoglobulinemia, antiphospholipid
●● Thromboangiitis obliterans (TAO), also known as syndrome), vascular occlusion, vasculitis, infections,
Buerger’s disease, is a progressive, non-atherosclerotic, calciphylaxis, drug-induced ulceration, primary or
segmental, inflammatory disease that most often affects metastatic tumors, hypertension (Martorell ulcer) and
small and medium arteries of the upper and lower other inflammatory disorders, including cutaneous
extremities. Crohn’s disease.
●● Risk factors include male gender, and smoking.

●● Claudication in the arch of the foot is an early sign and Necrotizing fasciitis17
is suggestive of, or even specific to, TAO. This condition ●● Necrotizing fasciitis (NF) is a severe, rare, potentially

is a manifestation of infrapopliteal vessel occlusive lethal soft-tissue infection that develops in the scrotum
disease. and perineum, the abdominal wall, or the extremities.
 Ulcer: Single or few  305

●● Responsible pathogens are as follows:

●● Type 1: Polymicrobial (obligate and facultative
anaerobes)
●● Type 2: Beta-hemolytic Streptococcus
●● Type 3: Clostridium, gram negative bacteria,
Aeromonas, Vibrio
●● Type 4:Candida, Zygomycetes
●● Risk factors include diabetes mellitus (the most
frequent comorbidity). Other common comorbidities
include liver cirrhosis, chronic heart failure, obesity,
alcohol abuse, immunodeficiency, systemic lupus
erythematosus, Addison’s disease, pre-existing
hypertension, and peripheral vascular disease.
●● Patients with NF usually present with the classic
triad of symptoms: local pain, swelling, and
erythema. Tachycardia (>100 beats/min) and fever
are the most common vital sign abnormalities,
followed by hypotension (SAP < 100 mmHg) and
tachypnea (>20/min).
●● However, in the fulminant form of disease, the patient
is critically ill, with signs and symptoms of severe septic
shock and multiple organ dysfunction syndrome along
with extensive necrosis of soft tissue.
●● The skin lesion starts with painful edema and
erythema. The condition progresses rapidly, and
gangrene sets in. The skin becomes dark and
ulcerates with eschar formation. The pain subsides
after ulceration (Figure 13.14).

Figure 13.13  (a) Ulcer of pyoderma gangrenosum with

raised, violaceous margin. (b) Pyoderma gangrenosum.
(c) Pyoderma gangrenosum progresses rapidly and may
assume a large size. (a,c – Courtesy Dr. Dependra Kumar
Timshina, Remedy Clinics, Siliguri, India; b – Courtesy
Dr. Piyush Kumar, Katihar, India.)

●● Trauma is the most common identifiable etiology. The

majority of patients have a history of minor or major
traumas, generally involving external injuries and Figure 13.14  Necrotizing fasciitis affecting anterior chest
surgical wounds. and left breast. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
306  Ulcer: Single or few

●● Gas formation can lead to crepitus in the overlying inhibition of vitamin K–dependent coagulation factors
skin, indicating anaerobic infection, such as relative to other factors, thus producing a transient
C. perfringens. imbalance that predisposes to clotting.
●● Systemic features include hypotension, elevated white ●● Protein C deficiency, protein S deficiency, and
blood cell count, metabolic acidosis, coagulopathy, antithrombin III deficiency are considered risk factors
changes in mental status, and weakness. in the development of WISN.
●● DD: Cellulitis. ●● DD: Heparin-induced necrosis associated with heparin-
induced thrombocytopenia and thrombosis (HITT).
Syphilitic gumma
●● Cutaneous gummas of tertiary syphilis are dermal or Chemotherapy agents and ulcer18
subcutaneous nodules that have a gummy or rubbery ●● The antineoplatic agents commonly associated with

consistency. ulcers are mentioned below.

●● They can occur singly or form grouped, annular or ●● Inhibitors of epidermal growth actor receptors (EGFR):

serpiginous lesions that have predilection for the scalp, Geftinib (pyoderma gangrenosum-like ulcer), Sorafenib.
face, chest, legs, and sites of trauma. ●● Angiogenesis inhibitor: Sorafenib, Sunitinib (pyoderma

●● They are painless and frequently ulcerate. gangrenosum like ulcer), Bevacizumab (ulcerations over
striae distensae).
Ecthyma gangrenosum ●● BCR-ABL, c-KIT, PDGFR inhibitors: Imatinib (oral ulcers).
●● Ecthyma gangrenosum is a rare and invasive cutaneous ●● m-TOR inhibitor: Sirolimus (aphthous ulcer).

infection caused by Pseudomonas aeruginosa in the ●● Antimetabolites: Methotrexate (dose-dependent

majority of cases, typically affecting immunocompromised ulceration of psoriatic plaques, Mucositis) (Figure 13.16).
patients, particularly those with neutropenia. ●● Antiproliferative: Hydroxyurea (perimalleolar and
●● The characteristic clinical appearance is red macules pretibial ulcers have been described).
that progress to a central area of necrosis surrounded by
an erythematous halo (Figure 13.15). Buruli ulcer (Bairnsdale ulcer)19
●● The main sites of ecthyma gangrenosum lesions are ●● This is caused by M. ulcerans.

the extremities and gluteal or perineal region (57%), ●● It grows best at low oxygen concentrations and is found

although this lesion can spread to other body sites. in muddy or marshy ground.
●● Classically, the pathogen is isolated from the skin ●● It causes necrotizing ulcers in otherwise healthy people.

lesions as well as from the blood. ●● The disease begins as indurated nodules that look like

●● DD: Calciphylaxis, septic emboli, cutaneous anthrax, insect bites but are painless and cold (not inflamed).
cutaneous aspergillosis, and pyoderma gangrenosum. ●● Within weeks, these lesions grow in diameter and depth,

producing edematous plaques with ill-defined borders.

Warfarin-induced skin necrosis
●● Sudden development of paresthesia, edema, petechiae,

and ecchymoses progressing to full-thickness necrosis

and deep ulcers within three to ten days after the
initiation of warfarin therapy should alert the clinician to
the possibility of warfarin-induced skin necrosis (WISN).
●● WISN has been described in patients on

anticoagulation, typically several days after therapy is

stopped or on retreatment. It is thought to be the result
of a paradoxical hypercoagulable state, possibly due to

Figure 13.15  Ecthyma gangrenosum with central ulcer

and eschar surrounded by erythema. (Courtesy: Dr. Piyush Figure 13.16  Erosive mucositis following methotrexate
Kumar, Katihar, India.) toxicity. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Ulcer: Single or few  307

●● On occasion there is no nodule or plaque and only ●● Systemic symptoms include headache, malaise, and
diffuse swelling. fever.
●● Eventually the indurated, necrotic skin sloughs off, ●● After a few weeks, metastatic lesions begin to appear,
creating a painless ulcer with irregular borders and which are grouped, dull red papules, pustules or bullae
characteristic undermined edges. especially over joints and face. These are followed by
●● The surrounding skin can show edema and changes in ulcers that enlarge and coalesce.
pigmentation. ●● Deep subcutaneous abscesses with multiple sinuses may
●● Most of the time there is a single lesion; however, small also occur.
satellite lesions can be present. In many instances the ●● DD: Melioidosis, pyoderma.
ulcer acquires secondary bacterial infections, giving it a
foul smell. Myiasis
●● DD: Pyoderma gangrenosum, metastatic ulcer. ●● Myiasis is an infestation of body tissues by larvae

(maggots) of the order Diptera.

Tularaemia ●● There are three types of cutaneous myiasis: furuncular,

●● It results from infection with Francisella tularensis, a traumatic (wound), migratory (creeping).
gram-negative bacteria. ●● Wound myiasis occurs as a complication of war wounds

●● Wild rodents and other small animals are the main or neglected open wounds (Figure 13.17).
reservoir of infection, which is transmitted to humans ●● Risk factors include the following:
by bites of ticks, usually of dermacentor species. – Wounds with alkaline discharges (attract blow
●● It occurs in all ages and affects males and females flies)
equally. – Presence of necrosis
●● Clinical features depend on portal of entry, which may – Lack of hygiene
be skin, eye, or respiratory and GI tract. – Low socioeconomic status
●● A red painful and then ulcerated nodule at point of – Extremes of age, mental retardation, diabetes,
inoculation or tick bite is associated with enlargement and alcoholism, vascular occlusive disorders
tenderness and later breakdown of regional lymph nodes. ●● Furuncular myiasis results from penetration of larva

●● During the toxemic stage of all forms, cutaneous lesions into healthy skin.
may develop; a generalized eruption, maculopapular ●● Caused by Dermatobia hominis, and Cordylobia
or resembling erythema multiforme, profuse crops of anthropophaga.
nodules, usually on limbs may occur. ●● It has a papule/nodule with a central punctum
●● DD: Tick-borne rickettsial infections. exuding serosanguinous or purulent fluid.
●● The clinical diagnosis rests on visualizing the
Cat scratch disease respiratory spiracle of larva through the punctum
●● Caused by Bartonella henselae, it usually develops after or presence of bubbles in the discharge.
a cat scratch or bite. ●● Pruritus, pain, movement sensation may be present.
●● It affects all ages but mostly children and teenagers; it ●● Vesicular, bullous, pustular, erosive, ulcerative, or
affects both genders. ecchymotic lesions are uncommon.
●● The main clinical feature is that three to five days after

inoculation a papule may form that progresses through

vesicular and blistering stages in two or three days and may
ulcerate and often leaves a superficial scar (90% of cases).
●● Constitutional symptoms are usually mild, but fever is

present in 60% of cases.

●● Lymphadenopathy is present in all cases and usually

develops within two weeks of the initial papule; it is

solitary in 85% of cases but two or three are seen in
occasional cases.
●● DD: Sporotrichosis, atypical mycobacterial infections.

Glanders disease
●● Also called farcy, Glanders is contacted from affected

horses, donkeys, mules, and goats.

●● Caused by Burkholderia mallei, it is extremely rare

nowadays and affects both sexes and any age.

●● It is characterized by cellulitis at the site of inoculation,
Figure 13.17  Myiasis complicating a traumatic wound in
which soon develops into an irregular ulcer with an a leprosy patient. (Courtesy: Dr. Piyush Kumar, Katihar,
offensive hemorrhagic purulent discharge. India.)
308  Ulcer: Single or few

Cutaneous Crohn disease

●● Cutaneous lesions in Crohn disease could be specific

(showing histopathology findings consistent with

Crohn disease) or reactive (pyoderma gangrenosum,
Sweet syndrome, etc). These cutaneous lesions are often
associated with systemic features like abdominal pain,
diarrhea, melena, malnutrition and weight loss, fever,
and bowel obstruction.
●● Specific lesions in Crohn disease may result from direct

extension from gastrointestinal Crohn disease or may

be metastatic. Cutaneous lesions resulting from direct
extension are noted in perianal and orofacial areas.
●● Cutaneous lesions developing after direct extension

include perianal fissures and fistulae, abscess, and

painful ulcers (Figure 13.18).
●● Metastatic lesions assume the morphology of red/purple

plaques and nodules and may ulcerate. Figure 13.18  Classical “knife cut” ulcers in Crohn disease.
(Courtesy: Dr Shekhar Neema, Armed Forces Medical
Additional images – Figure 13.19a,b College, Pune, India.)

  

Figure 13.19  (a) Erythema, edema, hemorrhagic

necrosis and ulcer in mucormycosis. (b) Ulcerated lesion
in mucormycosis. (a – Courtesy: Dr. Raghuraj Hegde,
Kempegowda Institute of Medical Sciences, Bangalore,
India; b – Courtesy: Dr. Safiqul Islam, School of Tropical
Medicine, Kolkata, India.)
 Ulcer: Single or few  309

REFERENCES 11. Dias MF, Bernardes Filho F, Quaresma MV, Nascimento LV, Nery
JA, Azulay DR. Update on cutaneous tuberculosis. An Bras
1. Lipsker D. Clinical Examination and Differential Diagnosis of Skin Dermatol 2014;89(6):925–938.
Lesions. 1st edition. France: Springer-Verlag; 2013. Chapter 42. 12. Garcia-Zuazaga J, Olbricht SM. Cutaneous squamous cell carci-
Erosions and Ulcerations. P. 219–224. noma. Adv Dermatol 2008;24:33–57.
2. Fitzpatrick JE, High WA, Kyle WL. Urgent Care Dermatology: 13. Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A,
Symptom-Based Diagnosis. 1st ed. Philadelphia: Elsevier; 2018. Broering G, Elsayes KM. Angiosarcoma: Clinical and imaging fea-
Chapter 14. Necrotic and Ulcerative Skin Disorders. P. 231–252. tures from head to toe. Br J Radiol 2017;90(1075):20170039.
3. Marks Jr JG, Miller JJ. Lookingbill and Marks’ Principles of 14. Boyko TV, Longaker MT, Yang GP. Review of the current man-
Dermatology. 6th edition. Philadelphia: Elsevier; 2019. Chapter 19. agement of pressure ulcers. Adv Wound Care (New Rochelle)
Ulcers. P. 257–261. 2018;7(2):57–67.
4. Singer AJ, Tassiopoulos A, Kirsner RS. Evaluation and management 15. Gupta D, Thappa DM. Dermatoses due to Indian cultural practices.
of lower-extremity ulcers. N Engl J Med 2017;377(16):1559–1567. Indian J Dermatol 2015;60:3–12.
5. Roett MA, Mayor MT, Uduhiri KA. Diagnosis and management of 16. Ahn C, Negus D, Huang W. Pyoderma gangrenosum: A review
genital ulcers. Am Fam Physician 2012;85(3):254–262. of pathogenesis and treatment. Expert Rev Clin Immunol
6. Hoffman MD. Atypical ulcers. Dermatol Ther 2013;26(3):222–235. 2018;14(3):225–233.
7. Janowska A, Dini V, Oranges T, Iannone M, Loggini B, Romanelli 17. Misiakos EP, Bagias G, Patapis P, Sotiropoulos D, Kanavidis P,
M. Atypical ulcers: Diagnosis and management. Clin Interv Aging Machairas A. Current concepts in the management of necrotizing
2019;14:2137–2143. fasciitis. Front Surg 2014;1:36.
8. Hoffman MD. Inflammatory ulcers. Clin Dermatol 2007;25(1):131–138. 18. D’Epiro S, Salvi M, Luzi A, Mattozzi C, Luci C, Macaluso L,
9. Chang CS, Kang GC. Efficacious healing of ulcerated infantile hem- Marzocca F, Salvo V, Cantisani C, Paolino G, Calvieri S, Richetta
angiomas using topical timolol. Plast Reconstr Surg Glob Open AG. Drug cutaneous side effect: Focus on skin ulceration. Clin Ter
2016;4(2):e621. 2014;165(4):e323–9.
10. Enwonwu CO, Falkler WA Jr, Phillips RS. Noma (cancrum oris). 19. Guarner J. Buruli ulcer: Review of a neglected skin mycobacterial
Lancet 2006;368(9530):147–156. disease. J Clin Microbiol 2018;56(4):e01507–17.
 E11
Leg ulcers

PIYUSH KUMAR, KOMAL AGARWAL

ABSTRACT
Lower-limb ulcers are a common presentation in the dermatology department. These ulcers are not only a cause of great con-
cern to the patient but also a diagnostic dilemma to the treating dermatologists. Given the wide array of causes and the often-
confusing presentation, the correct diagnosis of leg ulcers is often delayed. In order to address the above issue and for better
understanding, this chapter is presented in a lucid form focusing only on the relevant clinical presentations of various ulcers,
with due emphasis on the number, shape, site, pain, margin, and associated clinical features, if any.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

310 DOI: 10.1201/9781351054225-26

 E12
Ulcers: Multiple

PIYUSH KUMAR, RASHMI ROY

ABSTRACT
Skin ulceration is a major source of morbidity and is often difficult to manage. They can have various etiologies, such as
infections, inflammation, and malignancy, among others. Ulcers may be solitary or multiple. Multiple ulcers may indicate an
underlying systemic disease, and the physician must know the dermatological as well as systemic signs and symptoms so that
early intervention can be made. This chapter discusses the various causes and clinical features of important entities that can
present with multiple cutaneous ulcers.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-27 311

 E13
Scales: Localized

PC DAS

ABSTRACT
Scale is a secondary skin lesion. Many dermatoses may present with scales at some stage of the disease course, reflecting the
ongoing repair and remodeling in the skin. On the other hand, some other dermatoses have scales as the predominant skin
lesion. This chapter discusses common conditions presenting with scaling in a localized area.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

312 DOI: 10.1201/9781351054225-28

 E14
Scales: Generalized

AVIJIT MONDAL

ABSTRACT
Many dermatoses may develop scaling in a generalized distribution during the evolution or resolution of lesions. This chapter,
however, focuses on entities that present exclusively or predominantly with generalized scaling.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-29 313

 E15
Atrophy

CHIRAG DESAI, RASHID SHAHID

ABSTRACT
Atrophy of skin, a morphological entity described under secondary skin lesions, is a result of partial or significant loss of
epidermis, dermis, subcutis and/or skin appendages leading to overall thinning and loss of elasticity. Apart from primary
atrophic conditions, a large number of dermatological diseases and traumatic conditions leave atrophy as sequelae.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

314 DOI: 10.1201/9781351054225-30

 14
Purpura

SANTOSHDEV P RATHOD

INTRODUCTION ●● Usually the only symptom is a tendency to bleed from

gingivae after brushing teeth or into the skin-producing
Purpura is extravasation of red blood cells into the skin and/ crops of petechiae or larger hemorrhages (Figure 14.1).
or mucosa and does not blanch on diascopy. Understanding ●● The spleen is usually mildly enlarged; gross enlargement
the clinical characteristics of a purpuric lesion (Box 14.1) is should suggest an alternate diagnosis.
crucial for generating clinical differentials. Other features ●● DD: Systemic lupus erythematosus (SLE), drug-induced
that help in clinical diagnosis include age of onset, associ- purpura, disseminated intravascular coagulation, and
ated skin lesions, and general status of the patient. Purpuric renal failure.
lesions may be an ominous sign of many life-threatening dis-
eases, including septicemia, vasculitis, purpura fulminans,
bleeding disorders, etc., and hence need immediate attention Leukemia/bone marrow failure
●● Splenomegaly is the major feature.
and urgent evaluation by treating physicians. Additionally,
●● Purpura and ecchymoses are among the common
purpura in a child (child abuse) or in adults may be a medi-
colegal concern. This chapter focuses on clinical evaluation of cutaneous features.
purpuric lesions (outlined in Table 14.1), and salient features
of different entities are discussed below.1–6 Additional clinical Heparin-induced thrombocytopenia
clues are enumerated in Table 14.2. ●● This can occur in conjunction with heparin-induced

skin necrosis.
●● This may immediately occur if patient has been sensitized

PETECHIAE AND ECCHYMOSES and has received heparin within past 100 days.
●● Lesions are most common at subcutaneous injection

Idiopathic thrombocytopenic purpura sites but can occur elsewhere.

●● It commonly induces cutaneous findings of simple
●● Two-thirds of cases occur before 21 years of age.

●● There is a strong female preponderance in adults. hemorrhage with ecchymoses and occasionally triggers
●● The onset may be gradual or, especially in children, acute. urticaria or infiltrated plaques.

BOX 14.1:  Clinical characteristics of purpura

●● Based on size
●● Petechiae – flat lesions ≤4 mm, initially bright red and then fading to a rust color.

●● Ecchymosis – flat lesions >5 mm, typically initially red or purple, but color changes to yellow, brown, or green

as the lesion ages.

●● Based on morphology
●● Palpable purpura – elevated, round or oval, red or purple papules and/or plaques.

●● Retiform purpura – stellate-shaped or branching lesions, with angular or geometric borders. These are usually

palpable, but may be non-palpable too.

DOI: 10.1201/9781351054225-31 315

316 Purpura

Table 14.1  Clinical approach to purpura

Morphology Features Diseases

Petechiae Prolonged bleeding on trauma • Acute, mucosal bleeding – immune thrombocytopenia
• Rapid onset, meningism, hypotension, shock – acute
bacterial sepsis, including invasive meningococcal
disease, active hemorrhagic type of disseminated
intravascular coagulation (DIC)
• Subacute, lethargy, pallor – myelosuppression, non-
leukemic bone marrow failure (e.g., myelodysplasia,
aplastic anemia, solid tumor infiltration)
• Chronic, family history – congenital bleeding disorders
including hemophilia and von Willebrand disease
• Variable – drug-induced thrombocytopenia
Neurological signs (seizures, Thrombotic thrombocytopenic purpura
hemiplegia, paresthesias)
History of surgery, sepsis, delivery, Disseminated intravascular coagulation (DIC)
neoplasm, etc.
Others • Lower extremities – pigmented purpuric dermatosis,
hypergammaglobulinemic purpura of Waldenstrom
• Face and neck – Valsalva-associated petechiae
• Superior vena cava area – strangulation
• Random – trauma, local pressure
Ecchymosis Onset in elderly Solar/ senile purpura
Onset in childhood • Delayed wound healing – genetic disease with collagen/
elastin defects (Ehler–Danlos syndrome,
pseudoxanthoma elasticum)
• Excessive bleeding – clotting factor deficiency, acquired
thrombocytopenia secondary to sepsis or leukemia
• Patterned – battered baby syndrome/child abuse
Associated signs • Macroglossia – systemic amyloidosis
• Hemorrhagic bulla, skin necrosis – DIC
• Perifollicular petechiae, twisted broken hairs – scurvy
• Prolonged bleeding on trauma – liver disease, vitamin K
deficiency
Others Trauma, anticoagulant use
Palpable purpura • Subacute to chronic course – vasculitis (leukocytoclastic
vasculitis, small-vessel vasculitis, small- to medium-
vessel vasculitis, ANCA associated vasculitis; see
Chapter E48)
• Acute, viral illness, coryzal symptoms – papular-purpuric
“gloves-and-socks” syndrome
Retiform purpura Systemic inflammation Medium-vessel vasculitis, ANCA-associated vasculitis,
purpura fulminans (DIC)
Without systemic inflammation • Neurological signs – thrombotic thrombocytopenic
purpura
• Sick patients – vasoinvasive infections (invasive fungus,
pseudomonas, strongyloidiasis, or lucio leprosy)
• History of anticoagulant – heparin necrosis, warfarin
necrosis
• Painful lesions – antiphospholipid antibody syndrome,
emboli, calciphylaxis, livedoid vasculopathy
 Purpura 317

Table 14.2  Additional clues to clinical diagnosis of purpura

Clue Diseases
Acute onset Idiopathic thrombocytopenic
purpura, Henoch-Schönlein
purpura, medication,
trauma, Valsalva-associated
petechiae
Mucosal bleeding Platelet disorders,
thrombocytopenia, von
Willebrand disease
Figure 14.2  Perifollicular petechiae in scurvy. (Courtesy:
Lethargy, fever, bone pain Leukemia Dr. Debayan Dasgupta, Berhampore, India.)
Antecedent infection Idiopathic thrombocytopenic
purpura, Henoch-Schönlein
purpura Thrombotic thrombocytopenic purpura (TTP)
Family history of bleeding von Willebrand disease, and hemolytic-uremic syndrome (HUS)
hemophilia ●● TTP occurs in adults and HUS in children.
Restricted diet/malnutrition Scurvy or vitamin K deficiency ●● Purpura is associated with fever, hemolytic anemia and
Hepatosplenomegaly Leukemia renal and neurological symptoms.
●● Childhood HUS is distinguished clinically because

it develops one week after diarrhea due to E. coli

O157:H7.
●● Uncommonly, purpuric, tender, sharply demarcated
plaques develop at lesional margins. Erythema may Scurvy
sometimes accompany such lesions, and necrosis is ●● Vitamin C deficiency causes collagen defect.
frequent. ●● Initial skin change is follicular keratosis, with coiled
●● DD: Post-transfusion purpura in early stages.
hairs (corkscrew hairs) on the upper arms, buttocks,
and lower extremities.
●● Later, there is perifollicular hemorrhage with

blood pigment discoloration, mainly on the legs

(Figure 14.2).
●● Swollen bleeding gums (spongy bleeding gums),

stomatitis, painful joints, and epistaxis occur.

Actinic purpura
●● Actinic purpura presents as purpuric macules on the

forearms, hands, face, and neck. They resolve after

one to three weeks and may leave residual brown
pigmentation (Figure 14.3a).
●● It occurs most commonly in skin altered by both age

and solar radiation but may occur in premature aging

syndromes.

Corticosteroid purpura
●● Purpura occurs mainly in the atrophic skin on

exposed parts of the hands and forearms or on the

legs after minor trauma or spontaneously. It is usually
asymptomatic, and purpuras vary in size from a few
millimeters to several centimeters, arranged linearly or
in geometric shapes.
●● The characteristic appearance has non-palpable

irregular areas with very little inflammation, which are

usually dark purple rather than having the sequential
Figure 14.1  Petechiae in idiopathic thrombocytopenic color changes of normal bruises and may persist for
purpura. several weeks (Figure 14.3b).
318 Purpura

Hypergammaglobulinemic purpura
of Waldenstrom
●● Clinically the pattern of purpura is often non-specific,

usually consisting of crops of small erythematous

macules or palpable purpuric spots on the lower leg,
but unusual patterns with reticulate lesions may appear,
with itching and burning sensations.
●● Prolonged walking, standing, or sitting with legs in

dependent positions are obvious provocative factors.

Valsalva-associated petechiae
●● This is the mechanical cause of petechiae.

●● Raised pressure leads to a cervico-facial arrangement of

purpuric lesions (Figure 14.4c,d).

Coagulation-factor deficiency
●● This could be a congenital or acquired deficiency.
●● Most commonly encountered are coagulation factor

VIII deficiency, coagulation factor IX deficiency, and

von Willebrand disease (Figure 14.5a,b).
●● Onset occurs in neonates and infants.

Pigmented purpuric dermatoses

●● Schamberg’s disease

●● This involves a few or many lesions, occurring most

frequently on legs, but it may occur anywhere on the
body, including the palms.
●● They consist of irregular plaques of orange or
brown pigmentation due to hemosiderin, with
characteristic “cayenne pepper” spots appearing
within and at the edge of old lesions (Figure 14.6a).
●● They are usually asymptomatic but may be slightly
itchy.
●● Eruption is usually chronic and persists for many
years, with slow extension and some clearing of
original lesions.
●● DD: Itching purpura.
Figure 14.3  (a) Ecchymosis in sun-exposed area ●● Itching Purpura

in a farmer. (b) Purpura on the face in a female with ●● Purpuric lesions usually commence around the
long term topical corticosteroid abuse. Note ankles and in a few weeks spread to involve the
epidermal thinning. (a,b – Courtesy: Dr. Piyush Kumar, whole legs, sometimes the lower part of the body,
Katihar, India.) and even elsewhere.
●● They are more pronounced at sites of friction with
clothing. The lesions consist of erythematous and
Primary systemic amyloidosis purpuric macules that may become confluent and
●● Petechiae, purpura, and ecchymoses may occur often have a characteristic orange color.
spontaneously or after normal trauma on normal or ●● DD: Carbromal sensitivity, rubber or clothing
clinically involved skin, especially in the body folds dermatitis.
(e.g., the eyelids, sides of neck, axillae, umbilicus, oral ●● Pigmented purpuric lichenoid dermatosis of

and anogenital regions). Eyelid purpura after pinching, Gougerot-Blum

and periorbital purpura after proctoscopy, coughing, ●● The characteristic clinical feature of the dermatosis
vomiting, or the Valsalva maneuver are characteristic is the presence of lichenoid papules in association
(Figure 14.4a,b). with purpuric lesions similar to those of
●● Purpuric halos may appear around cherry angiomas. Schamberg’s disease,
 Purpura 319

Figure 14.4  (a) Primary systemic amyloidosis with facial purpura and macroglossia. (b) Periorbital purpura. (c) Petechiae
over the nose due to Valsalva procedure. (d) Periocular petechiae associated with severe coughing. (a,b – Courtesy:
Dr. Tanumay Raychaudhury, Skin and Cancer Foundation, The University of Sydney, Australia; c – Courtesy: Dr. Piyush
Kumar, Katihar, India; d – Courtesy: Dr Srinivas Devarashetty, Nizamabad, India.)
320 Purpura

●● They may resemble a bruise, and hence must

be distinguished from non-accidental injury in
children, but may persist for a few years.
●● Purpura annularis telangiectodes
●● Clinical features are distinctive and described in its
name. Lesions occur at any site, often in the absence
of venous stasis, and may be few in number or very
numerous. They consist of small plaques 1–3 cm
across that are usually annular from their onset
(Figure 14.6c).
●● Lesions consist of telangiectases and hemosiderin
staining of the skin. They may be purple, yellow, or
brown, and may contain “cayenne pepper” spots.
Individual lesions persist unchanged for many
months or years, or there may be slow centrifugal
extension with development of slight central
atrophy.

Battered baby syndrome

●● Up to 90% of victims of physical abuse present with

skin findings. Although bruising is the most common

physical sign of abuse, it is also a frequent finding in any
active child. Accidental bruising most commonly occurs
over the knees and anterior tibial area.
●● Bruising over relatively protected sites such as the

upper arms, medial and posterior thighs, hands, trunk,

cheeks, ears, neck, genitalia, and buttocks should raise
suspicion of abuse, especially if the bruises are extensive
and of varying age. Bruising of the genitalia and ears is
highly suspicious for abuse.
●● Bruises are extremely rare in babies less than six months

of age, as they are not yet mobile. Any single soft-tissue

injury in a preambulatory infant has a high correlation
with abuse. Another helpful factor is the shape of the
bruise, which can reflect the shape of the object used to
inflict it.
●● Pattern bruising is a strong indicator of abuse.

Vitamin K deficiency
●● Vitamin K deficiency bleeding (VKDB) disorder is an

uncommon entity that occurs due to inadequate activity

of vitamin K-dependent coagulation factors.
●● Clinical presentation of late VKDB ranges from life-

threatening intracranial hemorrhage to skin bleeds with

nodular purpura.
●● Though skin bleeding in the form of petechiae, purpura,
Figure 14.5  (a) Purpura in a child with hemophilia.
(b) Purpura in a girl child with hemophilia. (a,b – Courtesy: or ecchymoses may occur in 10–30% of patients, it is a
Dr. Piyush Kumar, Katihar, India.) presenting feature in only half of such infants.
●● It occurs on the lower extremities, back, chest

●● Lichen aureus abdomen, buttocks, upper extremities face, and neck in

●● This involves a more localized, more intensely descending order of frequency.
purpuric but often asymptomatic eruption that may ●● These lesions are bluish-violet in color with raised

have rather lichenoid morphology. infiltrated purplish centers, and their diameter may
●● The lesions are often solitary and may be yellowish, vary from 1.5 cm to 7.5 cm. Hematomas may occur at
golden, rust-colored or purple (Figure 14.6b). skin puncture sites.
 Purpura 321

Figure 14.6  (a) Schamberg’s disease with purpuric lesions

and hyperpigmented macules and patches. (b) Lichen
aureus seen as orange-rust–colored hemorrhagic patch
with discrete petechiae. (c) Purpura annularis telangi-
ectodes. (b – Courtesy: Dr. Tanumay Raychaudhury,
Skin and Cancer Foundation, The University of Sydney,
Australia; c – Courtesy: Dr. Bhushan Madke, Jawaharlal
Nehru Medical College, Datta Meghe Institute of Medical
Sciences, India.)
 
322 Purpura

PALPABLE PURPURA

Cutaneous small vessel vasculitis (CSVV)

●● CSVV typically presents with palpable purpura

ranging in size from 1 mm to several centimeters.

It is sometimes macular in early stages but may
progress to a wide array of lesions, including
urticarial papules, pustules, vesicles, petechiae, or
targetoid lesions (Figure 14.7).
●● The lesions favor dependent sites as well as

areas under tight-fitting clothing, reflecting the

influence of hydrostatic pressure and stasis on the
pathophysiology.
●● In general, the lesions are asymptomatic, but they may

itch, burn, or sting.

●● In differential diagnosis, vasculitis with systemic
Figure 14.8  Purpuric lesions on the lower limbs in
manifestations must be ruled out. Henoch-Schönlein purpura.

Henoch-Schönlein purpura
●● Angioedema and macular erythema may also occur;
●● At its outset this manifests with the classic findings of
livedo reticularis, nodules, and bullae may be evident.
purpura, arthralgia, and abdominal pain. ●● Patients with the hypocomplementemic form may
●● The cutaneous findings are typically erythematous
have constitutional symptoms such as fever, malaise,
urticarial papules, which evolve within 24 hours into myalgia, and other signs, including lymphadenopathy,
palpable purpura with hemorrhage. Furthermore, hepatosplenomegaly, and abdominal pain with or
urticaria, vesicles, bullae, and necrotic ulcers may without nausea and or diarrhea.
develop. ●● DD: Presence of ocular inflammation, angioedema and
●● Typically involving extensor aspects of the limbs and
COPD distinguish it from SLE. Other differentials include
buttocks in symmetrical fashion, HSP may also affect urticaria and atypical forms of erythema multiforme.
the trunk and face. It usually fades within five to seven
days; however, crops of lesions can recur from a few
weeks to several months (Figure 14.8). ANCA-associated vasculitis
●● DD: Cutaneous small-vessel vasculitis, Wegener’s ●● Granulomatosis with polyangiitis (GPA)

granulomatosis, Churg-Strauss syndrome, microscopic ●● The most common cutaneous manifestation of GPA
polyangiitis, and other vasculitides. is palpable purpura.
●● Others include tender subcutaneous nodules,
Urticarial vasculitis papules, vesicles, and petechiae as well as pyoderma
●● Individual lesions (hives) persist for more than
gangrenosum-like lesions.
●● Papulonecrotic lesions appear most commonly on
24 hours, often demonstrate purpuric foci, leave post-
the limbs but also occur on the face and scalp.
inflammatory hyperpigmentation, and cause symptoms
●● Oral ulcers and “strawberry” gingival hyperplasia
of burning.
are pathognomonic.
●● Saddle nose deformity may result from necrotizing
granulomas of the nasal mucosa.
●● DD: Churg-Strauss syndrome.
●● Eosinophilic granulomatosis with polyangiitis

●● The first phase, which may continue for years,

consists of allergic rhinitis, nasal polyps, asthma,
and peripheral blood eosinophilia.
●● The second phase is vasculitis.
●● Palpable purpura and infiltrated nodules (typically
located on the scalp or limbs) are the most common
skin manifestations.
●● Livedo reticularis, migratory erythema, new onset
Raynaud’s phenomenon, aseptic pustules or vesicles,
Figure 14.7  Palpable purpura in cutaneous small vessel and infiltrated papules may also be present.
vasculitis. (Courtesy: Dr. Piyush Kumar, Katihar, India.) ●● DD: Granulomatosis with polyangiitis (GPA).
 Purpura 323

Papular-purpuric “gloves-and-socks” syndrome ●● Digital gangrene can ultimately occur.

●● It is considered a reaction pattern to various viral ●● In cutaneous polyarteritis nodosa the disease is limited
infections and, possibly, bacterial infection and drugs. to skin for long periods of time; nodules and ulcers
●● The most established cause is parvovirus B19 infection. occur, usually on the legs.
Other documented causes include Coxsackie B virus, ●● DD: Antiphospholipid antibody syndrome, cholesterol
measles virus, human herpesvirus 6, and drugs emboli or other factors that can produce non-vasculitic
(co-trimoxazole). vessel occlusion.
●● Cutaneous lesions start with edema and erythema on
Purpura fulminans with DIC (septic vasculitis)
the hands and feet in a “gloves-and-socks’” distribution.
●● This is usually sepsis-related and presents with
This is followed by development of painful and/or
pruritic, erythematous papular and purpuric lesions disseminated intravascular coagulation (DIC).
●● There is widespread cutaneous hemorrhage with sepsis.
in the same distribution. A clinical clue is sharp
●● It clinically presents as non-inflammatory (bland)
demarcation of the rash at the wrists and ankles. Rarely,
the lesions may progress to vesicles and bullae and hemorrhage, usually with retiform, stellate or branching
ulceration. configuration, with rapid transition to necrosis and
●● Sometimes other sites, such as the face, trunk, and eschar formation (Figure 14.10a).
●● When patients are on vasopressors, there is an
thighs, may be affected.
●● Systemic findings include fever, asthenia, headache, additional component of peripheral gangrene.
●● It often affects distal extremities.
anorexia, and arthralgia.
●● Mucosal findings are as follows:
Post-infectious purpura fulminans (PF)
●● Oral mucosa – pharyngeal erythema, erosions,
●● This is a rapidly progressive disorder.
petechiae, and vesiculopustular lesions on the hard
●● It occurs most commonly in the setting of acute severe
and soft palates
●● Genital mucosa – painful edema and erythema bacterial or viral infection and as a post infectious
●● The condition resolves in a few weeks.
syndrome after infections such as primary varicella and
scarlet fever.
●● It usually occurs seven to ten days after the onset of

symptoms of an acute infection.

RETIFORM PURPURA ●● Post-infectious PF tends to occur on the lower body,

especially thighs, lower legs, and buttocks, and

Polyarteritis nodosa phenomenon such as large-vessel venous thrombosis
●● Dermal or subcutaneous nodules are most commonly and systemic microvascular thrombosis and multi-
located on the lower legs near the ankles and may organ failure are uncommon (Figure 14.10b).
●● Post-infectious PF should be considered strongly if
extend proximally to the thighs, buttocks, arms,
or hands. These nodules may be tender, which may purpura fulminans with extensive necrotic skin lesions
ulcerate, or more commonly demonstrate livedo with or without DIC develop following nonspecific
reticularis, which may be necrotizing (Figure 14.9). infection in an otherwise healthy child.

Lucio phenomenon (LPh)

●● It is characterized by the occurrence of painful, red

or purpuric macules, of irregular shape, angulated or

“stellar,” in a patient with diffuse lepromatous leprosy
(Lucio-Latapí leprosy).
●● Lucio phenomenon in lepromatous leprosy or type 2

leprosy reaction is clinically characterized by crops of

sharply delineated, purple-red lesions of irregular shape,
angulated or “stellar” on the extremities. Lesions are
accompanied by a burning sensation and indurations
that evolve into necrotic-hemorrhagic lesions.
●● Lesions appear on the lower extremities and then

spread to the trunk and upper extremities. Patients may

present with fever, myalgia, or arthralgia.

Periumbilical thumbprint parasitic purpura (PTPP)

●● It is cutaneous sign of strongyloidiasis seen in the

Figure 14.9  Purpuric lesions in polyarteritis nodosa. Note more advanced stages of Strongyloides infection and
two ulcerated lesions. represents a poor prognostic sign for the patient.
324 Purpura

Figure 14.10  (a) Stellate purpura in purpura fulminans. (b) Purpura and epidermal necrosis in post infectious purpura fulminans.
(c) Mucormycosis causing periorbital edema with hemorrhagic surface. (d) Hemorrhagic bulla in mucormycosis. (a – Courtesy:
Dr. Anuradha Priyadarshini, Sri Ramachandra Medical College, Chennai, India; b – Courtesy: Dr. Rajeev Ranjan and Dr. Shalini
Sinha, Ara, India; c,d – Courtesy: Dr. Raghuraj Hegde, Kempegowda Institute of Medical Sciences, Bangalore, India.)

●● It usually presents as periumbilical purpura that Angioinvasive fungal infections

spreads centrifugally throughout the abdomen and ●● These are acute, rapidly evolving, often fatal infections
groin. due to vasculotropic invasive mycoses, such as
●● The purpura is caused by the dermal migration of Aspergillus (flavus, fumigatus, niger), Fusarium (solani,
filariform larvae through vessel walls or the large oxysporum, verticillioides), and Zygomycetes (Mucor,
bowel wall. Rhizopus, Rhizomucor, and more) (Figure 14.10c,d).
●● It has been proposed that the periumbilical distribution ●● Lesions typically present as tender, rapidly enlarging,
may result from retrograde venous migration. bright-red to purple papulonodules that rapidly develop
Additionally, larvae may penetrate into the skin from areas of ischemic necrosis, ulcers, and/or hemorrhagic
the abdominal cavity, following migration through the bullae, which may be accompanied by purpura,
large-bowel wall. erythema, warmth, and induration.
 Purpura 325

Warfarin necrosis
●● Warfarin necrosis typically affects areas of the body

with greater adipose tissue, including the breasts,

thighs, and buttocks.

Heparin skin necrosis

●● Heparin skin necrosis shows a predilection for sites of

subcutaneous heparin or low-molecular-weight heparin

(LMWH) injections; if patients are on intravenous
(IV) infusions, sites distal to the IV site are more
commonly involved.
●● It commonly induces cutaneous findings of simple

hemorrhage with ecchymoses and occasionally triggers

urticaria or infiltrated plaques.
●● Purpuric, tender, sharply demarcated plaques

develop; retiform extensions that sometimes develop

at the lesional margins are characteristic of heparin
necrosis.

Cryoglobulinemia
●● Cryoglobulinemia, especially type I, involves colder

areas of the body, such as the distal digits and ears.

●● Recurrent showers of dependent palpable purpura,

sometimes with burning or itching and frequently

associated with arthritis or arthralgia, is the Figure 14.11  Acral purpuric lesions affecting the digits
classic presentation of mixed (type II and type III) in cholesterol embolization. (Courtesy: Dr. Preema Sinha,
cryoglobulinemia. Armed Forces Medical Sciences, Pune, India.)
●● The combination of purpura, asthenia, and arthralgia is

termed Meltzer’s triad.

●● Ulceration, hemorrhagic crusting, or cutaneous Anti-phospholipid syndrome (APLS)
infarction is seen in 10–25% of cases. ●● Clinically, APLS presents with a variety of cutaneous

●● Acrocyanosis, Raynaud’s phenomenon, urticarial findings; retiform purpura, anetoderma, and atrophic
lesions, ulceration, and livedo-reticularis may be blanche are seen, with widespread cutaneous necrosis
present. and multiorgan failure, especially renal and pulmonary.
●● APS is characterized by typical clinical manifestations

Calciphylaxis (livedo reticularis, livedo racemosa, livedo vasculitis,

●● Calciphylaxis, also known as calcific uremic thromboembolic phenomena with necrosis and
arteriolopathy, may also produce an occlusion within ulceration of the extremities, digital gangrene, purpura,
the vessels, although the most characteristic finding and purpura fulminans) and persistent aPL positivity
consists of calcification of the media of muscular small- (at least 12 weeks apart).
●● Additionally, Behçet-like lesions, nail-fold ulcers,
and medium-sized arteries.
●● It typically starts as livedo reticularis and progresses to leg ulcers, cholesterol emboli-like proximal
intensely painful, indurated, symmetrical reticulated livedo-reticularis with or without distal retiform
violaceous plaques with necrosis, usually affecting acral purpura, acral livedo, degos-like lesions, pyoderma
sites as well as fat-rich body areas. gangrenosum-like lesions, and splinter hemorrhages
may be present.
Cholesterol emboli
●● Livedo reticularis of the lower extremities and buttocks Livedoid vasculopathy
is the most common cutaneous manifestation, although ●● It initially manifests as erythematosus or purpuric

the trunk and upper extremities may also be involved. plaques or papules that are painful and/or pruritic
●● The livedoid change may be more pronounced in the and are most commonly located bilaterally around the
standing position and may be missed if only supine ankles (Figure 14.12).
examination is performed. ●● These lesions may ulcerate and then slowly heal
●● Cholesterol emboli also result in “blue toe syndrome” over a period of three to four months, forming residual
and dry gangrene of the digits (Figure 14.11). atrophic stellate white scars called atrophie blanche.
●● Less commonly, petechiae and purpura of the lower

extremities are seen with this syndrome. Additional images – Figures 14.13 and 14.14
326 Purpura

Figure 14.12  Purpuric lesions, ulcers, and atrophie

blanche in livedoid vasculopathy.

Figure 14.14  Rifampicin-induced thrombocytopenia

Figure 14.13  Traumatic purpura. Note color changes. presenting with purpuric lesions. (Courtesy: Dr. Soumyajit
(Courtesy: Dr. Piyush Kumar, Katihar, India.) Roychoudhury, Berhampore, India.)

REFERENCES 4. Leung AK, Chan KW. Evaluating the child with purpura. Am Fam
Physician 2001;64(3):419–428.
1. Arakaki R, Fox L. Updates in the approach to the patient with 5. Wysong A, Venkatesan P. An approach to the patient with retiform
purpura. Curr Derm Rep 2017: 6;55–62. purpura. Dermatol Ther 2011;24(2):151–172.
2. Piette WW. Purpura: Mechanisms and Differential Diagnosis. In: 6. Georgesen C, Fox LP, Harp J. Retiform purpura: A diagnostic
Bolognia JL, Schaffer JV, Cerroni L, editors. Dermatology. 4th edi- approach. J Am Acad Dermatol 2020;82(4):783–796.
tion. North York: Elsevier Limited; 2018. p. 376–389.
3. Lamadrid-Zertuche AC, Garza-Rodríguez V, Ocampo-Candiani JJ.
Pigmented purpura and cutaneous vascular occlusion syndromes.
An Bras Dermatol 2018;93(3):397–404.
 E16
Erythema: Localized

APARAJITA GHOSH, PC DAS

ABSTRACT
Erythema is an effect on the skin induced by enhanced blood flow. Different physiological and pathological conditions are
responsible for the same. Based on the extent of skin involvement, erythema may be localized or generalized, and at the same
time it may be a primary event or a coincidental condition. The differentials depend on the pattern and localization of ery-
thema, age of presentation, preceding events, and associated skin lesions.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-32 327

 E17
Erythema: Generalized

SWETALINA PRADHAN, ARPITA NIBEDITA ROUT

ABSTRACT
Erythema represents a blanchable red or pink color of skin or mucous membrane. It is caused due to dilation of arteries and
veins in the papillary or reticular dermis. Based on the extent of skin involvement, erythema may be localized or general-
ized, and at the same time it may be a primary event or a condition with prominently associated features such as fever, rash,
pustules, crusts, scales, epidermal detachment, and pruritus. This chapter discusses the clinical differentials of generalized
erythema.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

328 DOI: 10.1201/9781351054225-33

 E18
Telangiectasia

SUNIL K KOTHIWALA, KANYA RANI VASHISHT

ABSTRACT
Telangiectasias are visibly and persistently dilated blood vessels, coursing through the surface of the skin or mucous mem-
brane. They may be localized or widespread in distribution. Telangiectasia in certain disorders may have a site predilection,
such as involvement of the photo-exposed areas, upper or lower body half, bulbar conjunctiva, lips, oral mucosa, anterior
chest, or proximal nail folds. Segmental and nevoid forms occur as well. Morphologically, localized telangiectasia can be
either macular (and further linear, arborized, spider like, punctate, mat-like or diffusely erythematous) or papular. Their
color depends on the vessel of origin (capillaries or venules). The presence or absence of atrophy aids in further classification.
Etiologically, they may be primary or secondary. In addition, several genodermatoses present with telangiectasia or inher-
ited poikiloderma. While telangiectasia may arise from a multitude of causes, their morphological features, distribution/site
predilection, associated cutaneous features, and systemic features as derived from a careful history and examination help in
reaching the correct diagnosis. This chapter reviews the possible causes to consider and diagnostic approach in a patient pre-
senting with telangiectasia.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-34 329

 E19
Cysts and pseudocysts

PC DAS

ABSTRACT
Cysts may occur in any organ system of the body, and the symptomatology relates to the function subserved by the involved
organ. Cutaneous cysts are common lesions that present to dermatologists for cosmetic reasons, mass effect, or secondary
changes, such as discharge, inflammation, and infection. Cysts present as superficial or deep swelling morphologically mim-
icking a papule, nodule, or tumor.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

330 DOI: 10.1201/9781351054225-35

 15
Draining sinuses and fistulas

SUNIL K KOTHIWALA, KANYA RANI VASHISHT

INTRODUCTION symptoms. Imaging studies are thus advised prior to

excision to exclude an intracranial connection.
A sinus tract is an abnormal blind-ended tract that opens ●● DD: Epidermoid cysts are not present at birth; they may
onto the skin or into a cavity. A fistula is an abnormal tract occur elsewhere and are not characteristically midline.
connecting the cavities of two internal organs or the cavity Nasal gliomas may present as nasal masses.
of an internal organ with the skin. As sinus and fistula can-
not be easily differentiated from each other on cutaneous Preauricular cysts and pits
examination alone, this chapter clubs them together for the ●● Imperfect fusion of tubercles leads to entrapment of

purpose of discussing clinical differentials. Etiologically, epithelium to form preauricular cysts which connect to
sinuses and fistulae may be congenital (developmental), the skin via preauricular sinuses.
traumatic, infective or non-infective. A broad approach ●● Presentations:

considering morphological aspects is outlined in Table 15.1 ●● These usually present as a unilateral asymptomatic
and Figure 15.1. The salient features of different entities are cystic nodule or invagination in the preauricular area
discussed below.1–7 (just in front of the ascending limb of the helix).
●● Acute infection presents as tenderness and purulent
CONGENITAL OR DEVELOPMENTAL discharge.
SINUS ●● Chronic infection may result in granulomatous
nodule over the opening of sinus.
●● DD: First branchial arch sinus, granulomatous nodule
Congenital head and neck swellings are usually present at
birth but may manifest up to youth. These cystic swellings may be confused with lupus vulgaris.
may be complicated into sinuses and fistulae. They may be Thyroglossal duct cyst
facial or cervical in location, and the latter may be either
●● During development, the thyroid gland descends from
medial or lateral cervical lesions (Figure 15.2). The exact
the floor of the pharynx to the anterior neck, forming
position provides important clinical clues as some of the
a tract called thyroglossal duct. Remnants of this duct
lesions are characteristically midline (e.g., dermoid cyst,
take the form of fistulae, cysts, or solid masses of ectopic
dermal spinal tract). Any midline sinus or cystic lesion in
thyroid tissue.
a child should be approached with the understanding that
●● This is the most common cause of midline swelling of
deeper connections may be found. Congenital midline cysts
neck in children/young adults.
should be imaged to exclude deep, especially intracranial
●● It may be located anywhere from the suprasternal notch
connections.
to the foramen cecum of the dorsal tongue. Hence it
Dermoid cysts may be located either above or below the hyoid. A tract
●● These present in an infant as non-tender subcutaneous connecting it to the hyoid bone results in characteristic
nodules along an embryonic fusion plane around the upward movement on swallowing or tongue protrusion.
eyes or nose. Those on the bridge of the nose often have ●● Fistulae may form following infection or procedure for

a sinus tract and a tuft of hairs protruding out, with the cyst. Intraoral opening may result in unpleasant taste.
sebaceous discharge (pathognomonic). It may produce ●● Preoperative neck ultrasonography is the diagnostic

nasal widening with hypertelorism. tool, whereas postoperative histopathology of the

●● Dermoid cysts can rarely present as sinus or fistula and excised cyst and fistula is mandatory to exclude rare
may have an intracranial extension passing through occurrence of malignancy.
the foramen cecum, giving rise to meningeal signs and ●● DD: Branchial cleft cyst.

DOI: 10.1201/9781351054225-36 331

332  Draining sinuses and fistulas

Table 15.1  Site predilection of various developmental and acquired sinuses and fistulae

Onset Site Disease Comment

Congenital/ Head and neck Dermoid cyst Along embryonic closure lines of the head
Developmental and neck
Common sites: around lateral third of the
eyebrows, bridge of nose
Face Preauricular cyst or pit Small area anterior to the ear
Lip pit On lower lip on one or either side of midline
Neck Thyroglossal cyst Anterior neck, in close proximity to the
hyoid bone
Branchial cyst or sinus Along anterior border of
sternocleidomastoid, mandibular region
(over parotid gland)
Bronchogenic cyst Suprasternal notch, pre-sternal area, lower
anteromedial aspect of neck
Acquired Face Dental sinus Along mandibular jaw line or submandibular
region
Scrofuloderma Commonly on the neck or submandibular
region
Actinomycosis Mandibular region; less commonly chest
wall or iliac fossa
Pyoderma faciale Cheeks, chin, and/or forehead
Oral squamous cell carcinoma Lateral and undersurface of tongue, floor of
mouth are common sites
Thorax Actinomycosis
Axilla and/or groin Hidradenitis suppurativa Axillae (preferential location in women) and
perineum (preferential location in men)
Scrofuloderma
Inguinal region Lymphogranuloma venereum Inguinal region
Enterocutaneous fistula Iliac fossa
Actinomycosis
Extremities Milker’s sinus 2nd or 3rd webspace of dominant (usually
right) hand
Botryomycosis Limbs, perianal area
Antitrypsin-deficiency panniculitis Proximal extremities and lower trunk
Pancreatic panniculitis Legs
Mycetoma Foot
Perianal area Pilonidal sinus In or just above the gluteal cleft
Cutaneous amoebiasis Perianal region, buttocks
Malakoplakia of the skin Perianal and genital area
Crohn’s disease Genital or perianal area (metastatic
cutaneous Crohn’s disease); parastomal
(non-metastatic cutaneous Crohn’s
disease)
Variable Wound myiasis Pre-existing non-healing wound
Chronic osteomyelitis Vertebral bodies, long bones (tibia and
femur), foot (in vascular insufficiency/
neuropathy)
Atypical mycobacterial Nodules and sinuses, surgical sites infection
or follows trauma
Cutaneous metastasis
Others Trauma, radiotherapy
 Draining sinuses and fistulas  333

Discharging
sinus

Early onset Late onset

Head & Neck Other sites Head & neck Extremities Perianal/perineum/groin

Head: Umbilical fistula Actinomycosis Mycetoma Lymphogranuloma

Preauricular pits/sinus Bronchogenic cyst Oral squamous cell Actinomycetoma venerum
Dermoid cyst Epidermoid cyst carcinoma Botryomycosis Amoebiasis
Hodgkins disease Deep fungal Hiadrenitis
Neck: Pyoderma faciale infection suppurativa
Medial cervical cysts: Folliculitis keloidalis Osteomyelitis Malakoplakia
Thyroglossal duct cyst Odontogenic fistula Milker’s sinus Pilonidal sinus
Tuberculosis Crohn’s disease
Lateral cervical cysts:
Brachial cyst

With nodules Without nodules

Scrofuloderma Osteomyelitis
Mycetoma Lymphogranuloma venerum
Botryomycosis Actinomycosis
Chromoblastomycosis Squamous cell carcinoma
Elephantiasis verrucosa Amoebiasis
Atypical mycobacterial Myiasis
infection Milker sinus
Hidradenitis suppurativa Traumatic
Folliculitis keloidalis Peristomal fistula
Pyoderma faciale Pilonidal sinus
Panniculitis Enterocutaneous fistula
Hodgkins disease
Milker’s sinus
Malakoplakia of skin

Figure 15.1  Approach to a case of discharging sinus or fistula.

Figure 15.2  Common sites of developmental cysts, sinuses, and fistulae of the head and neck.
334  Draining sinuses and fistulas

Branchial cleft cyst (lateral cervical cyst) ●● The suspicion of a dermal sinus tract calls for pediatric
●● Incomplete fusion of branchial arches and their referral and further imaging. This is because of a
remnant cleft or pouches results in cysts, sinuses, or possible intradural connection and further chances of
fistulae. Second branchial arch anomalies are often infection. Such infants may present with unexplained
implicated. Branchial cleft anomalies result in sinus and neurological signs (lower limb weakness, altered bowel/
fistulae communicating with skin (ectoderm), while bladder function, meningeal signs).
branchial pouches result in channel opening to internal ●● DD: It must be differentiated from “coccygeal dimple,”
mucosa (endoderm). which occurs within the gluteal cleft (a few millimeters
●● These lesions present between ages 10 and 30 years, away from the coccyx top), has no associated cutaneous
in the preauricular area, mandibular region (over the stigmata, and has a base that is visible and pointed
parotid gland and below the angle of mandible), or the inferiorly towards the coccyx. It is benign and requires
lower third of the neck along the anterior border of the no further management.
sternocleidomastoid.
●● The tract can be palpated, going upwards in the neck

from the opening. The opening may be more noticeable ACQUIRED SINUS AND FISTULA
because of secretion, infection, granulation tissue, or
skin tag. Epidermoid cyst (infundibular cyst)
●● DD: Thyroglossal duct cyst, dermoid cyst, cystic
●● This usually presents as a firm, dome-shaped swelling
hygroma.
that is movable over the deeper structures.
●● It is attached to the epidermis, and there may be a
Bronchogenic cysts (subcutaneous bronchogenic
cysts) central punctum (Figure 15.3) representing the follicle
●● A bronchogenic cyst is the most common cystic lesion
from which it is derived and from which keratin may be
expressed on squeezing.
of the mediastinum.
●● It presents in the suprasternal notch as a solitary cystic

swelling at birth, at times connected to the epidermis

with a fistulous tract.
●● The cyst is unilocular and contains clear fluid.

Histologically it is lined by columnar ciliated

epithelium with cartilage and bronchial mucous
glands in the walls.
●● The only reported symptoms are swelling and discharge

from the sinus. Potential complications are infection

and malignant changes.
●● DD: Branchial cyst, thyroglossal cyst.

Umbilical fistula
●● Failure of obliteration of the omphalomesenteric duct

(vitelline) or urachus (allantois) leads to formation of

sinuses, fistulae, cysts or polypoid tumors.
●● Urine may be observed at the opening due to a patent

urachus duct, while fecal discharge is the result of

persistent vitelline duct.
●● Intermittent bleeding or mucoid discharge may be

present due to development of polyp at the opening.

●● An ilio-umbilical fistula may result from laparotomy for

Crohn’s disease or may rarely occur spontaneously.

Dermal sinus tract (dermal spinal tract)

●● It presents as a congenital midline opening along the

spine, most commonly lumbosacral.

●● It is usually located just above the gluteal cleft,

with the tract heading inwards with surrounding

cutaneous stigmata (hair, pigmentation, erythema or
inflammation, skin tag, or lipoma). Inability to see the Figure 15.3  Epidermoid cyst with central punctum.
base of the tract by gently separating the skin on either Central punctum may be mistaken for a sinus. (Courtesy:
side is another red flag. Dr. Piyush Kumar, Katihar, India.)
 Draining sinuses and fistulas  335

●● It may be primary or secondary to traumatically

implanted epithelium (thus called “inclusion cyst.”)

Mammary duct fistula

●● This typically occurs in a young adult woman who has

a history of recurrent abscesses in a breast that has

been treated by surgical drainage or has discharged
spontaneously.
●● There is typically partial inversion of the nipple and a

scar or scars at the edge of the areola.

Milker’s sinus
●● This is an uncommon foreign body reaction resulting

from deep penetration of fragments of cow hair.

●● Most lesions involve the second or third web of the

right hand, forming tender nodules and discharging

sinuses.
●● The possible disease courses include disability due to

tendon sheath involvement, secondary infections like

recurrent cellulitis, or spontaneous cure.

Dental sinus or fistula (odontogenic fistula)

●● It is caused by chronic periapical infection, leading to

abnormal tooth canalization, and a periapical abscess

that drains externally through the skin.
●● It usually consists of one of the mandibular teeth

(as maxillary apical abscesses drain internally).

●● There is a history of tooth pain. Clinically, either

an inflamed abscess or in later stages sprouting

granulation tissue around a sinus tract opening may
be found along the jaw line or submandibular region
(Figure 15.4a–c). Palpation may help to course along
the sinus tract.
●● Dental examination and appropriate radiological

evaluation aid in diagnosis.

Chronic osteomyelitis
●● Long-persisting infection of the bone usually follows

inadequately treated acute osteomyelitis.

●● The most common presentation is persistent

discharging of the sinus, with sprouting granulation

tissue at its opening (Figure 15.5). The sinus tract is
adherent to the underlying bone, and the discharge may
contain bone chips or spicules. The bone is irregularly
thickened, and adjacent joints may be stiff.
●● DD: Septic arthritis, gout, sickle cell anemia, mycetoma.

Scrofuloderma
●● This is the most common form of cutaneous

tuberculosis seen in children.

●● It represents direct extension to the overlying skin from
Figure 15.4  (a) Dental sinus presenting as erythematous
underlying tubercular focus such as lymph node, bone, swelling and pus discharge. (b) A young boy with dental
joint, lacrimal gland, or duct. sinus seen as erythematous crusted swelling in the
●● Usually it starts as a bluish-red nodule overlying focus mandibular region. (c) Same boy with dental caries.
and breaks down to form ulcerations with undermined (a–c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
336  Draining sinuses and fistulas

Figure 15.5  Sinus associated with chronic osteomyelitis.

margins (Figure 15.6a–c). Numerous discharging

sinuses or fistulae intercommunicate beneath involved
skin. Sporotrichoid spread has been reported. The most
common sites are the neck and submandibular region.
●● DD: Lesions caused by environmental (non-
tuberculous) mycobacteria, tertiary syphilis,
sporotrichosis, actinomycosis, acne conglobata,
hidradenitis suppurativa.
Mycetoma
●● This is a chronic granulomatous condition of fungal

(more common) or bacterial origin.

●● It is characterized by a triad of induration, painless Figure 15.6  (a) Scrofuloderma presenting as nodules
swelling, and multiple sinuses with discharging granules. and discharging sinuses on thorax. (b) A young boy with
●● Fungal eumycotic mycetoma is caused by Madurella scrofuloderma of inguinal regions. (c) Old lesions of
mycetomomatis or M.grisea (dark grains) or Scedosporium, scrofuloderma heal with extensive, disfiguring scarring.
(b,c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
Neotestidina, Acremoniumspecies (pale grains).
●● An inciting factor is penetrating trauma causing

subcutaneous implantation of organism. ●● Bacterial “Actinomycetoma” can be caused by

●● The earliest stage is a firm, painless nodule, but with time, Actinomyces (A. Israeli or A. bovis) or Nocardia species.
papules and pustules appear on the skin surface then The swelling is more diffuse without distinct margins,
break down to form draining sinuses. The whole area and inflammatory with a more rapid progression and
becomes indurated and swollen but remains relatively more sinuses (Figure 15.7a–c). Bone involvement is
painless. Extension to underlying bones and joints gives faster, producing numerous small cavities.
rise to periostitis, osteomyelitis, and arthritis. In advanced ●● Fungal “Eumycetoma” is encapsulated as a well-defined
cases, destruction of bone within an infected area may be margin. It is less inflammatory with slower progression
almost complete, and gross deformity may result. Usually and fewer sinuses (Figure 15.7d). Bone involvement
there are multiple sinus tracts draining purulent or occurs after a longer period of time, forming few but
seropurulent discharge; they may remain open for months. large, clearly defined cavities.
 Draining sinuses and fistulas  337

Figure 15.7  (a) Unilateral foot swelling with multiple

erythematous nodules and sinuses in actinomyce-
toma. (b) Actinomycetoma of the hip region regions.
(c) Actinomycetoma presenting as multiple discharging
sinuses on a background of induration on the forearm.
(d) A farmer with eumycetoma clinically seen as unilat-
eral foot swelling and multiple discharging sinuses of
three years’ duration. (a – Courtesy: Dr. Rizwana Barkat,
Anugrah Narayan Magadh Medical College & Hospital,
India; b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
338  Draining sinuses and fistulas

Table 15.2  Clinical differences between actinomycetoma Lymphogranuloma venereum (LGV)

and eumycetoma ●● This is caused by Chlamydia trachomatis (L1, L2,

Features Actinomycetoma Eumycetoma and L3).

●● The organism enters through skin breaks or mucous
Progression Rapid Slow membranes (sexually transmitted) and travels via the
Inflammation More Less lymphatics to multiply within mononuclear phagocytes
Swelling Deforming, More uniform; in regional lymph nodes. Lymphangitis and suppurative
disfiguring contour of the lymphadenitis occur. Fistulae and sinus tract formation
affected part may may ensue. Healing with fibrosis disrupts lymphatic
be maintained drainage, leading to chronic edema (Figure 15.8).
Nodules at the Large Small ●● LGV occurs in three stages:

sinus openings ●● The primary stage, occurring within one month

Granules White, yellow, red White, yellow, black after transmission, is characterized by the
Regional lymph Common Uncommon appearance of a transient herpetiform ulcer at
node the inoculation site. It is painless and may go
involvement unnoticed, healing rapidly without a scar. DD:
Bone Early, numerous Late, large cavity Chancroid, granuloma inguinale, herpes genitalis,
involvement small cavities syphilis.
●● The secondary stage occurs two weeks after
the appearance of the primary lesion. It is
●● Eumycetoma may have black granules that show thick classically described as the inguinal syndrome,
septate filaments. Bacterial mycetoma have white/ and is characterized by suppurative inguinal
yellow/red granules that, on smear, delineate grains lymphadenitis (“Bubo”) with matted lymph nodes.
with gram-negative centers and gram-positive radiating, The overlying skin becomes violaceous and breaks
thin hyphae (Table 15.2). down into multiple fistulae.
●● DD: Osteomyelitis, botryomycosis. Bilateral lymph node involvement occurs in
one-third of cases. Enlargement of lymph nodes
Botryomycosis
●● This is a chronic granulomatous bacterial infection,

mostly caused by Staphylococcus aureus and

occasionally Pseudomonas.
●● The site predilection is limbs more than the perianal

area.
●● Clinically it presents as multiple subcutaneous nodules,

abscesses, ulcers, sinuses, and fistulae discharging

yellow granules composed of bacterial masses.
Histologically these are coarsely lobulated granules
(hence the term “Botryomycosis,” referring to groups of
granules that resemble grapes).
●● Single or multiple abscesses of skin and subcutaneous

tissues break down to form serous fluid discharging

sinuses that heal in months, leaving behind atrophic
scars.
●● DD: Actinomycetoma.

Coccidioidomycosis
●● Coccidioidomycosis is caused by Coccidiodes immitis, a

highly virulent fungus acquired through inhalation.

●● Following a flu-like syndrome, lesions may present as
●● Papules, pustules, abscesses, suppurative nodules
and discharging sinuses
●● Ulcerations
●● Molluscum contagiosum-like lesions occur in Figure 15.8  Lymphogranuloma venereum with inguinal
HIV bubo and sinus. Overlying skin shows scaling of tinea
●● Besides pulmonary involvement, systemic involvement cruris. (Courtesy: Dr. Neethu Mary George, Sri Siddhartha
includes osteomyelitis and meningitis. Medical College, Tumkur, India.)
 Draining sinuses and fistulas  339

above and below the inguinal ligament (femoral and

inguinal lymph nodes) produces the “sign of the
groove” in one-third of cases. Constitutional symp-
toms may be associated. Instead of inguinal bubo,
pararectal bubo may also occur. This may present
in the rectum as proctitis, with bloody, mucopu-
rulent discharge. DD: Hidradenitis suppurativa,
scrofuloderma.
●● The tertiary stage of LGV may occur many
years after the initial stages. The manifestations
result from fibrosis and lymphatic obstruction.
Elephantiasis of the external genitalia causes
fistulae and deformity. Esthiomene and
saxophone deformity are a result of such
involvement of the vulva and penile shaft
respectively.

Actinomycosis
●● Actinomycosis is a chronic granulomatous

bacterial infection caused by Actinomyces israelii

and characterized by swelling, discharging sinus,
and granules. Histologically there is suppurative
granulomatous inflammation.
●● These following clinical types haves been described:

●● Cervico-facial (“lumpy jaw”): This is the most Figure 15.9  Facial actinomycosis presenting as erythem-
common type. Trauma from dental procedures is atous nodules and sinuses on the cheek. Prior lesions
an inciting factor, with an adjacent tooth or the have healed with scarring. (Courtesy: Dr. Rizwana
tonsils being the infective focus. In the early stage Barkat, Anugrah Narayan Magadh Medical College &
it presents as a slow-growing hard swelling that Hospital, India.)
softens and bursts to form multiple erythematous
nodules, abscesses, and draining sinuses in the
mandibular region (Figure 15.9). Discharge lesions in infants with dysentery and penile involvement
contains sulphur granules. in homosexual individuals have been reported. A
●● Thorax: The lungs and pleura get infected by direct history of dysentery can be a clue in early diagnosis
spread from the pharynx or by aspiration. Later of cutaneous amoebiasis and may be associated with
a chest-wall nodule appears, which may form chronic urticaria.
discharging sinuses. ●● DD: Tuberculosis verrucosa cutis, syphilis,
●● Iliac fossa: This presents as an abdominal mass with lymphogranuloma venereum.
discharging sinuses.
●● DD: Blastomycosis, nocardiosis, tuberculosis,
Cutaneous myiasis
odontogenic abscess (dental cyst). ●● Cutaneous myiasis is an infestation of the skin with

larvae (maggots) of a variety of fly species.

Cutaneous amoebiasis ●● It may present as furuncular, migratory, or wound

●● Cutaneous amoebiasis is caused by Entamoeba myiasis.

histolytica transmitted by feco-oral or sexual routes. ●● Furuncular myiasis: Dermatobia hominis,
●● Skin lesions include one or more lesions that appear at cordylobia, cuterebra species, Wohlfahrtia vigil and
the anus or on the buttocks and spread as sloughing, Wohlfahrtia opaca.
coalescing, and deeply invading ulcers or ulcerated ●● Migratory myiasis: Occurs in those who work
granuloma. with horses (Gasterophilus intestinalis) or cattle
●● It is usually seen as a serpiginous painful ulcer with (Hypoderma bovis). Presentation is similar to
distinct, raised, thickened, often undermined, edges cutaneous larva migrans but the migration is slower
and with an erythematous rim about 2 cm wide, and lasts longer for months.
hemopurulent exudate, and necrotic slough. It is ●● Wound myiasis: Caused by
associated with regional lymphadenopathy. Pus- Cochliomyiahominivorax and infected wounds.
discharging sinuses involving the buttocks, perianal/ ●● DD: Insect-bite reaction, cutaneous larva migrans,

perineal area, and thighs have been described. Vulval delusion of parasitosis, abscess, furunculosis.
340  Draining sinuses and fistulas

Figure 15.10  Atypical mycobacterial infection presenting

as ulcers, sinuses and puckered scarring. (Courtesy: Dr.
Piyush Kumar, Katihar, India.)

Atypical mycobacterial infections

●● The atypical mycobacteria Mycobacterium fortuitum,

Mycobacterium abscessus, Mycobacterium chelonae

are together grouped as the “fortuitum complex,”
atypical mycobacteria that may cause papulopustules,
erythematous subcutaneous nodules (most common
presentation), ulcers, abscesses, discharging sinuses,
or cellulitis. The lesions may at times occur in a
sporotrichoid pattern (Figure 15.10).
●● Trauma, tattoos, and surgery are the major predisposing

factors. Resistance to treatment may also be a feature of

atypical mycobacterial infection.
●● DD: Deep fungal infection, scrofuloderma.

Hidradenitis suppurativa
●● This post-pubertal condition preferentially affects folds

that contain both terminal hair and apocrine glands.

These include the axillae (preferential location in
women) and perineum (preferential location in men).
The incidence is higher in women.
●● It is characterized by recurrent tender nodules that

become fluctuant and suppurate to form sinus tracts.

The scarring is extensive. Bridged comedones are a
characteristic feature (Figure 15.11a,b). Figure 15.11  (a) Hidradenitis suppurativa of the axilla seen
●● It forms a tetrad along with conglobate acne, dissecting as erythematous nodules with sinuses and fluctuant swell-
cellulitis, and pilonidal sinus. ing. (b) Hidradenitis suppurativa of the inguinal region.
●● DD: Actinomycosis, lymphogranuloma venereum,
(a – Courtesy: Dr. Bhushan Madke, Jawaharlal Nehru
Medical College, Datta Meghe Institute of Medical Sciences,
granuloma inguinale, noduloulcerative syphilis. India; b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

Severe acne
●● Nodules may extend deeply over a large area, showing Antitrypsin deficiency panniculitis
little surface involvement. However, if they connect with ●● This is an inherited deficiency of alpha-1 antitrypsin

each other or with deep pustules they may lead to sinus (a protease inhibitor), producing an ulcerating
formation as soon as within 24 hours. The lesions are neutrophilic panniculitis.
tender, chronic, and treatment-resistant and inevitably ●● Lesions present as purpuric, tender nodules and plaques

result in scarring. on the proximal extremities and lower trunk. The

 Draining sinuses and fistulas  341

lesions may become necrotic and break down to form more (secondary) tracts that are interconnected and
ulcers and sinuses that express an oily brown discharge. open through multiple sinus openings.
The disease course is prolonged, and healing occurs ●● DD: Perirectal abscess, hidradenitis suppurativa, anal
with atrophy and scarring. fistula.
●● DD: Factitious panniculitis. (antitrypsin deficiency
panniculitis has lesions more pronounced than those Pyoderma faciale
of trauma); other causes of panniculitis; pyoderma ●● Pyoderma faciale is an uncommon eruptive facial

gangrenosum is differentiated by its necrotic, disease involving post-adolescent women and

undermined border. resembling severe acne or rosacea.
●● It presents as an acute eruption of painful large

Malakoplakia of the skin erythematous coalescing nodules, superficial and deep

●● It is a rare chronic condition in an immunocompromised abscesses, and discharging sinuses, with pronounced
host (especially organ transplant recipients) characterized background erythema and edema of the cheeks, chin,
by granulomatous inflammation that is unable to contain and/or forehead, and may resolve with scars.
a localized bacterial infection (mostly E. Coli) effectively. ●● It differs from acne by the absence of features such as

The characteristic macrophages (von Hansemann cells) comedones, truncal lesions, and infective organisms on
contain intracytoplasmic Michaelis Gutmann bodies culture.
histologically. ●● It differs from rosacea by the absence of flushing and

●● The most common site involved is the genitourinary telangiectatic erythema on the convexities.
tract. Skin involvement occurs due to extension from ●● DD: Acne fulminans, severe rosacea, gram negative

affected internal organ and most commonly affects the folliculitis, Sweet syndrome.
perianal and genital area.
●● It clinically presents as yellowish-pink soft papules, Oral squamous cell carcinoma
erythematous indurated nodules, ulcerations, and ●● This is the most common malignancy of the oral cavity,

abscesses with draining sinuses. seen primarily in middle-aged or elderly men and having
a significant association with tobacco and alcohol. The
Pancreatic panniculitis lateral and undersurface of the tongue and floor of the
●● Pancreatic fat necrosis is a rare complication of mouth are the common sites. Clinical presentation may
pancreatic diseases. All three enzyme categories range from an irregular exophytic lesion to a painless
(lipase, trypsin, and amylase) contribute to the process. non-healing ulcer fixed to deeper tissues.
●● Clinically it resembles other nodular panniculitis ●● Untreated disease may destroy oral tissue and extend

(such as erythema nodosum), with erythematous to the skin on the outer surface of the face to produce a
edematous subcutaneous nodules on the legs more nodular or lobulated growth that appears as a cutaneous
than other areas. discharging sinus.
●● These lesions may become fluctuant with ulcer or sinus ●● DD: Actinomycosis, tuberculosis, nocardiosis,

formation, discharging brown oily material representing blastomycosis.

necrosed adipose tissue.
●● DD: Erythema nodosum, erythema induratum, lupus Hodgkin’s disease
panniculitis, traumatic, alpha-1 antitrypsin deficiency ●● There are few reports of abscess presentation of Hodgkin’s

panniculitis. disease involving liver, lung, chest wall, and brain.

●● Suppurative lymphadenitis and discharging sinuses in
Pilonidal sinus neck region have also been reported in association with
●● This arises in or just above the gluteal cleft, where nodular indurated mass in neck.
the prerequisites of hair-bearing skin, pressure,
and maceration result in chronic inflammation. Crohn’s disease (cutaneous Crohn’s disease)
The pathology initiates when a broken hair follicle ●● Cutaneous Crohn’s disease presents as lesions that are

penetrates the skin to create a sinus tract filled with either metastatic (i.e., distant or non-contiguous with
cellular debris. Pilonidal sinus may be viewed as a respect to the intestinal disease) or non-metastatic (i.e.,
complicated scar with epithelial lined tracts. contiguous with the intestinal disease). Fistulae may be
●● It forms a part of a tetrad along with acne conglobata, found in both forms. Histologically all forms uniformly
dissecting cellulitis of the scalp, and hidradenitis show non-caseating granulomas.
suppurativa. ●● Metastatic:

●● Clinically, it is found in teenagers or young adults. It ●● It presents with genital (labial, scrotal, perianal)
presents as a midline sinus in the sacrococcygeal region or extragenital lesions in the form of dusky
(within 5 cm of the gluteal cleft). Chronic, recurrent erythematous indurated plaques, fissures, ulceration
infection may result in intermittent foul-smelling with undermined edges, draining sinuses, fistulae,
discharge and pain. Abscess formation may result in and scarring.
342  Draining sinuses and fistulas

●● Pain usually means that an abscess has formed ●● It can be categorized into low-, moderate-, or high-
because of blockage of a fistula. output fistulae based on the output of enteric contents.
●● Multiple external openings can be encountered all
over the buttocks, on the scrotum, and on the thigh; a
distinctive sign is the cyanotic hue of the indurated skin. REFERENCES
●● Non-metastatic:
●● It involves parastomal fistula associated with 1. Rook A, Barker J, Bleiker T, Chalmers R, Creamer D, Griffiths C.
Rook’s Textbook of Dermatology. 8th edition. Chichester, West
ileostomy or colostomy for intestinal Crohn’s
Sussex (UK): Wiley Blackwell; 2016.
disease. They are often preceded by abscesses and 2. James W, Berger T, Elston D. Andrews’ Diseases of the Skin. 11th
may be multiple. edition. Philadelphia: Elsevier; 2011, pp. 672–679.
●● They signify recurrent disease. 3. Antaya RJ, Schaffer JV. Developmental anomalies. In: Bolognia JL,
Schaffer JV, Cerroni L, editors. Dermatology. 4th ed. North York:
Enterocutaneous fistula (ECF) Elsevier Limited;2018, pp. 1057–1073.
4. Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Cysts and
●● Most (75%) of ECF are a postoperative complication
Sinuses. In: Dermatology. Berlin, Heidelberg: Springer; 2000.
and the rest are due to other causes (abdominal trauma, 5. Foster MT, Moxon CA, Weir E, Sinha A. Dermal sinus tracts. BMJ
cancer, irradiation, inflammatory bowel disease (IBD), 2019;366:l5202.
ischemic, or infective conditions). 6. Haroun H. Congenital craniofacial and cervical cysts, sinuses and
●● The ilium is the most common site. It may present with fistulas - A review article.. Sch J Oto 2018;1(1).
7. Goldsmith L, Fitzpatrick T. Fitzpatrick’s Dermatology in General
postoperative pain, tenderness, distention, or discharge Medicine. New York: McGraw-Hill Medical; 2012.
of enteric contents from the drain site. Peritonitis is a
potential complication.
 16
Eschar

SUDHIR SINGH

INTRODUCTION
Eschar is a circumscribed, adherent, hard, black piece of dead
tissue (Figure 16.1a) that is moist initially, protein rich, and
avascular. It implies tissue necrosis, infarction, deep burns,
gangrene, or other ulcerating processes. Mostly eschar devel-
ops due to some infectious causes, though it may develop due
to vasculitis, emboli, etc. It is very important to analyze eschar
in a proper context, to reach a definitive diagnosis. For derma-
tologists, the eschar diagnostic approach and clinical clues to
diagnosis are given in Table 16.1 and Table 16.2 respectively.
The salient features of the diseases are discussed below.1-5

Mucormycosis (zygomycosis)
●● Mucormycosis is common in diabetes mellitus

and immunocompromised patients. Infections in

humans are mostly caused by the order Mucorales
(mucormycosis) and include the genera of Mucor,
Rhizopus, Absidia, Rhizomucor, and Cunninghamella.
●● Primary infection can occur by inhalation, by direct

inoculation into damaged skin, or by ingestion. Wounds

and burn injuries are predisposed to primary cutaneous
infection.
●● More often it starts with acute onset of pain and

swelling on or near the eye or nose.

●● The clinicopathologic hallmarks of cutaneous zygomycosis

are vascular invasion, ischemic infarction, and necrosis,

which result in painful erythematous nodules and plaques
that ulcerate rapidly and form central black eschars.
●● Rhinocerebral disease is the most common form of the

disease and starts as acute sinusitis with fever, nasal

congestion, purulent nasal discharge, headache, and
sinus pain. Soon it progresses to involve contiguous
structures over a course of few days. Tissue necrosis
is a prominent feature of the disease and manifests
Figure 16.1  (a) A well-developed eschar seen as thick,
as palatal eschars (Figure 16.1b), destruction of the
well-circumscribed, hard, dark black area of dead skin.
turbinates, perinasal swelling, and cutaneous necrosis (b) Palatal hemorrhage and necrosis in mucormycosis.
with eschar formation and black discharge. (a – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy:
●● Cutaneous mucormycosis follows after trauma
Dr. Raghuraj Hegde, Kempegowda Institute of Medical
(including surgical sites) or wounds. The disease starts Sciences, Bangalore, India.)
DOI: 10.1201/9781351054225-37 343
344 Eschar

Table 16.1  Clinical approach to diseases presenting with Table 16.2  Clinical clues to eschar
eschar
General
General Number state Clues to the diagnosis
state of lesions Diseases
Febrile • Diabetes mellitus – mucormycosis,
Febrile Solitary • Around eye and nose – necrotizing fasciitis (Fournier gangrene)
mucormycosis • Underlying malignant disease, initial lesion
• Extremities – necrotizing fasciitis hemorrhagic vesicle or pustule – ecthyma
• Genital and perineal area – gangrenosum
Fournier gangrene • History of travel to endemic areas – scrub
• Perineum, groin, axilla, neck – typhus, tularemia, plague, anthrax
scrub typhus • Sinonasal or pulmonary disease – invasive
• Hands, fingers, leg, face – anthrax Aspergillosis
• Extremities – tularemia, rat bite • Disease starting as purpura – purpura fulminans
fever, capnocytophaga • History of trauma or surgical interventions –
• Finger, toes, nose – plague Fournier gangrene
A few • Anterior nares, nasal bridge, • History of (H/O) Interventional procedures
palate – Aspergillosis or cardiovascular surgery – cholesterol
• Anogenital region, extremities – embolization syndrome
ecthyma gangrenosum • Infiltrated skin lesions – Lucio’s phenomenon
• Toes and feet – cholesterol • Rash with migratory polyarthralgia – rat-bite
embolization syndrome fever
• Extremities – purpura fulminans, • H/O splenectomy, alcohol-induced liver
phaeohyphomycosis, disease – capnocytophaga
hyalohyphomycosis • H/O HIV/organ transplant – amebic infections
Afebrile Solitary • Extremities – antiphospholipid • Immunosuppressed patient –
antibody syndrome phaeohyphomycosis
• Mastectomy skin flaps/reverse • Immunocompromised hosts –
sural artery flaps – flap necrosis hyalohyphomycosis
• Breast, buttocks, abdomen, Afebrile • Cardiovascular diseases – arterial insufficiency,
thigh – warfarin necrosis • Arterial and venous thrombosis and
• Subcutaneous injection site – pregnancy loss – antiphospholipid antibody
heparin necrosis syndrome
• Extremities snakebite, spider • Renal disease – calciphylaxis
bite (Loxosceles) • Flap surgery – flap necrosis
• Face and extremities – amebic • Involvement of pressure prone areas –
infections decubitus ulcer
A few • Abdomen, thigh, and hips – • On treatment with anticoagulants –
calciphylaxis warfarin/ heparin necrosis
• Extremities – Lucio’s phenomenon • White/bluish skin with numbness – frostbite
• Feet, hand, ears, lips, nose – • Burn – thermal or chemical burn
frostbite • Bite – snakebite, Loxosceles bite
• Over sacrum, ischial tuberosity,
greater trochanter, heel –
decubitus ulcer Necrotizing fasciitis
●● It is a life-threatening infection by group A

Streptococcus, involving soft tissue and fascia.

as painful cellulitis-like lesions, progressing rapidly ●● It is commonly seen in diabetes, immunosuppressive

with eschar formation. conditions, chronic alcoholics, and smokers.

●● Post-traumatic mucormycosis is an uncommon variant; ●● In the early stage, pain (out of proportion to skin

it differs from cutaneous mucormycosis in having a rare involvement), swelling, and erythema develop at the
association with diabetes mellitus, mostly localized to site; these progress rapidly to dusky bullous lesions in
the skin, and having a better prognosis. one to three days (Figure 16.2a).
●● It is caused by various species (Apophysomyces elegans ●● DD: Often confused with cellulitis (Figure 16.2b)

complex and Saksenaea vasiformis). and abscesses; pain out of proportion to erythema
●● A history of trauma following traffic accidents (37%), tenderness beyond involved area, indistinct margin,
domestic violence (15.1%), or natural disasters (13.4%) is and near absence of lymphangitis differentiate early
present. necrotizing fasciitis from other entities.
 Eschar 345

Fournier gangrene
●● It is a type of necrotizing fasciitis affecting genital

or perineal skin and soft tissue following trauma or

instrumentation.
●● It is commonly seen in diabetes patients in the fifth to

the sixth decade with a male to female ratio of 10–25:1.

●● Cultures commonly show polymicrobial infections

by aerobes and anaerobes, which include Escherichia

coli, Staphylococcus aureus, Streptococcal species, and
Pseudomonas aeruginosa.
●● The scrotum, penile shaft, perineum, and abdomen

are involved commonly in men, whereas in women the

vulva and perineum are affected (Figure 16.3).
●● Initially, patients develop pain and swelling, which

progress rapidly to tissue ischemia and eschar formation.

Rickettsia infection4,6
●● Rickettsiae now include a polyphyletic group of

microorganisms in the class proteobacteria, comprising

species belonging to the genera Rickettsia, Orientia,
Ehrlichia, Anaplasma, and Neorickettsia.
●● Rickettsiae are separated into the spotted fever group and

the typhus group based on common genetics, immunologic

patterns, and intracellular growth characteristics.
●● Spotted fever rickettsiae are injected into the host

through the saliva of the feeding tick, whereas typhus

group rickettsiae enter through the feces of infected
human body lice or fleas.
●● There are two major families: the Ixodidae (hard ticks)

and the Argasidae (soft ticks).

Figure 16.2  (a) Necrotizing fasciitis with eschar and ulcer.

(b) A severe case of cellulitis with eschar. (a,b – Courtesy: Figure 16.3  Fournier gangrene. (Courtesy: Dr. Rajesh Kumar
Dr. Piyush Kumar, Katihar, India.) Mandal, North Bengal Medical College, Darjeeling, India.)
346 Eschar

●● Dermacentor ticks typically attach to the head and neck or ●● The cutaneous form of tularemia in humans is caused
the upper trunk. Ixodes ticks tend to attach on the trunk, by direct contact with an animal reservoir and tick
whereas Amblyomma ticks prefer the lower extremities. bites or exposure to a contaminated hydro-telluric
●● Rickettsial diseases of dermatological importance are environment.
summarized in Table 16.3. ●● Lesions are located on the upper part of the body
(extremities).
Tularemia 7
●● In ulceroglandular tularemia, a painful red papule
●● Tularemia is caused by Francisella tularensis, a gram-
appears at the inoculation site that evolves rapidly into
negative coccobacillus found in rabbits and rodents and a necrotic chancreform ulcer often covered by a black
reported mainly in the northern hemisphere. eschar. Regional lymph nodes are large and tender.

Ecthyma gangrenosum8,9
●● This is most commonly caused by Pseudomonas

aeruginosa (73%), other bacteria (Escherichia coli,

Aeromonas, Klebsiella pneumoniae, Haemophilus
influenzae), as well as fungi (Candida, Aspergillus
species, Fusarium).
●● It occurs in immunocompromised patients with severe

neutropenia, particularly those with an underlying

malignant disease.
●● It is described in two types: localized (non-septicemic)

form, in which lesions are localized at the site of

inoculation of the organism into the skin, and classical
(bacteremic) form due to hematogenous spread.
●● It is most commonly located in the anogenital area or

on the extremities.
●● Lesions progress rapidly within 12 to 24 hours from

erythematous or purpuric macules to hemorrhagic

vesicles or bullae, which rupture and become necrotic
ulcers with a central black eschar. The surrounding
tissue is erythematous and tender (Figure 16.4a–c).

Figure 16.4  (a) Ecthyma gangrenosum. Central black eschar with surrounding erythematous tissue. (b) Ecthyma gangreno-
sum. Necrotic ulcers with a central black eschar and erythematous halo. (c) Multiple lesions of ecthyma gangrenosum in a
child due to sepsis following an episode of varicella. (c – Courtesy: Dr PC Das and Dr Piyush Kumar, Katihar, India.)
 Eschar 347

Table 16.3  Rickettsial diseases

Incubation
Disease Vector/ reservoir period Clinical features Systemic features
Scrub typhus Trombiculid mite 1–2 weeks Eschar is common in warm, damp High grade fever
(Orientia (Leptotrombidium areas of the body (perineum, (40°–40.6°C).
tsutsugamushi) deliense)/ groin, and axilla). Hepatosplenomegaly
(Figure mites and the An erythematous papule appears and relative
16.5a–c) rodents within two days of the chigger bradycardia
bite, which ulcerates and forms pneumonitis may
eschar in 50–70% patients. develop.
The regional lymph nodes are Deafness and tinnitus
enlarged and tender. occur in about 20%.
Rocky Dermacentor variabilis >2–14 days Males; adults 40–64 years, Neurologic disease,
Mountain (dog tick), children <10 years. thrombocytopenia,
spotted fever Dermacentor Rash begins on ankles, wrists, and acute renal failure,
RMSF andersoni forehead and spreads hepatitis.
(Rickettsia (wood tick), centripetally. The classic triad of fever,
rickettsii) Amblyomma Erythematous blanching macules rash, and history of
americanum (lone and papules 2–4 days after fever tick bite. 
star tick), onset; and may be hemorrhagic.
Rhipicephalus Originally called “black measles.” 
sanguineus (brown
dog tick)/pet dog
Mediterranean Rhipicephalus 5–7 days Tache noir, occurring in about Mild disease (mainly in
spotted fever sanguineus (brown 13%–68% as an erythematous, children) has a good
(Rickettsia dog tick) indurated papule with a central prognosis.
conorii) necrotic eschar. Lymphadenitis;
African tick bite Amblyomma Rash may be morbilliform or distinguish ATBF from
fever ATBF (R. hebraeum and vesicular. RMSF
africae) Amblyomma Multiple tick bites, multiple eschars
variegatum tick (seen in 50% of patients with
ATBF)
Rickettsial pox Liponyssoides 7 days It starts as a painless, Clinically resembles
(Rickettsia sanguineus rodent (eschar), erythematous, indurated papule varicella (chickenpox)
akari) mite/house mouse, 7–24 days at bite site and evolves into a Self-limiting
Mus musculus (systemic vesicle, followed by eschar
symptoms formation.
and rash) Regional lymphadenopathy is
common.
Endemic Xenopsylla cheopis 1–2 weeks Extremely high fever, 41.1°C Thrombocytopenia,
typhus (rat flea), (12 days) (106°F), malaise, headache, rash transaminitis, and
(Rickettsia Ctenocephalides after 4–5 days hyponatremia are
typhi; Rickettsia felis (cat flea) Erythematous macules and/or common, though
felis) papules involving the trunk more usually mild
than the extremities.
Palms and soles are rarely involved.
Epidemic Pediculus humanus 1–2 weeks Starts as erythematous macules Acral gangrene, cerebral
typhus var. corporis (human (8 days, over axillary folds and upper thrombosis may occur.
(Rickettsia body louse), average) trunk after 4–5 days, which Brill–Zinsser disease
prowazekii) Neohaematopinus become papular and petechial. (BZD) can occur in
sciuropteri lice, Lack of eschar survivors who may
Orchopeas The eruption spreads centrifugally suffer recrudescent of
howardii fleas but spares the face, palms, and the previous infection
soles.
Predilection for colder months
348 Eschar

●● DD: Noma caused by Fusobacterium, which occurs

in young debilitated infants; septic emboli due to
organisms such as E. coli, Aeromonas hydrophila,
Burkholderia cepacia, Candida, Fusarium, Aspergillus,
and other saprophytic fungi.

Anthrax
●● Anthrax is caused by Bacillus anthracis.

●● It can present in four forms: cutaneous (95%),

inhalational (known as woolsorter’s disease),

gastrointestinal, and injectional (seen in IV drug use,
primarily in heroin addicts).
●● Cutaneous anthrax transmission (approximately 95%

of cases) is by the introduction of spores via breaks in

exposed skin. The sites involved are exposed parts of the
hands, legs, and face.
●● Incubation period is about three to seven days.

●● It presents initially as an anti-inflammatory papule that

rapidly develops into a bulla surrounded by intense

edema and infiltration within another 24 to 36 hours. It
then ruptures spontaneously, and a dark-brown or black
eschar is visible, surrounded by vesicles situated on an
indurated area (Figure 16.6).
●● The lesion is painless and non-tender, associated with tender

suppurative regional adenitis. Pustules are seldom present.

●● DD: Staphylococcal carbuncle (prominent tenderness),

brown recluse spider bite.

Plague10
●● It is a flea-borne zoonosis caused by Yersinia pestis,

associated with the term “the Black Death.”

●● Transmission to humans occurs through the bite of

infected fleas.
●● In humans, it classically presents with three forms:

bubonic (80–85% cases), septicemic, and pneumonic.

●● A skin lesion may develop at the site of the bite in 25%

cases, especially over fingers, toes, or the nose, and

often go unnoticed. The patient may develop a pustule,
vesicle, papule, or an eschar.
●● Within a few days, painful regional lymphadenopathy

develops, involving single or multiple nodes. Most

commonly inguinal nodes, followed by axillary or
cervical nodes, are involved.

Aspergillosis
●● Invasive infection with Aspergillus is classically seen

during acute periods of neutropenia in hematopoietic

stem cell transplant patients, certain hematologic
malignancies, GVHD, cytomegalovirus infection, and
solid-organ transplant patients.
●● A. fumigatus is the most common cause of disseminated

infections, although emerging strains of A. flavus, A.

Figure 16.5  (a) Scrub typhus with erythematous plaque
niger, and A. terreus are accounting for more disease.
with central eschar. (b) Well-developed eschar in scrub
●● Cutaneous manifestations of disseminated Aspergillosis
typhus. (c) Scrub typhus. (a – Courtesy: Dr. Kalyan
Ghosh, Darjeeling, India; b – Courtesy: Dr. Kilangwabang occur in only 5%–10% of patients.
Pongener, Nagaland, India; c – Courtesy: Dr. Kilangwabang ●● Aspergillosis should be suspected in patients presenting

Pongener, Nagaland, India.) with pneumonia, CNS involvement, and nasal passage
 Eschar 349

Purpura fulminans
●● Purpura fulminans is usually seen in relation to acute

sepsis, idiopathic post-infectious form, and in neonates.

●● In acute sepsis-associated or secondary purpura

fulminans, infection with Neisseria meningitidis,

Staphylococcus aureus, groups A and B hemolytic
streptococci, Streptococcus pneumoniae, and
Haemophilus influenzae.
●● Postinfectious purpura fulminans can manifest within

10 days of an antecedent illness, most commonly

following streptococcal or varicella infections in children.
●● Neonatal purpura fulminans are most often seen in

association with homozygous protein C deficiency.

●● Cutaneous microvascular occlusion in sepsis with

DIC presents clinically as ecchymosis, usually with

a retiform, stellate, or branching configuration and
symmetrical gangrene of extremities. Patients develop
non-blanching tender purpura with erythematous halo
and indurated borders with rapid transition to necrosis
and eschar (Figure 16.7).
●● DD: Catastrophic antiphospholipid antibody syndrome.

Cholesterol embolization syndrome

●● Also known as blue or purple toe syndrome, this is an

entity in which cholesterol crystal or atheroma debris

(cholesterol, platelet, and fibrin) embolizes distal arteries.
●● It is commonly seen in elderly patients after

endovascular procedures (valve replacement or stent

placement) but can occur spontaneously.
●● Constitutional features, such as fever, anorexia, myalgia and

weight loss, may be seen. It commonly affects the kidneys

(31.5%), skin (15.5%), and gastrointestinal tract (13.4%).
●● The most common skin finding is livedo reticularis;

blue or purple toes, ulcers, gangrene, and purpuric

lesions are also seen.
●● DD: In arterial thromboembolism onset is acute

and leads to abrupt organ dysfunction, whereas in

Figure 16.6  Anthrax. Eschar with a rim of induration.

(Courtesy: Dr Ajay Rai, Lucknow, India.)

and paranasal sinuses disease that can develop ulcer

followed by an eschar.
●● Lesions occur in the anterior nares and on the nasal
septum, palate, and skin overlying the nasal bridge.
Patients with disseminated Aspergillosis often present
with unremitting fever despite antibiotic use.
●● Lesions begin as single or multiple painful, erythematous
papules, nodules, or plaques. They rapidly expand and
develop central hemorrhagic vesicles or bullae, then eschar. Figure 16.7  Purpura fulminans following disseminated
●● In burn victims, Aspergillus typically colonizes the intravascular coagulation (DIC). (Courtesy: Dr. Piyush
burn eschar. Kumar, Katihar, India.)
350 Eschar

cholesterol embolization syndrome onset is subacute ●● DD: Antiphospholipid antibody syndrome and
or chronic and causes slow end organ dysfunction. necrotic ENL (Figure 16.8b). ENL is associated with
Another distinguishing feature of cholesterol lymphadenopathy and arthritis. Bacteriological and
embolization syndrome is that arterial pulsations are morphological indices show high positivity in Lucio’s
palpable as it involves smaller arteries and arterioles. phenomenon.

Lucio’s phenomenon Arterial insufficiency (ischemic) ulcer

●● Lucio’s leprosy (LuLp) is a diffuse form of LL, ●● This is generally seen in hypertensives, smokers,

commonly seen in Mexico and Costa Rica. diabetic, and hyperlipidemia patients. In a progressive
●● The skin looks waxy and shiny, as there is diffuse disease, the diagnosis of thromboangiitis obliterans, or
infiltration of skin without the previous appearance of Buerger’s disease, should be considered.
discrete lesions. ●● The ratio of the popliteal to brachial pressure is called

●● Infiltration of the ear lobes and forehead, and loss the ankle-brachial index (ABI); if it is less than 0.75,
of eyebrows and sometimes eyelashes give a mask- arterial insufficiency exists; and if it is less than 0.5, the
like appearance known as lepra bonita, or beautiful insufficiency is substantial.
leprosy. Lucio’s phenomenon may be seen in ●● Ischemic ulcers are mostly located on the lateral surface

untreated patients. of the ankle or the distal digits.

●● It is characterized by multiple, well-defined, irregular ●● The initial red, painful plaque breaks down into a

purpuric lesions evolving into massive ulcerations, painful superficial ulcer with a surrounding zone of
involving the extremities and healing with atrophic purpuric erythema (Figure 16.9a,b).
scarring (Figure 16.8a). ●● Granulation tissue is minimal; little or no infection is

present; and a membranous inactive eschar forms over

the ulcer.
●● Associated findings include decreased or absent pulses,

coolness of the extremity, and claudication on exercise.

●● DD: Cholesterol emboli.

Antiphospholipid antibody/lupus anticoagulant

syndrome (APLS)
●● It is characterized by venous thrombosis, arterial

thrombosis, pregnancy loss, or thrombocytopenia in

conjunction with the presence of antiphospholipid
antibodies.
●● It is mostly seen in young to middle-aged patients, with

a strong female predominance.

●● APLS may occur as a primary or secondary disorder.

●● Primary APLS comprises 53% of cases. Secondary

antiphospholipid syndrome can be associated

with systemic lupus erythematosus, malignancy,
lymphoproliferative disorders, infections, drugs, and
autoimmune diseases.

Figure 16.8  (a) Lucio’s phenomenon. Multiple well-defined round to oval and geographic ulcers covered with eschar.
(b) Erythema nodosum leprosum necroticans with multiple ulcers covered by eschars. (a – Courtesy: Dr. Vikrant Saoji,
Nagpur, India; b – Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)
 Eschar 351

Figure 16.9  (a) Arterial insufficiency ulcer. Membranous

eschar over the ulcer with a surrounding zone of purpu-
ric erythema. (b) Arterial insufficiency ulcer with eschar
of distal foot. (a – Courtesy: Dr. Ajay Rai, Lucknow, India;
b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● The most common finding is livedo reticularis found

on the extremities; other findings include leg ulcers,
pseudovasculitis, digital gangrene, cutaneous necrosis,
and splinter hemorrhages.
●● Catastrophic APLS is an uncommon variant that
presents with widespread cutaneous necrosis and
multiorgan failure, especially renal and pulmonary.

Calciphylaxis/calcific uremic arteriolopathy

●● Also known as calciphylaxis, this is a rare but life-

threatening cutaneous condition associated with

hyperparathyroidism.
●● Calciphylaxis is seen in patients of chronic renal failure,

secondary hyperparathyroidism, alcoholic liver disease,

and occasionally after chemotherapy, even in patients
with normal renal function.
●● Peak incidence is after the sixth decade, more

commonly in females (>80%). Calciphylaxis results

from calcium deposition in the walls of small- to
medium-sized vessels, resulting in vascular occlusion
and skin necrosis.
●● Typical sites involved are abdomen, thighs, and hips,

but breasts may be involved.

●● Patients initially develop violaceous patches that may

exhibit a mottled or reticular pattern that become

indurated and painful, with eventual ulceration and Figure 16.10  Calciphylaxis with reticulate purpura and
eschar formation that proceeds to tissue gangrene cutaneous necrosis (eschar). (Courtesy: Dr. Neethu Mary
(Figure 16.10). George, Sri Siddhartha Medical College, Tumkur, India.)
352 Eschar

●● A calcium-phosphate product of greater than 70 mg/dL Table 16.4  European Pressure Ulcer Advisory Panel
has often been cited as a risk factor. (EPUAP) classification scheme
●● DD: Hyperoxaluria.
Stage I Non-blanching erythema of intact skin
Flap necrosis 11
Stage II Partial-thickness skin loss involving epidermis
●● Local flap reconstruction may cause complications and/or dermis
related to ischemia, tension, hematologic, or infectious Stage III Full-thickness skin loss with necrosis of
cause. subcutaneous tissue down to but not through
●● The incidence of mastectomy skin flaps ranges from underlying fascia
5%–30%, but in dermatologic surgeries, the incidence of Stage IV Full-thickness with penetration to fascia,
flap necrosis is lower. muscle, bone
●● Skin flap viability is influenced by surgical and

patient factors (smoking, diabetes, hypertension, and

obesity). Compromised skin flap perfusion can lead ●● The risk of coumadin necrosis is increased if loading
to necrosis.
doses (10 mg or more) of warfarin are used and if a
●● For improving flap survival, minimize tissue
second form of anticoagulation such as heparin therapy
undermining and dissection, or use a delayed
is not used to cover the initial phase of anticoagulant
phenomenon.
therapy.
Pressure ulcer (decubitus ulcer) ●● The peak incidence is between the sixth and seventh
●● Defined as a localized area of necrosis caused by
decades and has a male:female ratio of 1:4.
●● Sites involved are the breasts (Figure 16.12), buttocks,
ischemia, this results from compression of soft tissue
abdomen, and thighs, with abundant fat and reduced
between a bony prominence and an external surface.
●● The predominance is 80% superficial ulcers and 20%
blood supply.
●● Coumadin necrosis usually presents as the
deep ulcers. The sacrum is the site of 60% of all sores are
sudden onset of pain within affected areas, followed
over, while the ischial tuberosities, greater trochanter,
by well-demarcated erythema, progressing to
and heel account for 15%.
●● The lesion evolves from blanchable erythema to
hemorrhage, necrosis, and often hemorrhagic bullae
or eschar.
necrosis of all tissues down to and including bone
●● DD: Although coumadin necrosis may rarely involve
and joints. When pressure is relieved, sores go
acral areas, acral cutaneous purpura in patients on
through a process of debridement, where necrotic
coumarin is more likely to be due to cholesterol
slough forms a hard dry black eschar (Figure 16.11,
embolus – so-called purple (blue) toe syndrome.
Table 16.4).

Coumadin (coumarin, warfarin) necrosis

●● Lesions usually develop within 10 days of starting

coumarin therapy in approximately 1 out of 10,000

patients.

Figure 16.12  Warfarin necrosis with superficial eschar and

Figure 16.11  Pressure ulcer covered with eschar. surrounding erythema. (Courtesy: Dr. Shekhar Neema,
(Courtesy: Dr. Piyush Kumar, Katihar, India.) Armed Forces Medical College, Pune, India.)
 Eschar 353

Heparin necrosis
●● Cutaneous heparin necrosis is thought to occur as

part of the heparin-induced thrombocytopenia (HIT)

syndrome in at least 22% of patients with heparin-
induced skin lesions.
●● A heparin-dependent antiplatelet antibody is the

pathogenic basis of HIT and apparently of heparin-

induced skin necrosis. This antibody causes both
thrombocytopenia and the aggregation of platelets in
vessels, leading to thrombosis (white-clot syndrome).
●● Unfractionated heparin is three times more likely to

be associated with HIT following orthopedic surgery

compared with low molecular-weight heparin.
●● Lesions appear around five to seven days after initiation

of heparin. Lesions are most common at subcutaneous

injection sites but can occur elsewhere.
●● Heparin administration commonly induces ecchymoses,

purpuric tender, sharply demarcated plaques sometimes

with retiform extensions. Erythema may sometimes
accompany such lesions, and necrosis is frequent.
●● Unlike skin necrosis induced by warfarin, heparin-

induced skin necrosis can recur if heparin is restarted,

as it is an immune-related condition.

Burns
●● Due to different modalities such as thermal, chemical,

electrical, or radiation, burns usually present acutely

to clinicians. Burn depth usually is determined by the
intensity of the source, duration of contact, and location
on the body.
●● Heat (and other injury mechanisms) can denature

proteins, leading to loss of plasma membrane integrity

and cell necrosis (Figure 16.13).
●● Burns may be classified according to the depth of

thermal injury (Table 16.5).

Frostbite12
●● Tissue damage resulting from exposure to sub-zero
Figure 16.13  Electric burn. Dry, waxy irregular eschar over
the scalp. (Courtesy: Dr. Ajay Rai, Lucknow, India.)
degree Celsius temperature is known as frostbite or
freezing cold injury.
●● Sites prone to develop frostbite are feet, hands, ears, lips, ●● Within 4 to 24 hours upon rewarming superficial
and nose. frostbite develops pale white blisters, while deep
●● Initially, the skin becomes white and blanched, along frostbite may show hemorrhagic blisters or gangrenous
with numbness. Later the exposed part becomes dark changes.
and purplish. ●● Eschar will be evident in 10 to 15 days.

Table 16.5  Classification of burns

Type Depth of thermal injury Sensation Morphology

Superficial burns Epidermis Painful Erythematous, blanch on pressure
(first degree)
Partial-thickness burns Epidermis and dermis Painful Pink or cherry red, blister, blanch on
(second degree) pressure
Full-thickness burns Epidermis, dermis, and Not painful Form an eschar, appear dry and
(third degree) subcutaneous tissue leathery, can feel firm and waxy on
palpation, and is non-blanching 
354 Eschar

Loxosceles13
●● Bites of the brown recluse spider or fiddle-back spiders

(Loxosceles reclusa) vary from mild, local reactions to

severe ulcerative necrosis, a reaction known as necrotic
arachnidism.
●● In necrotic cutaneous loxoscelism, systemic symptoms

are mild. Within minutes or hours, severe pain develops

at the site of the bite, accompanied by erythema, edema,
and a central bulla.
●● The hallmark – central blue/purple discoloration –

develops, surrounded by an ischemic halo and an outer

ring of erythema (the “red, white, and blue” sign). The
initial wound may progress to become necrotic, and an
eschar develops within three or four days. Eventually,
deep ulcers develop (Figure 16.15).

Figure 16.14  Chromic induced ulcers and eschars on the

hands. Note pigmentation of the nails. (Courtesy: Dr.
Piyush Kumar, Katihar, India.)

Chemical burns
●● It results in irreversible cell damage and necrosis at the

site of exposure. There is usually a rapid onset of painful

erythema, often within minutes.
●● The dead skin turns brown and later black, usually without

blistering, and forms a hard eschar (Figure 16.14).

●● Phenol (carbolic acid) is a protoplasmic poison that

produces a white eschar on the surface of the skin. It

can penetrate deep into the tissue.
●● Most acids (e.g., sulphuric, nitric, hydrochloric,

chromic) coagulate skin proteins.

●● Some acids can discolor (e.g. nitric acid turns the skin

yellow).
●● Alkalis (e.g., sodium, calcium, potassium hydroxides;

wet concrete; sodium, and potassium cyanides) degrade

lipids and penetrate deeper into the skin. The dead skin
turns brown and later black, usually without blistering,
and forms a hard eschar.

Snakebite
●● Signs and symptoms of snakebite depend upon the

species of snake, so identification of snake is essential.

●● Venomous snakes belong to three major families, Elapidae

(cobras, kraits, adders, mambas, coral snakes), Viperidae

(rattlesnakes, vipers), and Hydrophiidae (sea snakes).
●● The venom of elapid snakes is highly neurotoxic, and

some species, such as Naja spp. Cobras, have additional

cytotoxicity. Viper venoms are unique for causing bite-
site swelling and tissue destruction.
●● Local effects, including skin blistering, limb swelling,

and tissue necrosis, are caused by vipers. Local

blistering can lead to necrosis of full skin thickness,
which can get secondarily infected. Figure 16.15  Loxosceles with thick adherent eschar.
●● Puncture marks at the wound help in the diagnosis of (Courtesy: Dr. Shekhar Neema, Armed Forces Medical
snakebite. College, Pune, India.)
 Eschar 355

●● DD: Pyoderma gangrenosum, erythema migrans of ●● Disseminated disease may also begin as a skin infection,
Lyme disease, cutaneous anthrax, and chemical burns. although catheter sepsis is a recognized cause of
disseminated infection.
Tegenaria ●● The lesions usually appear as dry, black, leathery
●● Tegenaria agrestis, the hobo spider or “aggressive house eschars with a scalloped, erythematous, edematous
spider,” is the predominant cause of necrotic arachnidism. border.
●● The local cutaneous effects are similar to those caused

by the brown recluse. Induration and paresthesia of the Hyalohyphomycosis

bite site may develop within 30 minutes. ●● Hyalohyphomycosis is caused by Penicillium,
●● Vesicle formation often occurs during the first 36 hours. Acremonium, Trichoderma, Scedosporium, and
Eschar formation may follow in severe cases, with Paecilomyces.
necrosis and sloughing of the underlying tissue. ●● They generally do not cause the disease except

in immunocompromised patients. Localized

Amebic infections hyalohyphomycosis has also occurred in
●● Amebas of the genera Acanthamoeba, Naegleria, and immunocompetent patients after traumatic
Balamuthia may also cause skin lesions. Infection is implantation.
more common in HIV-positive patients and organ ●● There is no classic clinical morphology to the
transplant patients. lesions, but keratotic masses, ulcerations, ecthyma
●● The extremities (lower more than upper) and the face
gangrenosum-like lesions, erythematous nodules,
are commonly involved. dark eschars, and disseminated erythema have been
●● Lesions present as pink or violaceous nodules that then described.
enlarge, suppurate, and form ulcers with a necrotic eschar.
●● DD: Pyoderma gangrenosum. Injuries caused by fire coral and coral cuts
●● Coral injuries may be caused by nematocyst stings or
Rat-bite fever (sodoku, spirillum minor)
lacerations.
●● Caused by a spiral flagellate organism
●● In contrast to the stings of true corals, the sting of the
Streptobacillus moniliformis, and is transmitted by the fire coral, Millepora alcicornis, is quite painful.
bite of a rat. It can be transmitted through food or water ●● Within one to several hours a pruritic erythematous
contaminated with feces or urine. papular eruption appears, which in severe cases may
●● Inflammation and an eschar may form at the wound site
become pustular and, in rare cases, may progress to
within 10 to 12 days, accompanied by fever, malaise, and necrosis and eschar formation.
regional lymphadenopathy. Approximately 75% of patients ●● Lesions heal in 1 to 2 weeks, often with post-
develop the maculopapular, petechial, or purpuric rash. inflammatory hyperpigmentation.
●● As the infection progresses, 50% of individuals will

develop migratory polyarthralgia and myalgia in large Cowpox

and small joints of extremities. ●● This is caused by orthopoxvirus after contact with

infected cats or cattle.

Capnocytophaga (formerly dysgonic fermenter ●● Sites involved are usually the hands, arms, or face.
type 2 [DF2]) ●● A lesion evolves from a painful papule within five to
●● Capnocytophaga canimorsus is part of the normal oral seven days at the inoculation site and becomes vesicular
flora of healthy dogs and cats. and hemorrhagic, which ulcerates within two weeks,
●● Capnocytophaga infections occur following exposure to and forms crusts with a hard black indurated eschar
or bites from dogs. 1–3 cm in diameter. Healing takes place in four to eight
●● Necrotizing eschar at the site of the bite is a weeks and usually leaves scarring.
characteristic finding. Cellulitis, non-specific macular ●● Constitutional symptoms and lymphangitis are

or maculopapular lesions, erythema multiforme, common.

petechiae, purpura fulminans, and symmetrical ●● DD: Orf and milker’s nodules as well as primary

peripheral gangrene may be seen. tuberculosis, anthrax. In anthrax, the eschar forms
by day six. The amount of surrounding edema and
Phaeohyphomycosis induration is much more marked than in orf. Scarring
●● Phaeohyphomycosis is caused by dematiaceous fungi is common after cowpox.
Exophiala jeanselmei.
●● Subcutaneous disease occurs most frequently as Milker’s Nodule
indolent abscesses at the site of minor trauma (so-called ●● Milker’s nodule is caused by the paravaccinia virus

“phaeomycotic cyst”). (also called pseudocowpox virus) in individuals in close

●● Systemic phaeohyphomycosis is caused by contact with cattle.
Bipolaris spicifera and Scedosporium prolificans in ●● Incubation period for the milker’s nodule is four to

immunocompromised patients. seven days.

356 Eschar

●● It commonly occurs on the hands, occasionally on the face. 4. Prakash JAJ. Scrub typhus: Risks, diagnostic issues, and manage-
ment challenges. Res Rep Trop Med 2017 Aug 7;8:73–83.
●● Lesions start as red or pruritic macules and then
5. Wysong A, Venkatesan P. An approach to the patient with retiform
evolve into papules and papulovesicles with a target- purpura. Dermatol Ther 2011 Mar–Apr;24(2):151–172.
like appearance. The lesions then develop into bluish 6. Weerakoon K, Kularatne SA, Rajapakse J, Adikari S, Waduge R.
or violaceous tender nodules. Some ulcerate or have a Cutaneous manifestations of spotted fever rickettsial infections in
central depression, which results in the formation of the Central Province of Sri Lanka: A descriptive study. PLoS Negl
Trop Dis 2014 Sep 18;8(9):e3179.
eschars with a crust. Lesions heal in four to eight weeks.
7. Maurin M, Gyuranecz M. Tularaemia: Clinical aspects in
Lymphangitis is often present. Lymphadenopathy is not Europe. Lancet Infect Dis 2016;16(1):113–124. doi:10.1016/
common. S1473-3099(15)00355-2.
●● DD: The lesion of anthrax is more hemorrhagic, with 8. Vaiman M, Lazarovitch T, Heller L, et al. Ecthyma gangrenosum
rapid progression to an eschar. Tularemia and syphilis and ecthyma-like lesions: Review article. Eur J Clin Microbiol
Infect Dis 2015;34:633–639.
are associated with chancres.
9. Martínez-Longoria CA, Rosales-Solis GM, Ocampo-Garza J,
Guerrero-González GA, Ocampo-Candiani J. Ecthyma gangreno-
REFERENCES sum: A report of eight cases. An Bras Dermatol 2017;92(5):698–
700. doi:10.1590/abd1806-4841.20175580.
1. Dunn C, Rosen T. The rash that leads to eschar forma- 10. Dillard RL, Juergens AL. Plague. In: StatPearls. Treasure Island (FL):
tion. Clin Dermatol 2019;37(2):99–108. doi:10.1016/j. StatPearls Publishing; August 10, 2020.
clindermatol.2018.12.003. 11. Robertson SA, Jeevaratnam JA, Agrawal A, Cutress RI.
2. Liaqat M, Halpern AV, Green JJ, Heymann WR. The Rickettsioses, Mastectomy skin flap necrosis: Challenges and solutions. Breast
Ehrlichioses, and Anaplasmoses. In: Kang S, Amagai M, Bruckner Cancer (Dove Med Press) 2017;9:141–152. Published 2017 Mar 13.
AL, Enk AH, Margolis DJ, McMichael AJ, Orringer JS, editors. 12. Basit H, Wallen TJ, Dudley C. Frostbite. In: StatPearls. Treasure
Fitzpatrick’s Dermatology. 9th edition. New York, NY: McGraw-Hill Island (FL): StatPearls Publishing; June 30, 2020.
Education; 2019. P. 3306–3323. 13. Tibballs J, Winkel KD. Envenomation Syndromes. In: Shaffner DH,
3. Monsel G, Delaunay P, Chosidow O. Arthropods. In: Griffiths Nichols DG, editors. Rogers Textbook of Paediatric Intensive Care.
CEM, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook’s 5th edition. Philadelphia: Wolters Kluwer; 2016. P. 515–540.
Textbook of Dermatology. 9th edition. West Sussex: John Wiley &
Sons; 2016. P. 34.1–34.55.
 17
Cutaneous horn

SATISH S SAVANT, SUSHIL S SAVANT

INTRODUCTION ●● They are found to be more prevalent in sun-

exposed areas, with underlying various benign
●● Cutaneous horn (Cornu cutaneum) is a morphological (61.1%), premalignant (23.2%), and malignant
designation referring to an elongated, densely (15.1%) conditions (Table 17.1). Clinical importance
compacted hyperkeratotic conical projection above the attached to cutaneous horn is in detection of type of
surface of the skin, overlying any of the wide variety of underlying lesions.1–4
cutaneous lesions and resembling a miniature horn of
an animal but differing from it by having a cornified CLINICAL FEATURES1-6
instead of bony core.
●● Its peak occurrence is reported in people aged 50 to ●● Individuals with fair to light-skinned complexions
mid-70s because cutaneous horns with underlying are more susceptible, though it can be seen in all
premalignant and malignant lesions are more common populations.
in this age group. ●● They are found more commonly over sun-exposed areas
of the body (approximately 30%), such as the scalp,
Table 17.1  Conditions associated with cutaneous horn upper face, peri-orbicular, ear, lips, neck, chest, and
shoulders. However, they are also found over areas not
Condition Diseases
exposed to sun, such as penis, mucosa of the lower lip,
Benign Verruca vulgaris, seborrheic keratosis, and nasal vestibule.
pyogenic granuloma, molluscum ●● Cutaneous horns are well-circumscribed keratinous
contagiosum, fibroma, epidermal cyst, in white, yellow, pink, brown, black (mixtures of various
fundibular cyst, dermatofibroma, shades) projections situated above the skin surface
angioma, angiokeratoma, benign over the underlying benign premalignant or malignant
lichenoid dermatosis, epidermolytic lesions (Figures 17.1 to 17.7).
acanthoma, verrucous epidermal nevus,
organoid nevus, nevus sebaceous of
Jadassohn, sebaceous adenoma,
papillary eccrine adenoma, benign
epithelial hyperplasia, prurigo nodularis,
cutaneous leishmaniasis, discoid lupus
inverted follicular keratosis, micaceous
pseudoepitheliomatous balanitis,
trichilemmoma, trichilemmal cyst.
Premalignant Actinic keratosis, arsenic keratosis,
keratoacanthoma, Bowen’s disease.
Malignant Squamous cell carcinoma, basal cell
carcinoma, sebaceous carcinoma,
granular cell tumor, adenocarcinoma,
metastatic renal cell carcinoma, Kaposi
sarcoma, Paget’s disease, malignant
melanoma.
Figure 17.1  Cutaneous horn on the neck.

DOI: 10.1201/9781351054225-38 357

358  Cutaneous horn

Figure 17.5  Multiple cutaneous horns on the penis in a

Figure 17.2  Cutaneous horn on lower lip. The underlying case of Bowen’s disease.
lesion in this case was verruca vulgaris.

Figure 17.3  Cutaneous horn on the tip of finger. Figure 17.6  Multiple cutaneous horns in a case of Bowen’s
disease with squamous cell carcinoms (SCC) on the abdomen.

Figure 17.7  Multiple cutaneous horns on the great toe in

Figure 17.4  Long slender cutaneous horn on the scalp. a case of subungual Bowen’s disease.
 Cutaneous horn  359

●● Cutaneous horns are slow-growing and cause very little

BOX 17.1: Risk factors indicating possibility physical discomfort unless they are mechanically pulled
of underlying lesions being malignant or when they are present in areas of physical irritation.
However, they are cosmetically disfiguring and cause
●● Previous history of actinic keratosis or skin cancer social discomfort for the patient.
●● Male sex ●● The main clinical importance, as stated earlier,
●● Fair skin is concern for the lesion underlying it, and hence
●● Advanced age deep excisional biopsy is indicated to rule out any
●● Presence of lesion in sun-exposed area premalignant or malignant lesion. The clinical diagnosis
●● Presence of erythema, inflammation, tenderness, of underlying lesions is almost impossible; however,
infiltration, or indurations at the base certain factors (enlisted in Box 17.1) raise suspicion of
●● Large base or larger width to height ratio malignant lesions.
●● Bleeding
●● Giant size
●● Increased verrucosity and presence of multiple horns REFERENCES
1. Fernandes NF, Sinha S, Lambert WC, Schwartz RA. Cutaneous
horn: A potentially malignant entity. Acta Dermatovenerol Alp
●● They grow slowly over a long period of time (several
Pannonica Adriat 2009;18(4):189–193.
years) and vary in sizes from a few millimeters to as 2. Rush PS, Cockerell CJ. Cutaneous horn. Available from https://
long as 25 centimeters. emedicine.medscape.com/article/1056568-overview. Accessed on
●● They are considered giant if they are more than 1 cm. in 09.04.2020.
height. 3. Mantese SA, Diogo PM, Rocha A, Berbert AL, Ferreira AK, Ferreira
TC. Cutaneous horn: A retrospective histopathological study of
●● They occur in different shapes, such as conical,
222 cases. An Bras Dermatol 2010;85(2):157–163.
cylindrical, curved, straight, pointed, and can be 4. Copcu E, Sivrioglu N, Culhaci N. Cutaneous horns: Are these
corrugated transversely as well as longitudinally. lesions as innocent as they seem to be? World J Surg Oncol
●● They are firm to hard in consistency, with little or no 2004;2:18.
sensation. 5. Sathyanarayana SA, Deutsch GB, Edelman M, Cohen-Kashi KJ.
Cutaneous horn: A malignant lesion? A brief review of the litera-
●● The base of the lesion may be flat, nodular, or
ture. Dermatol Surg 2012;38(2):285–287.
crateriform. 6. Pyne J, Sapkota D, Wong JC. Cutaneous horns: Clues to invasive
●● They are usually solitary but can have an associated squamous cell carcinoma being present in the horn base. Dermatol
smaller satellite lesion, or they can also be multiple. Pract Concept. 2013 Apr 30;3(2):3–7.
 Section     3
Regional dermatology

PIYUSH KUMAR, PC DAS

The skin is the largest organ of the human body, and despite occluded, and moist, leading to frequent macerations noted
its apparent uniform appearance, it has numerous regional in diseases like Darier disease. Additionally, skin has some
variations both structurally and functionally. These varia- specialized structures such as mucosa, hairs, and nails that
tions enable skin to perform the region-specific tasks effi- are affected by some unique dermatoses and may have dis-
ciently. Structural variations include difference in thickness tinctive presentations of various dermatoses.
of skin, presence or absence of specialized structures such Another factor that makes one skin region different
as mucosa or nails, density of pilosebaceous units and from another one is external influences in the form of fric-
eccrine glands, adipose tissue layer thickness, vascularity, tion, occlusion, pressure, sun exposure, external trauma,
innervation, presence/absence of horny layer, colonization and temperature, to name a few. One of the most common
by skin flora, and underlying lymph nodes. One or more environmental insults is sun exposure, which is localized to
of these structural variations make skin a heterogeneous sun-exposed areas and can be easily appreciated if one looks
structure composed of many unique topographical regions. at areas that are not generally exposed to sun, such as the
For example, thicker skin in palms and soles can handle post-auricular area, upper eyelid, and so on. Skin folds are
the trauma more effectively, while thinner skin over eye- special areas that create a special environment (occlusion,
lids makes it easy for periorbital muscles to lift it; buccal moist, warmth) that makes it suitable for growth of certain
mucosa houses the special receptors for taste, while genital organisms such as dermatophytes.
mucosa has dense sensory receptors for sexual pleasures. Considering anatomical variations and influence of exter-
All these regional variations are also associated with dis- nal factors, it becomes obvious that skin has many unique
eases that are localized to the specific regions of the body. regions that are predisposed to a different set of dermatoses.
Thus, each region is anatomically predisposed to develop Recognition of variations in the localization of various der-
a unique set of dermatoses. In addition, the microenviron- matoses and awareness of the changes in the morphology of a
ment of a region may modify the morphology of a particu- particular dermatosis in different regions are essential com-
lar dermatosis. For example, axillae are constantly warm, ponents of skills required for making a clinical diagnosis.

DOI: 10.1201/9781351054225-39
 E20
Scalp

PC DAS

ABSTRACT
While most skin disorders affect the scalp skin in a manner the same as elsewhere on the body, a few conditions affect the
scalp more frequently than other body sites. Additionally, the frequency and morphology of various dermatoses on scalp may
be affected by presence or absence of hairs on the scalp. This chapter discusses the clinical approach to the diagnosis of various
dermatoses commonly affecting scalp.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-40 361

 E21
Periorbital area

ANUP KUMAR TIWARY

ABSTRACT
There are various cutaneous diseases that preferentially involve the periorbital region. Some systemic diseases, such as der-
matomyositis, lupus, and histiocytosis, may also present with cutaneous changes in this area. To differentiate and make the
correct diagnosis, it is imperative to identify the morphology, distribution, and other clinical characteristics of periorbital
dermatoses. This chapter briefly describes the clinical approach to the diagnosis of periorbital dermatoses.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

362 DOI: 10.1201/9781351054225-41

 E22
Ears

DIVYA SACHDEV

ABSTRACT
Dermatoses of the ear fall under the purview of various medical disciplines such as dermatology, otorhinolaryngology, and
general medicine. The ear is anatomically a unique structure as it is composed of elastic cartilage covered by skin and is poorly
vascularized. The dermatoses of the external ear may serve as a mirror to various systemic diseases such as Frank’s sign for
coronary artery disease, bluish discoloration of ear in alkaptonuria, etc. At the same time, there are numerous dermatoses that
are specific for the ear, such as juvenile spring eruption, acanthoma fissuratum, etc. Therefore, it is imperative to familiarize
ourselves with the various dermatoses that may affect the ear.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-42 363

 E23
Nose

DIVYA SACHDEV

ABSTRACT
The nose is affected in a variety of dermatological diseases. Being the most prominent part of mid-face, it is anatomically,
physiologically, esthetically, and psychologically important. Any dermatosis affecting the nose has physical as well as grave
psycho-social impacts on the health of the patient. Therefore, it is imperative to familiarize ourselves with the various presen-
tations of skin diseases that may affect the nose in order to facilitate early diagnosis and proper management.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

364 DOI: 10.1201/9781351054225-43

 18
Oral mucosa

SUNIL KOTHIWALA, KANYA RANI VASHISHT

INTRODUCTION WHITE LESIONS

The oral cavity is oval shaped and is separated into the White lesions of the oral mucosa are multifactorial group
oral vestibule and the oral cavity proper. It is bound by of disorders, the color of which is produced by scattering of
the lips anteriorly, the cheeks laterally, the floor of the the light through an altered epithelial surface (Figure 18.1).
mouth inferiorly, the oropharynx posteriorly, and the pal- The oral mucosa becomes white due to
ate superiorly. The epithelium that lines the oral mucosa
derives mostly from ectoderm except posterior one-third 1. Excess production of keratin intrinsically or from
of the tongue, which derives from endoderm. In contrast benign keratotic disease (genodermatoses) or dysplasia
to the skin, the oral epithelium exhibits different patterns
of keratinization, with higher proliferation rate. The masti- Table 18.1  Approach to oral pathologies based on color
catory mucosa (hard palate, gingiva, and alveolar mucosa) of lesion
has keratinized or parakeratinized (retained nuclei in the Pigmented
stratum corneum) squamous epithelium. The tongue has White lesions Red lesions lesions
parakeratinized, non-­ keratinized, and specialized epi-
thelia (papillae). The buccal mucosa and vestibule have Leukoplakia Hereditary Melanotic macule
non-­keratinized stratified or parakeratinized squamous Leukoedema Vascular Amalgam tattoo
epithelia, respectively. The supporting connective tissue is Hairy malformation Heavy metal
of ectomesenchymal origin. leukoplakia Hereditary deposition
The oral cavity can be affected primarily by disease of Lichen planus mucoepithelial Drug-induced
mucosa and adjoining structures as well as by systemic Geographic dysplasia pigmentation
diseases. Many dermatological conditions, including tongue Acquired Smoker’s
genetic, inflammatory, infective and neoplastic disor- Hairy tongue Trauma melanosis
ders, can also occur in oral mucosa with similar clinico- Candidiasis Thermal burn Freckles and
pathologic features. Occasionally, the oral cavity can be Fissured tongue Radiation mucositis lentigines
the only site of a skin disease, as in lichen planus, pem- Nicotinic Median rhomboid Pigmented nevi
phigus, and mucous membrane pemphigoid. Diagnosing stomatitis glossitis Nevus of Ota
and treating mucosal lesions of the mouth, including the White sponge Denture-related Melanoma
gums, is challenging for most clinicians because of the nevus stomatitis Melanoacanthoma
wide variety of conditions that can present with similar- Dyskeratosis Erythroplakia Addison’s disease
appearing lesions. This chapter explores both common congenital Plasma cell Peutz-Jeghers
and uncommon oral pathologies, and it follows con- Pachyonychia gingivitis syndrome
ventional classification of lesions. Oral mucosal lesions congenital Lupus
can be divided based on color of lesion (i.e., white, ery- Chemical burn erythematosus
thematous, and pigmented) and morphology of lesions, Anemia
such as ulcerative, verrucous, and lump and swellings Thrombocytopenic
(Tables 18.1 and 18.2). It emphasizes clinical descrip- purpura
tion of lesions, common sites of pathology, and differ- Inflectional
ential diagnosis. Gingival hyperplasia and cheilitis are mononucleosis
described separately.1-9 Reiter’s disease

DOI: 10.1201/9781351054225-44 365

366  Oral mucosa

Table 18.2  Approach to oral pathology based on morphology of lesions

Ulcerative lesions Verrucous lesions Lump and swelling

Prior history of vesicobullous Without history of
lesions vesicobullous lesions
Herpes simplex Aphthous ulcer Papilloma Angioedema
Herpes zoster Behçet’s disease Verruca vulgaris Abscess
Herpangina Syphilis Condyloma accuminatum Mucocele
Hand, foot, and mouth disease Tuberculosis Focal epidermal hyperplasia Ranula
Erythema multiforme Leprosy Verrucous xanthoma Fibroma
Stevens-Johnson syndrome Leishmaniasis Acanthosis nigricans Pyogenic granuloma
Toxic epidermal necrolysis Eosinophilic ulcer Darier disease
Pemphigus Squamous cell carcinoma
Bullous pemphigoid
Cicatracial pemphigoid
Pemphigoidgestationis
Linear IgA disease
Dermatitis herpetiformis
Bullous lichen planus
Epidermolysis bullosa
Epidermolysis bullosa acquisita

2. Excess production of keratin in response to injury ●● Tobacco, alcohol, chronic local friction, and Candida
(e.g., friction or biting) albicans are important predisposing factors.
3. Thickening of the epithelium ●● Clinically it can be divided into two types:
4. Damage to epithelial cells from direct and identifi- homogeneous (common) and non-homogeneous
able contact injury (Table 18.3).
●● The homogeneous type is usually a thin, flat, and
Leukoplakia uniform white plaque with at least one area that
●● This is defined as a precancerous white patch or plaque is well demarcated, with or without fissuring
firmly attached to the oral mucosa. (Figure 18.2a,b).

White lesion

Fixed to the surface Rubs off Translucent surface Mucosal fibrosis

Smooth on palpation Cysts

Flat or
Elevated - History of Immunosuppression
minimally
papillary chemical use
elevated

Candidiasis
Burn
Ragged Frictional
surface keratosis
Linear Reticular Verrucous Fissured Plaque

Extend from Wickham’s Small Broad Tobacco

striae pedunculated sessile Leukoplakia
commissure to smoking
posterior teeth HIV+, tongue Child, Disappear on
lateral border, B/L congenital, stretching/inversion
B/L B/L buccal mucosa
With
Hypertrophic
Linea Oral lichen Papilloma Verrucous Oral hairy surrounding
White sponge LP
alba planus carcinoma leukoplakia Leukoedema striae
Verruca nevus

Figure 18.1  Clinical approach to white lesions of the mucosa.

 Oral mucosa  367

Table 18.3  Main differences between localized leukoplakia and proliferative leukoplakia

Proliferative verrucous
Localized leukoplakia leukoplakia
Epidemiology Men > Women Women > Men
Association with smoking Strong Weak
Location Single site, Multifocal
Ventral tongue and floor of Gingiva is most common site
mouth are most common sites
Dysplasia or SCC at first 40% <10%, mostly atypical verrucous
biopsy hyperplasia or KUS
Malignant transformation 3–15% overall, 1–3% per year 70–100% overall, 10% per year
Treatment Easy to ablate or excise because Difficult to treat because multifocal
localized

●● Non-homogeneous lesions can have the presence of

speckled or erythroplakic and nodular or verrucous
areas (Figure 18.3).
●● Proliferative verrucous leukoplakia presents as
irregular, white, exophytic plaque with a papillary
surface and characterized by relative progression
and spread (Figure 18.4).
●● The most common sites are the buccal mucosa, tongue,
floor of the mouth, gingiva, and lower lip.
●● Malignant transformation for localized leukoplakia
varies between 3% and 15%. Speckled and verrucous

Figure 18.2  (a) Well-demarcated localized white

plaque on buccal mucosa lip in homogenous leuko-
plakia. (b) Homogenous leukoplakia of the lip.
(a – Courtesy: Dr. Piyush Kumar, Katihar, India; Figure 18.3  Non-homogenous leukoplakia having
b – Courtesy: Dr. Hiral Shah, Baroda Medical College, mild verrucous surface. (Courtesy: Dr. Piyush Kumar,
Vadodara, India.) Katihar, India.)
368  Oral mucosa

lesions of human immunodeficiency virus

(HIV) infection,
●● Epstein–Barr virus seems to play an important role in
the pathogenesis.
●● It commonly appears bilaterally on the lateral margins
of the tongue, and may spread to the dorsum and the
ventral surface.
●● Clinically it presents as asymptomatic white, elevated
plaque with vertically oriented corrugations on surfaces
that cannot be rubbed off.
●● DD: Geographic tongue, frictional keratosis, pseudo-
hairy leukoplakia, hyperplastic candidiasis.

Lichen planus
●● This is a common chronic mucocutaneous disease

mediated by CD8+ T cell-mediated autoimmune

phenomena.
●● Skin involvement in mucosal lichen planus is 40%.

●● Mucosal involvement in lichen planus is 40–60%.

●● The buccal mucosa, tongue, and gingiva are the sites of

predilection.
●● Six clinical types have been described.

●● Reticular: white papules that coalesce to form a

network of lines (Wickham’s striae, Figure 18.5a–d)
●● Erosive/ulcerative: well to ill-defined shallow ulcer
with overlying fibrin clots (Figure 18.6a–d)

Figure 18.4  Proliferative verrucous leukoplakia. (Courtesy:

Dr. Piyush Kumar, Katihar, India.)

leukoplakia have a greater risk for malignant

transformation than the homogeneous form.
●● DD: Leukoedema, lichen planus, keratosis of unknown
significance (KUS), squamous cell carcinoma, in
situ and invasive, verruca vulgaris, chronic chewing,
chemical burn.

Leukoedema
●● This is a normal anatomical variation. It usually occurs

over the buccal mucosa.

●● It results from increased thickness of the

epithelium and intracellular edema of the prickle-

cell layer.
●● Clinically, it is characterized by a whitish-gray

edematous lesion with a slightly wrinkled surface,

which characteristically disappears when the mucosa is
averted and stretched.
●● DD: Leukoplakia, white sponge nevus, oral hairy

leukoplakia.

Hairy leukoplakia
●● Hairy leukoplakia is a non-precancerous lesion, Figure 18.5  (a) Reticular lichen planus with Wickham
one of the most common and characteristic striae on buccal mucosa. (Continued)
 Oral mucosa  369

Figure 18.5 (Continued)  (b) Reticular whitish plaque of

lichen planus on tongue. (c) Reticular oral lichen planus
affecting buccal mucosa and lip. (d) Reticular lichen pla-
nus with pigmentation. (a – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

Figure 18.6  (a) Erosive lichen planus on buccal mucosa.

(Continued)
370  Oral mucosa

Figure 18.6 (Continued)  (b) Erosive lichen planus on buccal

mucosa. (c,d) Erosive lichen planus affecting gingiva.
(a,c – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Atrophic: shiny plaques often with Wickham striae

in surrounding mucosa (Figure 18.7)
●● Hypertrophic: leukoplakia with Wickham striae
(Figure 18.8a,b)
●● Bullous: intact blisters that lead to erosive LP after
rupture (Figure 18.9)
●● Pigmented: discrete to coalescing brown patches
(Figure 18.10a,b)
●● The lesions may appear as a mixture of clinical
subtypes.
●● DD: Lichenoid drug reaction, mucous membrane
pemphigoid.

Geographic tongue
●● Geographic tongue is a common benign

condition, primarily affecting the tongue and

rarely other oral mucosa sites (geographic
stomatitis).
●● It presents as multiple, irregularly shaped, well-

demarcated, red, map-like smooth patches, Figure 18.7  Atrophic lichen planus. (Courtesy: Dr. Piyush
typically surrounded by a slightly elevated Kumar, Katihar, India.)
 Oral mucosa  371

Figure 18.9  Ulcer following bullous lichen planus.

Figure 18.8  (a,b) Hypertrophic lichen planus. Figure 18.10  (a) Multiple dark-brown macules on buccal mucosa
(Courtesy: Dr. Piyush Kumar, Katihar, India.) and lip. (Continued)
372  Oral mucosa

Figure 18.10 (Continued)  (b) Pigmented lichen planus.

(a – Courtesy: Dr. Ashutosh Ranjan, Chhapra, India;
b – Dr. Hiral Shah, Baroda Medical College,
Vadodara, India.)

whitish border on the dorsum of the tongue

(Figure 18.11a,b).
●● It is also known as benign migratory glossitis because
the lesions persist for a short time in one area, then
disappear completely and reappear in another area. The
condition is usually asymptomatic and often coexists
with a fissured tongue.
●● DD: Chemical burns, fissured tongue, lichen planus,
candidiasis.

Hairy tongue
●● This is characterized by a marked accumulation of

keratin on the filiform papillae of the tongue, resulting

in a hair-like pattern.
●● Poor oral hygiene, oxidizing mouthwashes, antibiotics,

excessive smoking, radiation therapy, emotional stress,

and bacterial and Candida species infections are Figure 18.11  (a) Geographic tongue presenting
predisposing factors. with map-like well-demarcated smooth patches sur-
●● It is usually asymptomatic and appears as elongation rounded by white borders. (b) Geographic tongue.
of the filiform papillae on the dorsal surface of (b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Oral mucosa  373

Figure 18.12  Hairy tongue in a patient of paraneoplastic

pemphigus. (Courtesy: Dr. Hiral Shah, Baroda Medical
College, Vadodara, India.)

the tongue, giving a whitish to brown-black color

(Figure 18.12).
●● DD: Amalgam tattoo, drug-induced pigmentation,
heavy metal deposits.
Candidiasis
●● This is the most common oral fungal infection.
●● It is usually caused by Candida albicans and less

frequently by other fungal species (C. glabrata, C.

krusei, C. tropicalis, C. parapsilosis).
●● Predisposing factors are local (poor oral hygiene,

xerostomia, mucosal damage, dentures, and antibiotic

mouthwashes) and systemic (broad-spectrum
antibiotics, steroids, immunosuppressive drugs, Figure 18.13  Oral candidiasis presenting as (a) slightly
radiation, HIV infection, hematological malignancies, elevated white plaque on lip mucosa, (b) with erythema
neutropenia, iron-deficiency anemia, cellular all over oral cavity in case of oral thrush. (Courtesy:
Dr. Piyush Kumar, Katihar, India.)
immunodeficiency, endocrine disorders).
●● Oral candidiasis is classified as

●● Primary, consisting of lesions exclusively on the oral – Nodular candidiasis is a chronic form of the
and perioral area. disease; it appears clinically as a white, firm,
– Pseudomembranous candidiasis is the most and raised plaque that usually does not detach
common form of the disease and is clinically from mucosa (Figure 18.14).
characterized by creamy-white, slightly elevated, – Other subtypes include erythematous, papillary
removable spots or plaque (Figure 18.13a,b). hyperplasia of the palate and candida-associated
374  Oral mucosa

Figure 18.14  Nodular candidiasis presenting as white,

raised, non-scrapable plaque on soft palate, oropharynx, Figure 18.15  (a,b) Mucocutaneous candidiasis showing dif-
and buccal mucosa. fuse involvement of oral cavity and angle of mouth. (Courtesy:
Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)

lesions (angular cheilitis, median rhomboid

glossitis, denture stomatitis).
●● Secondary, consisting of oral lesions of
mucocutaneous disease.
●● Mucocutaneous candidiasis is a heterogeneous and rare
group of clinical syndromes, characterized by chronic
lesions of the skin, nails, and mucosae, and usually
associated with immunological defects. Oral lesions
appear as white and usually multiple plaques that
cannot be wiped off (Figure 18.15a,b).

Fissured tongue
●● This is a benign condition affecting the dorsum of

tongue in the form of shallow or deep and prominent

groves of variable depth and size. Many times it is
associated with a focal whitish coating of tongue
(Figure 18.16) or geographical tongue (Figure 18.17).
●● The etiology is not clearly defined, but sometimes it is

seen in orofacial granulomatosis, Melkersson-Rosenthal

syndrome, Down syndrome, and pustular psoriasis.
●● It is usually asymptomatic, but painful or burning

sensations develop when food debris get arrested in

grooves or it is associated with geographic tongue. The
prominence of the condition appears to increase with Figure 18.16  Multiple shallow and deep grooves on dor-
increasing age. sum of tongue with whitish coating at tip of tongue.
 Oral mucosa  375

●● It is characterized by hyperpigmentation, atrophic skin

areas, telangiectasia, nail dystrophy, hyperhidrosis,
skin and mucosal bullae, blepharitis and ectropion, and
anemia.
●● The most common oral lesions are leukoplakia, atrophy
of papillae, intraoral brown pigmentation, recurrent
blisters, taurodont teeth (enlarged pulp chambers and
short roots), and dental caries.

Pachyonychia congenita
●● This is an autosomal dominant inheritance

●● It is characterized by symmetrical nail thickening,

palmoplantar hyperkeratosis, hyperhidrosis,

blister formation, follicular keratosis, and oral
lesions.
●● Oral leukokeratosis presents as thick, painful, white

or grayish-white plaques, usually on the tongue and

buccal surface in first year of birth. It may interfere with
suckling and breastfeeding.

Chemical burn
●● This is injury to the oral mucosa caused by topical

application of caustic agents.

●● Clinically, the affected mucosa is covered with a white

membrane due to necrosis. The necrotic epithelium can

easily be scraped off, leaving a red, bleeding surface. The
lesions are painful.
Figure 18.17  Fissured tongue with geographic tongue.

ULCERATIVE LESIONS
Nicotinic stomatitis
●● Nicotinic stomatitis is a common keratosis associated
Preceding with vesico-bullous lesion
with heavy pipe and cigar smoking; it exclusively occurs Herpes simplex virus infection
on the hard palate. It is non-premalignant, in contrast ●● This is a common viral infection usually caused by
to reverse smoker’s palate. herpes simplex virus (HSV) type 1 and rarely type 2
●● Clinically, it starts with erythema; gradually it becomes
(HSV-2).
wrinkled and diffuse grayish-white in color, with ●● The primary infection typically occurs early in life, with
numerous punctate red-centered micronodules that viral latency in neural ganglia.
represent the inflamed and dilated orifices of the minor ●● Reactivation can occur because of several triggers,
salivary gland ducts. including immunosuppression, any febrile disease,
●● DD: Candidiasis, leukoplakia, hairy leukoplakia.
physical trauma, and stress.
●● Clinical presentation and characteristics include the
White sponge nevus following:
●● This is a hereditary dyskeratotic hyperplasia of mucous ●● Primary herpetic gingivostomatitis:
membrane. – Clinically it starts with erythema and edema
●● Autosomal dominant inheritance usually appears in
with numerous coalescing vesicles that rapidly
early childhood. rupture, leaving painful small, round, shallow
●● It presents at symmetrical white, thickened,
ulcers covered by yellow fibrin. New lesions
velvety lesions with multiple furrows and a spongy continue to develop during the first three to five
texture. days. The ulcers heal in 10 to 14 days. Gingiva
●● Buccal mucosa and the ventral surface of the tongue are
may be purple and edematous. Perioral lesions
the sites of predilection may also present.
– The primary episode is commonly associated
Dyskeratosis congenita with malaise, anorexia, irritability, fever,
●● This is related to autosomal recessive or X-linked and enlarged and tender anterior cervical
inheritance. lymph nodes.
376  Oral mucosa

Figure 18.18  (a) Multiple grouped vesicles and erosions in herpes labialis. (b) Multiple grouped pustules in herpes labialis.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Recurrent herpes labialis: Prodromal symptoms such as pain and tenderness –

– The lesions develop as three to five vesicles at usually associated with headache, malaise, and fever –
vermilion border of lip and last at least 48 hours may develop two to four days before the appearance of
(Figure 18.18a,b). oral or skin lesions consisting of clusters of vesicles and
– Lesions may get secondarily infected with within two or three days evolve into pustules and ulcers,
staphylococcus or streptococcus (Figure 18.19). covered by crusts (Figure 18.20). The lesions persist for
●● Recurrent intraoral herpes simplex infection: two to three weeks.
– It presents as ulcers with white border and ●● DD: Herpes simplex, erythema multiforme.
sometimes with dendritic appearance. In
immunocompromised individuals, chronic
slowly progressive ulcerative or nodular lesions
may present.
●● DD: Herpetiform aphthous ulcer; herpangina; erythema
multiforme; hand, foot, and mouth disease.

Herpes zoster
●● It is an acute self-limiting viral infection caused by

varicella zoster characterized by unilateral involvement.

●● Oral manifestations are a result of involvement of the

second and third branches of the trigeminal nerve.

Figure 18.19  Herpes labialis with secondary infection. Figure 18.20  Multiple erosions on hard palate and lip in
(Courtesy: Dr. Hiral Shah, Baroda Medical College, case of herpes zoster. (Courtesy: Dr. Hiral Shah, Baroda
Vadodara, India.) Medical College, Vadodara, India.)
 Oral mucosa  377

Herpangina Hand, foot, and mouth disease

●● It is an acute self-limiting viral infection caused by ●● It is an acute self-limiting contagious viral infection

coxsackievirus group A, types 1–6, 8, 10, and 22, caused by coxsackievirus A16.
frequently affecting children and young adults. ●● It usually affects children and young adults, and often

●● Oral lesions consist of small, numerous vesicles occurs in epidemics.

surrounded by intense erythema that rupture to ●● Oral manifestations are always present, and are

form painful ulcers that heal within 7 to 10 days. characterized by small vesicles (5 to 30 in
Characteristically, the lesions appear on the soft palate number) surrounded by an erythematous halo.
and uvula, tonsillar pillars, and posterior pharyngeal wall. Vesicles rapidly rupture to form painful,
●● Other clinical features are fever, sore throat, dysphagia, shallow ulcers (2 to 6 mm in diameter)
headache, and malaise. (Figure 18.21a–d).
●● DD: Aphthous stomatitis, drug eruption, hand-foot- ●● The buccal mucosa, tongue, and labial mucosa are the

mouth disease, primary herpes gingivostomatitis. most commonly affected sites.

Figure 18.21  (a) Shallow ulcer surrounded by erythematous

halo on soft palate and uvula. (b) Skin lesions on palms.
(c) Multiple vesicles and ulcers on labial mucosa with skin
lesions on face. (d) Vesicles and ulcers on palate. (Continued)
378  Oral mucosa

Figure 18.21 (Continued)  (e) Ulcer on the lip and hard pal-
ate in varicella. (a–d – Courtesy: Dr. Piyush Kumar, Katihar,
India; e – Courtesy: Dr PC Das, Katihar, India.)

Figure 18.22  (a) Irregular erosions on tongue and buccal

●● Skin lesions are not constant, and present as small
mucosa with classical targetoid lesions on palm.
vesicles with a narrow red halo on dorso-lateral (b) Erosions covered with necrotic pseudomembrane on
surfaces of the fingers and toes, palm, soles, and labial mucosa.
buttocks.
●● The disease lasts five to eight days.
●● DD: Aphthous ulcer, herpes simplex, herpangina,
varicella (Figure 18.21e). ●● The lips, buccal mucosa, tongue, soft palate, and floor
of the mouth are most commonly involved. Other
Erythema multiforme mucosae, such as conjunctiva or genital mucosa, may
●● It is an acute, self-limiting immune-mediated also be involved.
mucocutaneous disease triggered by an infection such ●● The skin manifestations consist of erythematous,
as herpes or Mycoplasma pneumoniae, drugs, radiation, flat, round macules, papules, or plaques, usually
or malignancies involving skin and mucosa. in a symmetrical pattern. The characteristic skin
●● It may present within a wide spectrum of severity.
patterns are target- or iris-like lesions. Skin bullae may
Erythema multiforme minor represents a localized occasionally be seen. Prodromal symptom, such as
eruption of typical target-like lesions (predominantly headache, malaise, arthralgias, and fever, may also be
acral) with minimal or no mucosal involvement. present. Recurrences are common.
Erythema multiforme major is more severe presentation ●● DD: Herpes simplex infection, aphthous ulcer,
with involvement of up to 10% of body surface area and pemphigus vulgaris, erosive lichen planus.
one or more mucous membranes.
●● The oral lesions present as coalescing small vesicles that Stevens-Johnson syndrome
rupture within two or three days, leaving irregular, ●● This is predominantly drug induced.
painful erosions covered by a necrotic pseudomembrane ●● Oral lesions are always present, and presentation
(Figure 18.22a,b). includes erythema, edema, and extensive vesicle
 Oral mucosa  379

Figure 18.23  Erythema, edema, erosions with severe

hemorrhagic crusting on labial mucosa in patient with
Steven Johnson syndrome. (Courtesy: Dr. Hiral Shah,
Baroda Medical College, Vadodara, India.)

formation, followed by painful erosions covered by

grayish-white or hemorrhagic pseudomembrane or
necrosis (Figure 18.23).
●● The primary site is the oral cavity, and it may extend to
the pharynx, larynx, and esophagus. The ocular lesions Figure 18.24  Toxic epidermal necrolysis presenting with
erythema and edema of labial mucosa with erosions cov-
consist of conjunctivitis, uveitis, symblepharon, or even
ered with hemorrhagic necrotic pseudomembrane with
panophthalmitis. The genital lesions are balanitis or eye and skin lesions.
vulvovaginitis, and scrotal erosions.
●● The skin manifestations may vary from very mild to
severe in the form of maculopapular rash, blisters, bullae. The epidermis sloughs in sheets, leaving a moist,
urticarial plaques, or targetoid lesions with two zones of denuded dermis. Nikolsky’s sign is positive.
color. The innermost is vesicular, purpuric, or necrotic ●● DD: Stevens-Johnson syndrome, pemphigus vulgaris,
surrounded by erythema. severe and extensive burns.
●● DD: Erythema multiforme major, Behçet disease, Pemphigus vulgaris
pemphigus, pemphigoid, primary herpes simplex. ●● Pemphigus vulgaris is a severe chronic bullous

Toxic epidermal necrolysis autoimmune mucocutaneous disease due to antibodies

against antigens Desmoglein 1 and 3.
●● This is a potentially life-threatening condition triggered
●● Oral lesions are characterized by vesicobullous lesions,
by drugs (sulfonamides, anticonvulsants and NSAIDs)
which rapidly rupture, leaving painful erosions with a
in genetically sensitive individuals.
tendency to extend peripherally (Figure 18.25).
●● It usually begins with an influenza-like prodrome
●● The buccal mucosa, labial mucosa, palate, tongue, floor
characterized by low-grade fever, malaise, arthralgias,
of the mouth, and gingiva are often involved. Lesions
conjunctival burning sensation, rhinitis, and
may develop over other mucosa.
headache. ●● The skin lesions present as small vesicles or flaccid
●● The oral manifestations consist of diffuse erythema,
bullae on normal skin that rupture quickly, leaving
edema followed by vesicles and painful hemorrhagic
persistent erosions. Nikolsky’s sign is positive.
erosions, primarily on the lips and periorally as well as ●● DD: Cicatricial pemphigoid, bullous pemphigoid, linear
on the buccal mucosa, tongue, and palate. Hemorrhagic
IgA disease, epidermolysis bullosa acquisita, toxic
crusting of lips is a common finding. Ocular, genital,
epidermal necrolysis.
and other mucous membrane lesions are common
(Figure 18.24). Bullous pemphigoid
●● Skin lesions start with symmetrical morbilliform ●● This is a chronic autoimmune mucocutaneous bullous
discrete lesions and gradually coalesce to become flaccid disease.
380  Oral mucosa

Pemphigoid gestationis
●● This is a rare autoimmune subepidermal blistering

disease occurring in the second or third trimester of

pregnancy or in the early postpartum period.
●● The target antigen is 180 kD, a hemidesmosomal

antigen (BP 180).

●● It usually affects the skin, and mucosal lesions occur in

less than 20% of cases.

●● The oral lesions usually follow the skin lesions and

present as multiple, usually hemorrhagic, bullae that

rupture quickly, leaving painful ulcerations.
●● The buccal mucosa, palate, tongue, and gingiva are the

most frequently affected areas.

●● The skin manifestations present as urticarial plaques

that usually start from periumbilical region and

spread peripherally. Lesions progress to form tense
vesicobullous lesions and soon rapture, leaving painful
ulcerations.
●● DD: Bullous pemphigoid, cicatricial pemphigoid, linear

IgA disease, dermatitis herpetiformis, epidermolysis

bullosa acquisita.
Figure 18.25  Multiple coalescing erosions on soft palate.
(Courtesy: Dr. Piyush Kumar, Katihar, India.) Linear IgA disease
●● Linear IgA disease is a chronic autoimmune

subepidermal bullous disease characterized by the

●● Bullous pemphigoid antigens (BP180, BP230) are the
linear deposition of IgA along the basement membrane
main target antigens.
zone.
●● Oral mucosa is affected in about 20–40% of cases, other
●● Oral lesions occur in about 20–30% of cases, and are
mucosae may also be affected.
characterized by the formation of bullae that soon
●● The oral lesions usually follow cutaneous manifestations
rupture, leaving superficial, non-specific ulcerations.
and begin as bullae that soon rupture, leaving shallow
Other lesions include erythematous patches,
ulcerations.
desquamative gingivitis, or erosive cheilitis.
●● Skin lesions are always present and begin as a non-
●● The skin lesions consist of bullae that may be
specific generalized rash followed by large, tense bullae
discrete or arranged in a herpetiform pattern
that rupture, leaving denuded areas without a tendency
described as the “cluster of jewels” sign, or tense
to extend peripherally.
vesicles or bullae may be seen at the edge of annular
●● DD: Pemphigus, cicatricial pemphigoid, linear IgA
or polycyclic lesions giving appearance of string of
disease, dermatitis herpetiformis, epidermolysis bullosa
pearls.
acquisita, pemphigoid gestationis.
●● DD: Cicatricial pemphigoid, bullous pemphigoid,
Cicatracial pemphigoid pemphigus, dermatitis herpetiformis, pemphigoid
●● This is a chronic autoimmune bullous mucocutaneous gestationis.
disease that primarily affects mucous membranes and
results in atrophy or scarring. Dermatitis herpetiformis
●● Bullous pemphigoid antigen (BP180), laminin 5, integrin ●● This is a chronic autoimmune recurrent cutaneous

B4, and type VII collagen are the main target antigens. bullous disease associated with gluten sensitive
●● Oral manifestations are seen in almost all patients, but enteropathy.
other mucosae and, rarely, the skin may be involved. ●● The oral mucosa is affected in 5–10% of cases, and

The oral lesions often begin on the gingiva as recurrent oral lesions include erythematous patches, papules,
vesicles or bullae that rupture, leaving large, superficial or vesicles. The vesicles appear in a cyclic pattern and
painful ulcerations that lead to epithelial atrophy or rupture rapidly, leaving shallow, painful ulcerations.
scarring on healing. ●● The tongue, buccal mucosa, and palate are more

●● Among other mucosae involvement, ocular lesions consist of frequently involved.

conjunctivitis, symblepharon, entropion, trichiasis, dryness, ●● Cutaneous lesions are always present and appear as

and corneal opacity, frequently leading to blindness. erythematous papules or plaques, with herpetiform
●● DD: Bullous pemphigoid, linear IgA disease, vesicles symmetrically distributed over extensors.
epidermolysis bullosa acquisita, pemphigus, erosive ●● DD: Bullous pemphigoid, cicatricial pemphigoid, linear

lichen planus, discoid lupus erythematosus. IgA disease, pemphigus, herpetiform ulcers.
 Oral mucosa  381

Epidermolysis bullosa
●● This is an inherited mechanobullous disorder

characterized by blister formation in response to

trauma. The lesions appear at birth or early in infancy.
●● Oral manifestations are more common in the junctional

and dystrophic forms. Oral lesions present as bullae,

usually in areas of friction, that rupture, leaving shallow
ulcers and, later, atrophy and scarring.
●● Dysplastic teeth may be seen in the severe forms of the

disease. Leukoplakia and squamous cell carcinoma may

develop on the scars.
●● DD: Pemphigus, cicatricial and bullous pemphigoid,

linear IgA disease, bullous dermatosis of childhood,

epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita

●● This is a rare, non-inherited, chronic, autoimmune

bullous disease involving the skin and mucous

membranes.
●● The target antigen is type VII collagen.

●● Oral lesions occur in more than 50% of cases and

present as solitary or multiple bullae and painful

erosions involving gingiva and buccal mucosa.
Significant scarring and dysfunction may occur.
●● Skin lesions present as hemorrhagic bullae and

ulcerations, usually at the sites of mechanical irritation.

The lesions heal with scarring and milia.
●● DD: Cicatricial pemphigoid, bullous pemphigoid, linear Figure 18.26  Single soft ulcer with red base.
IgA disease, pemphigus, genetic epidermolysis bullosa,
systemic lupus erythematosus, porphyria cutanea tarda.
●● They heal without scarring within 6 to 10 days,
Without preceding with vesicobullous lesions spontaneously or after removal of the cause.
●● DD: Carcinoma, aphthous ulcer, infections like
Traumatic ulcers tuberculosis, syphilis and mycoses.
●● Cheek bite, facial trauma, dentures, and chemical or

thermal burn are common cause of trauma. Recurrent aphthous stomatitis

●● They occur on the tongue, lips, and buccal mucosa. ●● This is a common disease that affects at least 20% of the
●● They usually appear as a single, painful soft ulcer population.
with a smooth red or whitish-yellow surface and thin ●● There are three clinical patterns: minor, major, and

erythematous halo (Figure 18.26). herpetiform (Table 18.4).

Table 18.4  Different clinical patterns of aphthous ulcer

Minor aphthous Major aphthous Herpetiform aphthous

Prevalence 80% of RAS 10% of RAS 10% of RAS
Age Adolescence adolescence Young adult
Site Vestibule, labial, buccal mucosa Any site Any site
and floor of mouth
Number of lesions 1–6 1–6 10–100
Size of lesions 2–4 mm >10 mm 1–2 mm
Clinical Painful, round ulcers covered Larger, last longer deep painful Small, painful, shallow
by a whitish-yellow ulcers, recurs more frequently ulcers, with a tendency to coalesce
membrane and surrounded (Figures 18.28, 18.29) into larger irregular ulcers
by a red halo (Figure 18.27)
Duration 7–12 days 3–6 weeks 1–2 weeks
Evolution Heal without scarring May heal with scarring Heal without scarring
382  Oral mucosa

Figure 18.29  Aphthous ulcers seen as multiple ulcers with

whitish membrane on palate.

●● DD: Herpes simplex, hand-foot-and mouth disease,

chancre, Behçet’s disease, cyclic neutropenia,
erythema multiforme, FAPA syndrome, Sweet
syndrome.

Behçet’s disease
●● Behçet’s disease is a chronic multisystem inflammatory
Figure 18.27  Small, shallow ulcer with whitish-yellow disorder with an association with HLA-Bw51, HLA-B5,
membrane on base and surrounded by red halo. HLAB27, and HLA-B12 alleles.
(Courtesy: Dr. P C Das, Katihar, India.) ●● It is more prevalent in male of 20 to 30 years of age.

●● Diagnosis is made on the presence of recurrent

aphthous stomatitis (Figure 18.30) and two or

more of genital ulcers, eye lesions, skin lesions, and
pathergy test.
●● Major criteria include the following:

●● Recurrent oral ulceration: recurrent aphthous

stomatitis, which recurred at least 3 times in a
12-month period
●● Recurrent genital ulceration: aphthous ulceration or
scarring
●● Skin lesions: erythema nodosum, papulopustular
lesions
●● Neuro-ocular lesions: conjunctivitis, iritis with
hypopyon, uveitis, retinal vasculitis, reduced
visual acuity. Syndromes resembling disseminated
sclerosis, pseudobulbar palsy or neurosyphilis
meningoencephalitis
●● Positive pathergy test
●● Minor criteria include the following:

●● Arthritis, arthralgia, thrombophlebitis, vein

thrombosis, arterial occlusion and aneurysms,
pulmonary, renal, and gastrointestinal
Figure 18.28  Large, deep ulcer on inner aspect of lip. manifestations
 Oral mucosa  383

●● Congenital: Oral lesions are high-arched palate, short

mandible, rhagades, Hutchinson’s teeth, and Moon’s or
mulberry molars.
●● DD: Lepromatous leprosy, traumatic ulcer, aphthous
ulcer, tuberculosis, herpes simplex, other infective

Figure 18.30  Major aphthous ulcers with prominent sur-

rounding erythema on buccal mucosa in case of Behçet’s
disease.

●● DD: Recurrent aphthous stomatitis, Reiter’s syndrome,

ulcerative colitis, erythema multiforme, Stevens-
Johnson syndrome, syphilis, Sweet syndrome.

Syphilis
●● Syphilis is a common sexually transmitted disease

caused by Treponema pallidum.

●● Different types of oral lesions develop in primary,

secondary, tertiary, and congenital syphilis.

●● Primary: Known as chancre characterized by a painless

ulcer with a smooth surface, raised borders, and an

indurated base over lip, tongue, and tonsils.
●● Secondary: Three types of lesions may present.

●● Mucous patches (painless, shallow, rounded

erosions covered with gray macerated scaling)
●● Irregular, serpiginous erosions/ulcers – “snail track
ulcers”
●● Condylomata lata (smooth, papillated, or covered
with cauliflower-like vegetation)
●● Tertiary: A gumma is a soft, painless non-
Figure 18.31  (a) Gumma lesion presenting as soft
malignant growth mostly occurring on hard palate painless ulcer on hard palate in tertiary syphilis.
(Figure 18.31a), which eventually ulcerates. Palatal (b) Erythematous edematous plaque with central
perforation and fistula formation may also happen. ulceration on lip in case of leishmaniasis. (Continued)
384  Oral mucosa

●● Paracoccidioidomycosis – oral ulcers and

lymphadenopathy
●● Cryptococcosis – oral ulcers
●● Histoplasmosis – nodular lesion or ulcers
●● Phycomycosis (mucormycosis, zygomycosis) – palatal
ulceration
●● Sporotrichosis – oral ulcer rare
●● Coccidioidomycosis – oral ulcers rare
Eosinophilic ulcer
●● This is a rare self-limiting benign condition caused by

trauma.
●● It presents as solitary or multiple painful ulcers with an

irregular surface, a raised border, and covered with a

whitish-yellow pseudomembrane.
●● DD: Major aphthous ulcer, traumatic ulcer.

Figure 18.31 (Continued)  (c) Erythematous plaque with Rarer causes of oral ulcer
ulceration on hard palate and lips, with multiple skin ●● Other rarer causes of oral ulceration are summarized in
lesions in the case of leprosy. (a – Courtesy: Dr. Santoshdev Table 18.6.
Rathod, Ahmedabad, India; c – Courtesy: Dr. Hiral Shah,
Baroda Medical College, Vadodara, India.) Squamous cell carcinoma
●● This represents about 90% of oral cancers. It occurs

oral ulcers (Table 18.5), infectious mononucleosis, more frequently in men than in women.
candidiasis, erythema multiforme, lichen planus. ●● Predisposing factors are tobacco smoke, alcohol, sun

exposure, poor oral hygiene, dietary deficiencies, iron

Oral ulcer caused by fungal infection deficiency, liver cirrhosis, and infections.
●● Aspergillosis – palatal necrosis ●● Common sites are lateral border, the ventral surface of

●● Blastomycosis – oral ulcer the tongue, lips, and floor of the mouth.

Table 18.5  Other infective causes of oral ulcer

Acute necrotizing
Infection gingivitis (Noma) Tuberculosis Gonorrhea Leishmaniasis
Causative Fusobacterium Mycobacterium tuberculosis Neisseria Mucocutaneous-
organism nucleatum, gonorrhoeae L. braziliensis, L.
Prevotella intermedia, guyanensis, L.
Borrelia vincentii, panamensis
Streptococcus, Post-kala azar dermal
Staphylococcus leishmaniasis –
L. donovani,
L. infantum,
L. tropica.
L. chagasi
Sites Buccal mucosa, Dorsum of tongue, Tongue, lip, buccal
lips, and the adjacent lip, buccal mucosa mucosa, palate
bone palate
Clinical Necrotizing ulcerative Tuberculous ulcer; Oral mucosal Central ulceration with
presentation – gingivitis, painless and irregular, thin erythema, surrounding
oral necrotizing ulceration undermined border, vegetating edema and erythematous,
manifestations covered with yellow- surface, usually covered by a ulceration edematous plaque
brown fibrin and debris gray-yellowish exudate (Figure 18.31b)
Other Salivation, halitosis, Osteomyelitis of the
manifestations fever, malaise, jaws, periapical granuloma,
and regional regional lymphadenopathy, and
lymphadenopathy scrofula
Differentials Tuberculosis, leukemia Mycoses, malignancy, syphilis Lepromatous
leprosy (Figure 18.31c)
 Oral mucosa  385

Table 18.6  Other causes of oral ulceration

Causes Disease Oral lesions

Nutritional Low iron Aphthous ulcer
Folate, vitamin B12 deficiency Glossitis
Angular stomatitis
Hematological Leukopenia Painful, deep, irregular ulcer covered with whitish membrane with
minimum red halo
Agranulocytosis Multiple necrotic ulcers covered by a grayish-white or dark and
dirty pseudomembrane without a red halo.
Severe necrotizing gingivitis Periodontal destruction
Myelic aplasia Gingival bleeding,
petechiae, and ecchymoses.
Necrotic ulceration
Myelodysplastic syndrome Persistent and recurrent ulcerations
Gingival hemorrhage Periodontitis
Leukemia Ulceration
Mucosal pallor
Gingival hemorrhage and swelling
Petechiae, ecchymoses
Tooth loosening
Lymphoma Diffuse painless swelling with or without ulceration on soft palate,
tongue, gingiva and tonsillar area.
Wegners granulomatosis Solitary or multiple oral ulcer with red halo in tongue, palate and
buccal mucosa
Gingival enlargement (strawberry appearance)
Oral ulceration
Mycosis fungoides Red or white areas
Pseudolymphoma Ulcer over palate
Histiocytosis Gingival swelling
Periodontal destruction
Oral ulceration
Nodular/glandular lesions in labial or buccal mucosa in
multicentric reticulohistiocytosis
Hypereosinophilic syndrome Oral erosion (buccal, gingival or labial mucosa)

Hypoplasminogenemia Oral ulceration

Gingival swelling
Gastrointestinal Pyostomatitis vegetans Deep fissures
Pustules
Papillary projections
Crohn’s disease Linear ulcer along buccal sulcus
Orofacial granulomatosis Thickening and folding of mucosa (cobblestone appearance)
Mucosal tags
Lip swelling, angular stomatitis
Immune defects HIV Recurrent ulceration
Vasculitis Polyarteritis nodosa Transient submucosal nodule
Ulceration, hemorrhage
Giant cell arteritis Ulceration and necrosis of tongue or lip
Iatrogenic conditions Chemotherapy- or radiotherapy-
induced mucositis
Bone marrow transplantation
Graft-versus-host disease Atypical diffuse ulcerations Lichenoid lesions
Oral bleeding, xerostomia, and infections
386  Oral mucosa

●● Early lesions appear as a white lesion reddish-white colored papillary or verrucous surface
(Figure 18.32a), a red lesion, or both, or even as with indurated base.
an exophytic mass (Figure 18.32b). However, the ●● A chronic lesion is an indurated ulcer with an irregular
most common clinical presentation is erosion or an papillary surface, elevated borders, and a hard base on
ulcer (Figure 18.32c). Exophytic mass presents as palpation (Figure 18.33a–c).

Figure 18.32  Early lesions of squamous cell carcinoma,

(a) white plaque on buccal mucosa, (b) red white exophytic
growth on palate, (c) ulcer on buccal mucosa (a,c – Courtesy: Figure 18.33  Chronic lesion of squamous cell carcinoma
Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. Hiral Shah, in form of (a) hard verrucous leukoplakia with ulceration,
Baroda Medical College, Vadodara, India.) (b) verrucous growth with ulceration. (Continued)
 Oral mucosa  387

●● There is cervical lymph node enlargement, hard and

fixed to tissue.
●● DD: Traumatic ulcer, tuberculosis, systemic mycoses,
syphilis, eosinophilic ulcer, Wegener granulomatosis.

PIGMENTED LESIONS
Pigmented lesions represent a variety of clinical entities, ranging
from physiologic changes (e.g., racial pigmentation) to manifesta-
tions of systemic illnesses (e.g., Addison’s disease) and malignant
neoplasms (e.g., melanoma and Kaposi sarcoma). Oral pigmen-
tation may be exogenous or endogenous in origin. Exogenous
pigmentation is commonly due to foreign-body implantation in
the oral mucosa. Endogenous pigments include melanin, hemo-
globin, hemosiderin, and carotene (Figure 18.34a).

Melanotic macule
●● Most common oral mucosal lesions of melanocytic

lesions develop due to functional hyperactivity of

Figure 18.33 (Continued)  (c) Squamous cell carcinoma
regional melanocytes.
●● It appears as small, solitary, brown to black, well-
presenting as hard verrucous leukoplakia with ulcer-
ation. (Courtesy: Dr. Hiral Shah, Baroda Medical College, demarcated, uniformly pigmented lesions that develop
Vadodara, India.) mostly in adult females (Figure 18.34b).
●● The lower lip, gingiva, and palate are the most

common areas.

Pigmented lesions

Color of lesion

Brown-black Bluish-grey Red blue

Focal Diffuse Focal

Focal Diffuse Diffuse

Early onset Late onset

With systemic No systemic

Melanotic macule Physiological Addison disease Drug induced Amalgam tattoo Heavy metal Hemangioma Kaposi
Peutz Jeghers deposition sarcoma
Pigmented nevi Post inflammatory Other foreign body Varix
Syndrome
tattoo
Melanoma Smoker’s Thrombus
melanosis Blue nevus
Melanoacanthoma Hematoma

(a)

Figure 18.34  (a) Clinical approach to pigmented lesions of the mucosa. (Continued)
388  Oral mucosa

discoloration may be seen with exposure to silver

(Figure 18.35a,b).
●● DD: Amalgam tattoo, drug-induced pigmentation.

Drug-induced pigmentation
●● This is a relatively common condition caused by increased

melanin production or drug metabolite deposition.

●● Most common drugs causing mucosal pigmentation

are antimalarials, tranquilizers, minocycline,

azidothymidine, ketoconazole, and phenolphthalein.
●● It may appear as focal irregular brown or black macules

or plaques or diffuse pigmentation (Figure 18.36).

●● The buccal mucosa, tongue, palate, and gingiva are the

most commonly affected sites.

●● DD: Heavy-metal deposition, fixed drug eruption.

Smoker’s melanosis
●● It is a benign abnormal melanin pigmentation of the

Figure 18.34 (Continued)  (b) Melanotic macule. (b – Courtesy: oral mucosa resulting from stimulation of melanocytes
Dr. Piyush Kumar, Katihar, India.) from tobacco.
●● It more commonly occurs in women, and the intensity

Amalgam tattoo of pigmentation depends on duration and dose.

●● There are multiple brown-pigmented areas, usually
●● This is a common iatrogenic disorder developing due to
located on the anterior labial gingiva of the mandible.
implantation of dental amalgam into oral mucosa. ●● Pipe smoking may lead to pigmentation of the buccal
●● It presents as a well-defined irregular or diffuse flat
mucosa and palate.
gray, bluish-black discoloration, usually involving the
gingival mucosa of the lower jaw. Freckles and lentigines
●● DD: Freckles, lentigo, pigmented nevi and melanoma.
●● Freckles are associated with increased melanin

Heavy-metal deposition production, while lentigines are associated with an

increased number of melanocytes.
●● This is caused by ingestion or exposure to heavy metals
●● They appear as solitary and well-demarcated
(i.e., bismuth, lead, silver, mercury, etc.).
asymptomatic round brown macules, less than 5 mm
●● Exposure to lead or bismuth leads to development of a
in diameter. The vermilion border of the lower lip is the
bluish line along the marginal gingiva or similar spots
most common site and rarely occurs inside oral cavity.
within the gingival papillae, while diffuse bluish-black

Figure 18.35  (a,b) Diffuse bluish-black pigmented mac-

ule on lip, buccal mucosa, and gingiva due to exposure to
silver. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Oral mucosa  389

the first and second branches of the trigeminal nerve.

Ipsilateral sclera may also be involved.
●● The oral cavity may have blue-black macules commonly
involving the palate and buccal mucosa.

Melanoma
●● Primary oral melanomas are rare and represent less

than 1% of total melanomas. The most common sites are

the hard palate and maxillary gingiva.
●● Clinically, it presents as a well-circumscribed or

irregular, black or brown, focally or diffusely pigmented

macule, plaque, or nodule that may be ulcerated. Diffuse
but contiguous pigmentation is of more concern than
diffuse non-contiguous pigmentation.
●● Based on clinical and histopathological criteria, oral

melanoma is classified into three forms: lentigo maligna

melanoma (best prognosis), superficial spreading melanoma
(good prognosis), and nodular melanoma (poor prognosis).

Addison’s disease
●● Addison’s disease is caused by trauma, autoimmune

disease, infectious agents, neoplasia, genetic disease,

certain medications, and iatrogenic causes.
●● Mucocutaneous pigmentation is often one of the earliest

clinical manifestations of hypoadrenocorticism.

●● It is clinically characterized by diffuse or patchy dark-

brown pigmentation, due to melanin production. The

buccal mucosa, palate, lips, and gingiva are the most
Figure 18.36  Diffuse dark-brown irregular pigmented common sites of involvement (Figure 18.37a,b).
macules.

Pigmented nevi
●● These involve a benign proliferation of melanocytes and

“nevus cells,” rare in the oral mucosa.

●● Among pigmented nevi, the intramucosal type is the

most common in oral cavity, followed by blue nevi.

Junctional and compound nevi are rare in oral cavity.
●● They present as an asymptomatic small, well-

demarcated, solitary, brown or blue, well-circumscribed

macule or plaque.
●● The hard palate, buccal, labial and gingival mucosa, and

lips are the most typical sites.

●● Nevus cells tend to be round, ovoid, or spindle-shaped,

while the basal layer melanocytes are dendritic in

appearance.
●● Intramucosal, compound, and junctional nevi likely share

a common pathogenesis, while blue nevi cells usually reside

deep in the connective tissue, giving the lesion a bluish tint.

Nevus of Ota
●● It is a developmental disorder characterized by

hamartomatous presence of the melanocytes; it

predominantly involves the skin of the face and eyes,
and mucous membrane.
●● It usually appears in early childhood and more

commonly occurs in females.

●● Clinically it presents as multiple mottled black or brown
Figure 18.37  (a) Diffuse dark-brown pigmentation of buc-
macules of variable size over the face in distribution of cal mucosa and gingiva. (Continued)
390  Oral mucosa

●● The perioral skin, lips, buccal mucosa, and tongue are

the most common sites affected.
●● The skin lesions consist of numerous, usually perioral,
dark spots.
●● Intestinal polyps (hamartomas) are constant findings,
usually in the jejunum and ileum.

Melanoacanthoma
●● This is a relatively uncommon melanocytic lesion that

may cause rapid, diffuse, and dark pigmentation of a

large mucosal area.
●● It usually presents as an asymptomatic, solitary, ill-

defined, rapidly enlarging, macular pigmentation

mostly involving buccal mucosa.

RED LESIONS
Hereditary disorders
1. Vascular lesions (Table 18.7)
2. Hereditary mucoepithelial dysplasia
●● This is an autosomal dominant dyskeratotic
Figure 18.37 (Continued)  (b) Pigmentation of lip and epithelial syndrome affecting oral and other
gingiva in Addison’s disease. (a – Courtesy: Dr. Santoshdev
mucosae.
Rathod, Ahmedabad, India; b – Courtesy: Dr. Soumyajit
Roychoudhury, Berhampore, India.)
●● It occurs on the palate and gingiva.
●● It presents as intraoral erythematous maculo-
papular lesions and periorificial red patches.
Peutz-Jeghers syndrome
●● This is a rare genetic disorder of autosomal dominant
Acquired disorders
inheritance, characterized by mucocutaneous
pigmentation and intestinal polyposis. Traumatic erythema
●● Oral manifestations are the most important diagnostic ●● It can present either as an ecchymosis or as a hematoma,
findings and consist of oval or round, brown or black which appears as an irregular, flat area with a bright or
macules or spots, 1–10 mm in diameter (Figure 18.38a,b). deep red color.

Figure 18.38  (a) Multiple dark brown to black macules in oral

cavity. (b) Pigmented macules on the lip and periorbital area.
(Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Oral mucosa  391

Table 18.7  Vascular causes of erythematous mucosal

lesions

Vascular condition Type of lesions Sites

Vascular Depends on the Anywhere in
malformation nature of vessels the buccal
(Figure 18.39a–c) involved cavity
• Capillary
malformation:
Erythematous
patch
• Venous
malformation:
Red- blue
papulonodular
lesions
• Mixed: variable
clinical
presentation
Hereditary Telangiectasia Lip
hemorrhagic Hemorrhagic Gingiva,
telangiectasia vesicles, buccal
syndrome nodules, mucosa
(Figure 18.39d,e) papules, and
ulcers
Sturge-Weber- Slight vascular Gingiva
Krabbe syndrome hyperplasia to
(Figure 18.40a,b) Hemangioma
Klippel-Trenaunay- Hemangioma Gingiva
Weber syndrome Macroglossia
Maxillary
hyperplasia
Gingival
fibromatosis
Blue rubber-bleb Soft, compressible Buccal
nevus syndrome and non-tender mucosa,
bluish vascular retromolar
swelling trigone and
ventrolateral
tongue
Maffucci syndrome Hemangioma Tongue

●● Common sites include the lips, tongue, and buccal

mucosa.
●● DD: Other causes of hematoma (i.e., thrombocytopenia,
anticoagulant therapy).

Thermal burn
●● It appears as a red, painful erythema that may undergo

desquamation, leaving erosions.

●● It usually heals spontaneously in about a week.

●● DD: Chemical burn, traumatic lesions, herpes simplex.

Figure 18.39  (a) Diffuse swelling of lower lip in capil-
lary malformation. Note the surface ulceration. (b) Soft
Radiation mucositis compressible red-blue focal swelling of the lip in venous
●● This is an adverse effect of radiation therapy for head malformation. (c) Inner aspect of the swelling. Bluish dis-
and neck tumors. coloration of dorsum of tongue. (Continued)
392  Oral mucosa

Figure 18.39 (Continued)  (d) Hereditary hemorrhagic telangiectasia syndrome with multiple telangiectatic papules on
tongue. (e) Telangiectasis on the ventral aspect of tongue in Hereditary hemorrhagic telangiectasia syndrome. (a–c –
Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Early lesions in form of erythema and edema usually

start at the end of first week of therapy.
●● At a later stage it may have erosions and ulcers covered
with yellow-white exudate.
●● Associated features are dryness of mouth, taste
alteration, and burning and pain during mastication,
swallowing, and speech.

Median rhomboid glossitis

●● This is a rare condition that may be associated with

candidiasis and affect the dorsum of the tongue exclusively.

Figure 18.40  (a) Unilateral dusky erythematous plaque involving oral cavity, chin, lip, and nose. (b) Bright erythematous
macule involving the lip in a case of port wine stain. (Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)
 Oral mucosa  393

●● It is typically a rhomboidal-shaped erythematous central ●● It usually involves the floor of the mouth, the ventrum
lesion anterior to the sulcus terminalis. The surface of tongue, or the soft palate.
of lesion may be smooth or lobulated. Sometimes ●● It appears as a usually asymptomatic, erythematous,
erythematous candidiasis in the palate may coexist. smooth, well-demarcated plaque with velvety surface.
On biopsy, 75–90% cases of erythroplasia show severe
Denture-related stomatitis dysplasia, carcinoma, or carcinoma in situ.
●● It is a common condition consisting of mild ●● DD: Erythematous candidiasis, lichen planus, early SCC.
inflammation and erythema in patients who wear
dentures continuously for long time. Plasma cell gingivitis
●● Caused by mechanical trauma from dentures, it is a ●● This is a unique gingival disorder due to local allergens

tissue response to accumulated microorganisms between or chronic infection, characterized histopathologically

dentures and infections with Candida species mainly. by chronic inflammatory infiltration of plasma cells.
●● It is characterized by diffuse chronic erythema and ●● It appears as localized or widespread erythematous

edema, and sometimes petechiae are restricted glossy lesions with edema and frequently accompanied
to denture area and white spots that represent by burning sensation.
accumulations of candidal hyphae. Mucosa below lower ●● DD: Candidiasis, desquamative gingivitis,

dentures is rarely involved. erythroplakia.

●● Newton’s three clinical types:

●● Type 1: localized mild inflammation or a pinpoint Lupus erythematosus

hyperemia ●● Oral lesions may develop in 15–25% of cases in discoid

●● Type 2: generalized simple type, more diffuse erythema lupus erythematosus (DLE) and 30–45% of cases in
involving a part of or the entire denture-covered mucosa systemic lupus erythematosus (SLE).
●● Type 3: a granular type, inflammatory papillary ●● The oral lesions are characterized by a well-defined

hyperplasia commonly involving the central part of central erythematous atrophic area surrounded by
the hard palate and the alveolar ridge a sharp elevated white border with fine irregular
perpendicular white paint-brush–like lines. Common
Erythroplakia sites are the palate and buccal mucosa (Figure 18.41a–c).
●● It is a premalignant lesion frequently occurring on the ●● Telangiectasia, petechiae, edema, erosions, ill-defined

glans penis and rarely on the oral mucosa. It occurs superficial ulcerations, and white hyperkeratotic plaques
more frequently at 50 to 70 years of age. may be seen.

Figure 18.41  (a) Central erythematous atrophic plaque

with whitish border involving palate and (b) small ero-
sions surrounded by whitish border on buccal mucosa.
(c) Erythematous plaque of discoid lupus erythematosus
on the lip. There are some focal verrucous changes.
(a – Courtesy: Dr. Hiral Shah, Baroda Medical College,
Vadodara, India; c – Courtesy: Dr. Piyush Kumar, Katihar, India.)
394  Oral mucosa

●● Common symptoms are dryness, soreness, and a ●● Prodromal symptoms include anorexia, malaise,
burning sensation. headache, fatigue, and fever.
●● DD: Lichen planus, speckled leukoplakia, erythroplakia, ●● Oral manifestations consist of palatal petechiae, uvular
cicatracial pemphigoid. edema, tonsillar exudate, gingivitis, and rarely ulcers
may also appear.
Anemia ●● Other features are maculopapular skin rash, sore
●● In anemia, pernicious anemia, iron-deficiency anemia, and generalized lymphadenopathy, hepatosplenomegaly,
Plummer-Vinson syndrome usually affect the oral mucosa. maculopapular skin rash, and sore throat.
●● Iron-deficiency anemia may manifest as atrophic ●● DD: Leukemia, diphtheria, secondary syphilis,
glossitis (flattening of the tongue papillae), mucosal thrombocytopenic purpura.
pallor, and angular cheilitis.
●● In megaloblastic anemia, painful atrophy of the entire Reiter’s disease
oral mucosa and tongue (glossitis), stomatitis, recurrent ●● It is an uncommon multisystemic disorder involving the

aphthous ulcers, and magenta tongue are common features. genitourinary tract, conjunctiva, and joints.
●● Sickle cell anemia may manifest as orofacial pain, ●● The disease is mediated by an immunological

gingival enlargement, buccal mucosa pallor, and mechanism that is triggered by infectious
alveolar bone changes (step ladder appearance). agents in genetically susceptible individuals and
●● Aplastic anemia presents as oral and facial petechiae, predominantly affects young men aged 20 to
gingival hyperplasia, spontaneous gingival bleeding, 30 years.
hemorrhagic bullae, candidiasis, and herpetic lesions. ●● Major clinical manifestations are symmetrical arthritis,

nongonococcal urethritis, conjunctivitis, balanitis,

Thrombocytopenic purpura prostatitis, cervicitis, psoriasiform skin lesions, and
●● This is characterized by hemorrhagic petechiae, palmoplantar keratoderma.
ecchymoses, blisters, or even hematomas, usually located ●● Oral involvement may occur in 20–40% of the cases

on the palate and buccal mucosa (Figure 18.42a,b). and is characterized by erythematous papule or
●● Spontaneous gingival bleeding is a constant early finding. diffuse erythematous areas intermixed with thin
●● DD: Aplastic anemia, leukemias, SJS-TEN. whitish dots or lines and painful superficial
erosions over buccal mucosa, gingiva palate,
Infectious mononucleosis and lips.
●● It is an acute, self-limited infectious disease caused by ●● The tongue lesions may mimic geographic tongue.
Epstein-Barr virus transmitted through saliva transfer; ●● DD: Behçet’s disease, geographic tongue, and
it primarily affects children. SJS-TEN.

Figure 18.42  (a) Multiple hemorrhagic papules on ventral aspect of tongue. (b) Large vesicle and bullae on buccal mucosa.
(Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)
 Oral mucosa  395

VERRUCOUS LESIONS ●● Common sites are the ventral tongue, frenulum, and
soft palate.
Papilloma due to chronic irritation ●● DD: Verruca vulgaris, verrucous carcinoma, condyloma
●● Chronic irritation or trauma of mucosa may lead to acuminatum.
benign proliferation of stratified squamous epithelium.
●● Clinically presents as 0.5- to 1-cm whitish gray, Verruca vulgaris
painless, exophytic, well-circumscribed pedunculated ●● An infection caused by human papillomavirus (HPV),
lesions with finger like projections giving a rough or it rarely involves oral mucosa and is more common in
cauliflower appearance (Figure 18.43a,b). children.
●● Common types of HPV responsible for oral lesions are

types 2, 4, and 40.

●● The vermilion border and the lip mucosa, keratinized

surface of gingiva, commissures, and tongue are

common sites.
●● Clinically, it appears as a single or multiple

painless, white, small, sessile, and well-defined

exophytic growth with a cauliflower surface
(Figure 18.44).
●● DD: Papilloma, condyloma acuminatum, focal

epithelial hyperplasia.

Condyloma acuminatum
●● Sexually transmitted benign lesions usually involve the

anogenital region; they may rarely may occur in oral

cavity.
●● It is caused by the human papillomavirus types 2, 6, and

11.
●● Sites are the labial mucosa, soft palate, and lingual

frenum.

Figure 18.43  (a) Repetitive trauma resulting in solitary

pinkish-white growth forming a papilloma on labial Figure 18.44  Verrucous plaques of verruca vulgaris involv-
mucosa. (b) Single, sessile, well-defined exophytic ing the upper gingiva and adjacent inner surface of upper
cauliflower like growth on labial mucosa. (b – Courtesy: lip. (Courtesy: Dr. Dependra Kumar Timshina, Remedy
Dr. Shweta Jain, Mumbai, India.) Clinic, Siliguri, India.)
396  Oral mucosa

●● Clinically, lesions are similar to papilloma but larger

in size and more clustered, dense, and deeply rooted
(Figure 18.45).
●● DD: Papilloma, verruca vulgaris, focal epithelial
hyperplasia, early verrucous carcinoma.

Focal epithelial hyperplasia (Heck’s disease)

●● Benign hyperplastic lesions caused by HPV 13 and

32 are increasingly seen in HIV-positive

individuals.
●● Sites are the buccal, labial, and lingual mucosa.

●● Clinically it presents as mucosal-colored multiple soft,

smooth, painless, sessile, dome-shaped nodules or

plaques 3–10 mm in diameter and lacking papillary
surface.
●● DD: Condyloma acuminatum, verruca vulgaris, and

multiple papillomas.

Verruciform xanthoma
●● Verruciform xanthoma is rare hyperplastic disorder

caused by trauma.
●● Sites are the gingiva, alveolar ridge, tongue, and palate.

●● It presents as a reddish-yellowish, well-demarcated,

painless, sessile, slightly elevated lesion. It has a

cauliflower-like surface (Figure 18.46a,b).
●● DD: Papilloma, verruca vulgaris, condyloma

acuminatum, verrucous carcinoma.

Figure 18.45  Large verrucous growth with marked indura- Verrucous carcinoma
tion and hemorrhage on lower lip. ●● It is a slow-growing, well-differentiated low-grade

variant of squamous cell carcinoma presenting

Figure 18.46  (a) Multiple reddish-yellow verrucous

papules on dorsum of tongue. (b) Yellowish verrucous
plaque on the lower lip. (b – Courtesy: Dr Piyush Kumar,
Katihar, India.)
 Oral mucosa  397

Figure 18.47  Verrucous carcinoma with leukoplakia.

(Courtesy: Dr. Hiral Shah, Baroda Medical College,
Vadodara, India.)

Figure 18.48  Multiple whitish confluent papules on floor

as an exophytic, whitish mass with a papillary or of mouth. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
verruciform surface (Figure 18.47).

Acanthosis nigricans ●● C1 esterase inhibitor deficiency:

●● Approximately 40% of malignant acanthosis nigricans
– Hereditary – onset occurs in childhood
(AN) show oral lesions. – Type I: low levels of functionally normal
●● Sites are the vermilion border of lips, tongue, and
C1-esterase inhibitor
gingiva. – Type II: dysfunctional C1-esterase
●● Clinically it presents as mucosal-colored multiple,
inhibitor
small, painless, papillomatous and verrucous lesions. – Acquired – usually occurs in adulthood
Mild gingival swelling, hypertrophy, and elongation ●● The swelling may affect lips, tongue, or other
of the filiform papillae (shaggy tongue) have also areas.
been reported. Unlike skin lesions, oral AN is rarely ●● It is usually only mild and transient but has potential for
pigmented. obstruction of the airway.
●● DD: Focal epithelial hyperplasia, multiple papillomas,

lipoid proteinosis, multiple verruca vulgaris, pemphigus Abscesses

vegetans. ●● These are mostly odontogenic in origin as a

consequence of dental caries.

Darier disease ●● Rarely they may be the result of trauma, a foreign body,
●● Darier disease is an autosomal dominant inherited
or infections such as actinomycosis, nocardiosis, or
dyskeratotic acantholytic disorder. botryomycosis.
●● Site are the palate, gingiva, buccal mucosa, and tongue. ●● They usually discharge in the mouth on buccal gingiva
●● Oral lesions occur in 20–40% of cases and appear as
but sometimes discharge palatally, lingually, on the chin
small multiple whitish confluent papules, which may or submental region.
become hypertrophic, assuming a cobblestone or
papillary pattern (Figure 18.48). Mucocele (mucous cyst or ranula)
●● DD: Acanthosis nigricans, papillary hyperplasia of palate. ●● This is a benign, common, mucus-containing cystic

lesion of the minor salivary glands in the oral

LUMPS AND SWELLINGS cavity.
●● These lesions are not true cysts, not having an epithelial
Angioedema lining.
●● It can be caused by ●● Based on location, they can be superficial (directly

●● Allergic angioedema: drugs (local anesthetics, under the mucosa), classic (in the upper submucosa), or
angiotensin converting enzyme inhibitors), deep (in the lower corium). Deeper mucoceles are more
exposure to latex or certain food substances. common.
398  Oral mucosa

●● Based on histological features, two types of mucoceles ●● When the mucus accumulates in the deep soft tissues,
are described. the presentation is as an enlarging, painless mass
●● Extravasation cysts are pseudo-cysts that result assuming the pink coloration of the mucosa.
from extravasation of mucous in the peri- ●● The treatment of choice for a deep mucocele and the
glandular connective tissue by a rupture of the classic form is surgical excision, which should include
salivary duct with a partial epithelial lining the immediate adjacent glandular tissue.
(mucous pseudocyst, 92%).
●● Retention cysts surrounded by salivary tissue with Fibroma
an epithelial lining composed of one or two layers ●● It is considered a reactive hyperplastic connective tissue

of flat or cuboidal epithelial cells (true mucous growth rather than a true neoplasm. Accidental biting,
cyst, 8%). other trauma, and irritation are major causative factors.
●● It appears as a painless dome-shaped, translucent, ●● It typically presents as an asymptomatic, well-

whitish-blue smooth-surfaced swelling ranging in size defined, firm, sessile or pedunculated pink papule or
from a few millimeters to centimeters (Figure 18.49a). nodule with a smooth surface of normal epithelium
The lesion may become irregular and whitish due to (Figure 18.50a).
multiple cycles of rupture and healing caused by trauma ●● Most common sites are the tongue, buccal mucosa,

or puncture. and lips.

●● Sites are the lower lip, floor of the mouth, cheek, palate, ●● Angiofibroma is a characteristic lesion of

retromolar fossa, and the tongue; the upper lip is tuberous sclerosis. They are present as discrete to
usually spared. coalescing skin-colored to dark-brown firm
●● Larger lesions most often affect the floor of the mouth; papules, nodules, and plaques. Rarely an oral
these are called ranulas because of the similarity to the cavity can also be involved, and the gingiva is the
throat pouch of frogs (Figure 18.49b). most common site. Usually they are located on

Figure 18.49  (a) Single dome-shaped translucent swelling

(mucocele) on ventral aspect of tongue. (b) Single large
translucent swelling on floor of mouth. (a – Courtesy:
Dr. Devrashetti Srinivas, Nizamabad, India; b – Courtesy:
Dr. Rajesh Kumar Mandal, North Bengal Medical
College, Darjeeling, India.)
 Oral mucosa  399

Figure 18.50  (a) Well-defined firm papule on tip of tongue. (b) Gingival fibroma presenting as erythematous firm nodular
plaque with numerous angiofibromas on face in patient tuberous sclerosis.

the face, involving the nose, nasolabial fold, and ●● The gingiva is the most common site, followed
cheek (Figure 18.50b). by the tongue, lips, and buccal mucosa
(Figure 18.51a,b).
Pyogenic granuloma
●● A painless, red, firm, vascular reactive growth presents Gingival hyperplasia
as a pedunculated or sessile mass with a smooth or It is characterized by overgrowth of submucosal tissue cov-
lobulated surface. Sometimes it may ulcerate and have ered with normal epithelium, leading to enlargement of the
a covering of whitish fibrinous membrane. It is soft to gingiva (Table 18.8). It can present as generalized or local-
the touch and has a tendency to bleed easily. ized enlargement.

Figure 18.51  (a) Pyogenic granuloma seen as friable, eroded erythematous nodule on the lip. (b) Large protruding lesion
of pyogenic granuloma lip. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
400  Oral mucosa

Table 18.8  Causes of gingival hyperplasia

Generalized gingival Localized gingival

hyperplasia hyperplasia
Congenital Congenital
• Hereditary gingival • Fabry syndrome
fibromatosis • Cowden syndrome
• Mucopolysaccharidosis • Tuberous sclerosis
• Hypoplasminogenemia • Sturge-Weber
angiomatosis
Acquired
• Gingival granular cell
• Hyperplastic gingivitis
tumor
• Mouth-breathing gingivitis
• Pregnancy Acquired
• Puberty • Abscess (periodontal
• Scurvy abscess)
• Hematological (leukemia, • Cysts (gingival cyst, Figure 18.52  Lip-licking dermatitis presenting as scaling
aplastic anemia, eruption cyst) and pigmentation around the lips. (Courtesy: Dr. Piyush
Kumar, Katihar, India.)
lymphoma) • Pyogenic granuloma
• Drug-induced (phenytoin, • Neoplasms
calcium channel blockers, (peripheral giant-cell
cyclosporine, oral granuloma, peripheral Contact cheilitis
contraceptive) ossifying fibroma)
●● Common irritants or allergens include lipsticks,
• Systemic (Wegener’s
mouthwashes, dental preparations, and food.
granulomatosis, primary
●● It is confined to the vermilion border of both lips.
amyloidosis, sarcoidosis,
●● It is characterized by mild edema and erythema,
Crohn’s disease, Kaposi
followed by irritation and thick scaling
sarcoma)
(Figure 18.53).

LIP LESIONS
Cheilitis
It is an inflammatory condition that may be primary in
origin or secondary due to disease of nearby skin and
mucosa.
Causes
●● Chapping of lips

●● Eczematous cheilitis – atopic dermatitis

●● Contact cheilitis

●● Drug-induced cheilitis

●● Infective cheilitis

●● Angular cheilitis

●● Actinic cheilitis

●● Cheilitis glandularis

●● Granulomatous cheilitis

●● Factitious (exfoliative) cheilitis

Lip-licking dermatitis
●● It is due to chronic cheilitis.
●● It is characterized by erythema, scaling, and crusting of

the lips and the perioral skin (Figure 18.52). Fissuring

may also occur in severe cases. A burning sensation is
the most common symptom.
●● DD: Perioral dermatitis, eczematous cheilitis, contact Figure 18.53  Diffuse erythema, edema, and thick scaling
cheilitis. on both lips.
 Oral mucosa  401

Figure 18.54  Lower lip having central erosion surrounded

by erythematous plaque.

Actinic cheilitis
●● Actinic cheilitis is a chronic degenerative disorder of the

lower lip due to long sun exposure.

●● The premalignant condition usually occurs in people

more than 50 years of age.

●● It starts as mild erythema, edema with fine scaling

and dryness. It slowly progress to thinned, smooth

epithelium with mixed whitish-gray and erythematous
plaques. Erosions and nodular lesions may also develop
(Figure 18.54). Figure 18.55  Angular cheilitis.
●● DD: Leukoplakia, lichen planus, lupus erythematosus,

early squamous-cell carcinoma, cheilitis due to Cheilitis glandularis

radiation.
●● It is due to inflammation and swelling of the salivary glands

of the lips. It may be premalignant in 20–30% of cases.

Angular cheilitis ●● It clinically presents as swelling of lower lip, with
●● It is caused by infections, immune deficiency,
dilated pinhead-sized orifices of salivary glands.
nutritional deficiency, or mechanical trauma.
●● Characteristic triangular lesions consist of erythema,

maceration, and edema at one or both commissures,

associated with burning sensation and dryness. In
severe cases linear fissures radiating from the angle of
mouth to the mucocutaneous border may be present
(Figure 18.55).

Exfoliating cheilitis
●● This chronic inflammatory disorder more commonly

develops in young females with a personality disorder.

●● Most of the time it is confined to the vermilion border

of lips.
●● It is associated with a burning sensation.

●● Usually it starts from the middle of the lower lip and

spreads to involve the whole of the lower or both lips;

it persists in varying severity for months or years and
leads to hyperkeratotic thickening, fissuring, and
crusting (Figure 18.56). Figure 18.56  Exfoliating cheilitis.
402  Oral mucosa

Figure 18.57  Mucous fluid coming out from the orifices

in glandular cheilitis. (Courtesy: Dr. Sanjay Khare, MGM
Medical College, Indore, India.)

On squeezing them, mucous saliva comes out

(Figure 18.57).
●● Volkmann’s cheilitis is a severe and suppurative form
in which the lip is tender, enlarged, and covered with Figure 18.58  Diffuse swelling of lower lip in case of
crusts and scales covering salivary duct orifices. granulomatous cheilitis. (Courtesy: Dr. Hiral Shah, Baroda
●● DD: Cheilitis granulomatosa, Melkersson-Rosenthal Medical College, Vadodara, India.)
syndrome, Crohn’s disease, sarcoidosis, cystic
fibrosis.
REFERENCES
Granulomatous cheilitis 1. Laskaris G. Pocket Atlas of Oral Diseases. 2nd edition. New York:
●● This is a chronic swelling of the lip due to Thieme; 2005.
granulomatous inflammation of unknown cause. 2. Villa A, Woo SB. Leukoplakia—A Diagnostic and Management
●● The granulomatous changes are confined to the lip, and Algorithm. J Oral Maxillofac Surg 2017;75:723–734.
3. Maymone MBC, Greer RO, Burdine LK, Dao-Cheng A, Venkatesh
this is generally regarded as a monosymptomatic form S, Sahitya PC et al. Benign oral mucosal lesions: Clinical and patho-
of Melkersson-Rosenthal syndrome. logical findings. J Am Acad Dermatol 2019;81:43–56.
●● Melkersson-Rosenthal syndrome 4. Maymone MBC, Greer RO, Kesecker J, Sahitya PC, Burdine
●● It appears in a relapsing and remitting course. First LK, Cheng AD et al. Premalignant and malignant oral mucosal
lesions: Clinical and pathological findings. J Am Acad Dermatol
episodes may subside in hours or days, but recurrent
2019;81:59–71.
episodes may persist longer. 5. Scully C. Dermatoses of the Oral Cavity and Lips. In: Griffiths
●● It is characterized by a sudden diffuse nodular CEM, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook’s
swelling of upper lip, lower lip, or cheek in Textbook of Dermatology. 9th edition. Oxford: Blackwell Science;
decreasing order of frequency, labial swelling (75% 2016. pp. 110.1–110.93.
6. Çerman AA, Altuna IK. Oral pigmentation. J Pigment Disorder
of cases), facial edema (50% of cases), facial nerve
2016;3:234.
palsy of lower motor neuron type (30%), fissured 7. Fitzpatrick SG, Cohen DM, Clark AN. Ulcerated lesions of the
tongue (20–40%), and regional lymphadenopathy oral mucosa: Clinical and histologic review. Head Neck Pathol
(50% of cases). 2019;13:91–102.
●● It presents as a painless, persistent, and diffuse swelling 8. McNamara KK, Kalmar JR. Erythematous and vascular oral mucosal
lesions: A clinicopathologic review of red entities. Head Neck
of one or both lips. Small vesicles, erosions, and scaling
Pathol 2019;13:4–15.
may also occur (Figure 18.58). 9. Kauzman A, Pavone M, Blanas N, Bradley G. Pigmented lesions of
●● DD: Cheilitis glandularis, Crohn’s disease, sarcoidosis,
the oral cavity: Review, differential diagnosis, and case presenta-
cystic fibrosis, lymphangioma, angioneurotic edema. tions. J Can Dent Assoc 2004;70:682–683.
 E24
Tongue

MEENAZ KHOJA, SABHA MUSHTAQ

ABSTRACT
The tongue is the most mobile organ of the body and is the special organ for taste. It is also closely linked to the digestive sys-
tem and speech. The appearance of the tongue gives an assessment or diagnosis of various diseases of regional and systemic
importance.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-45 403

 E25
Axillae and groins

SHVETHA JAIN

ABSTRACT
The axillae and groin areas are commonly affected by various dermatoses. Most common lesions involving these areas may be
readily identified and easily managed. However, at times there may be diagnostic dilemmas when they are involved second-
arily to underlying systemic diseases.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

404 DOI: 10.1201/9781351054225-46

 E26
Umbilicus and periumbilical region

DIVYA SACHDEV

ABSTRACT
The umbilicus may be affected in various congenital or acquired malformations, primary cutaneous disease – both localized
and generalized – and various systemic conditions. Additionally, benign, malignant, and metastatic tumors may involve the
umbilicus, and some of them are unique to this site. This chapter discusses a morphological approach to the diagnosis of vari-
ous signs and dermatoses involving the umbilical and periumbilical region.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-47 405

 E27
Palms and soles

PRERNA, RASHID SHAHID

ABSTRACT
Palms and soles may be affected by a variety of dermatoses localized to these areas, widespread dermatoses, and systemic dis-
eases. As palms and soles are specialized skin with thicker stratum corneum, various dermatoses may not manifest themselves
with typical morphology. Also, mechanical trauma frequently experienced by these sites may alter the morphology, contribut-
ing to diagnostic confusion. This chapter discusses the clinical approach to various palmoplantar dermatoses.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

406 DOI: 10.1201/9781351054225-48

 19
Male genitalia

PIYUSH KUMAR, RASHID SHAHID

INTRODUCTION ●● DD: Lichen sclerosus et atrophicus, and post-

inflammatory hypopigmentation.
Various dermatoses are known to be exclusively localized
to or predominantly affecting male genitalia. Awareness of Melanocytic nevus
such conditions aids in the efficient management of such ●● A melanocytic nevus may be congenital or acquired.

patients. This chapter focuses on various dermatoses affect- ●● Acquired types have been further classified as

ing external male genitals. The diseases affecting the scro- junctional, intradermal, or compound.
tum have been discussed in a separate chapter. This chapter ●● Melanocytic nevi of genital region are rare and usually

primarily focuses on dermatoses affecting the penis. The present as an asymptomatic lesion, varying in color
clinical approach to penile dermatoses has been summa- from brown to jet black.
rized in Table 19.1.1–5 ●● Acquired lesions are typically less than a centimeter in

diameter, whereas congenital melanocytic nevi vary in

Vitiligo size and can be small (≤1 cm), intermediate (1–3 cm), or
●● Male genitalia may be affected exclusively or as a part large/giant (≥3 cm).
of widespread disease as sharply demarcated patches ●● Junctional melanocytic nevi present as brown to
of milk-white skin that have no evidence of texture brownish-black macular or thin papular lesions.
change, inflammation, or scales (Figure 19.1). Compound melanocytic nevi are tan to light-
brown papules; and intradermal melanocytic nevi
present as papules with no significant pigmentation
(Figure 19.2).
●● One subtype of genital melanocytic nevus, known as

atypical genital nevus, is characterized by the presence

of atypical melanocytes and may resemble melanoma
and dysplastic melanocytic nevus on histopathology but
exhibits a benign behavior.
●● DD: Dysplastic nevus, and cutaneous melanoma.

Lentigines
●● Lentigines are foci of macular hyperpigmentation

in which the number of epidermal melanocytes is

increased.
●● Benign genital lentiginosis is an uncommon,

idiopathic cutaneous abnormality consisting of

irregular hyperpigmented macules and patches
(Figure 19.3).
●● This totally flat hyperpigmentation sometimes displays

marked irregularity of browns, tans, and black that

mimic melanoma.
●● DD: Post-inflammatory hyperpigmentation, and

Figure 19.1  Well-demarcated depigmented patch. malignant melanoma.

DOI: 10.1201/9781351054225-49 407
408  Male genitalia

Table 19.1  Clinical approach to dermatoses of male genitalia

Morphology Feature Diseases Morphology Feature Diseases

Macule/ Depigmented/ Vitiligo Nodule Squamous cell
Patch hypopigmented carcinoma
Hyperpigmented Melanocytic nevus Lymphoma
Lentigines Epidermoid cyst
Dyspigmentation of Steatocystoma
median raphe multiplex
Erythematous Balanoposthitis Scrotal calcinosis
Plasma cell balanitis Penile thrombophlebitis
Fixed drug eruption Peyronie’s disease
Circinate balanitis Mucoid penile cyst
Seborrheic dermatitis Vesicle Long standing/ Lymphangioma
Irritant contact persistent Median raphe cyst*
dermatitis Recurrent/ short Erythema multiforme
Diaper dermatitis duration Genital herpes
Papule White Molluscum contagiosum Irritant contact
Lichen nitidus dermatitis
Hyperpigmented Melanocytic nevus Pustule Candidiasis
Lichen planus Dermatophytosis
Bowenoid papulosis Erosion Pemphigus vulgaris
Red Scabies Bullous pemphigoid
Dermatophytosis Cicatricial pemphigoid
Allergic contact Stevens-Johnson
dermatitis syndrome and toxic
Hemangioma epidermal necrolysis
Angiokeratoma Acrodermatitis
Yellowish Sebaceous hyperplasia enteropathica
Flesh-colored Pearly penile papule Ulcer Painless Chancre
Skin tag Squamous cell
Syringoma carcinoma
Granuloma annulare Melanoma
Plaque Red Hemangioma Basal cell carcinoma
Psoriasis Painful Genital herpes
Erythroplasia of Queyrat Aphthous ulcer
Extramammary Paget’s Chancroid
disease Donovanosis
Genitourinary tuberculosis Dermatitis artefacta
Leprosy Vasculitis
Lymphoma Crohn’s disease
Flesh-colored Condyloma lata Pyoderma gangrenosum
Calciphylaxis
Depigmented Lichen sclerosus
Malakoplakia
Hyperkeratotic/ Porokeratosis
Cyst* Median raphe cyst
verrucous Pseudoepitheliomatous,
keratotic, and Sinus Actinomycosis
micaceous balanitis Scrofuloderma
Buschke-Lowenstein Hidradenitis suppurative
tumor Edema Lymphedema
Verrucous carcinoma Paraphimosis
Verruciform xanthoma Priapism
Pigmented Acanthosis nigricans Penile fracture
Bowen’s disease No skin Phimosis
Melanoma lesion Hypospadias
Basal cell carcinoma Gonorrhea
*Clinically, it looks like a clear fluid containing vesicle.
 Male genitalia  409

Dyspigmentation of median raphe

●● Dyspigmentation of median raphe is a normal variant

of median raphe of the penis and usually presents as a

pigmentary problem.
●● The median raphe may be hyperpigmented or

hypopigmented.
●● It is one of the most common physiological variations.

●● DD: Post-inflammatory hyperpigmentation.

Balanoposthitis
●● Balanoposthitis is defined as inflammation of the

glans penis (balanitis) and prepuce (posthitis) in

uncircumcised males and presents as moist macular
erythema.
●● Infections (such as Candida species), mechanical

factors, trauma, contact dermatitis, and dermatoses

(such as genital herpes, Zoon’s balanitis, fixed
drug eruption, lichen sclerosus et atrophicus, and
Erythroplasia of Queyrat) are frequently responsible for
its appearance.
●● Candidal balanoposthitis is characterized by pustules

and tiny erosions, white scrapable sloughs, and fissures.

Sometimes, prepuce is edematous and transiently
adhered to glans (Figure 19.4a).
●● Genital herpes presents with painful, tiny vesicles and

erosions (Figure 19.4b).

Figure 19.2  “Kissing” melanocytic nevus on the penis. ●● Zoon’s balanitis is characterized by a well-
(Courtesy: Dr. Santoshdev P Rathod, Ahmedabad, India.) circumscribed, bright-red patch with a characteristic

Figure 19.4  (a) Glans penis in candidiasis appears ery-

Figure 19.3  Multiple hyperpigmented macules of lentigines. thematous and eroded. Prepuce cannot be retracted and
(Courtesy: Dr. Santoshdev P Rathod, Ahmedabad, India.) shows fissures and whitish slough. (Continued)
410  Male genitalia

Figure 19.4 (Continued)  (b) Genital herpes present-

ing as multiple grouped erosions. (c) Plasma cell
balanitis presenting with erythematous patch with
well-demarcated margin and cayenne pepper spots.
(d) Well-defined erosion with clean surface. Margin
shows some pigmentation. (d – Courtesy: Dr. PC Das,
Katihar, India.)

glazed appearance and multiple pinpoint redder spots – ●● It is clinically characterized by a solitary, asymptomatic
“cayenne pepper spots” (Figure 19.4c). erythematous, or erythematous-brownish, sharply
●● Fixed drug eruption presents as well-demarcated marginated patch on the glans and/or the inner
erythematous areas that may ulcerate and follows intake foreskin, 1.5 cm or more in diameter, with a bright,
of certain specific drugs (Figure 19.4d). smooth, and sometimes erosive surface.
●● Lichen sclerosus et atrophicus presents as white ●● When occurring on the glans, it is seen only in
indurated plaques on the glans and may involve the uncircumcised men (Figure 19.5a,b).
prepuce, which becomes thickened, fissured, and ●● DD: Lichen planus, psoriasis, candidiasis, and
non-retractile. squamous cell carcinoma in situ.
●● Erythroplasia of Queyrat is a premalignant lesion and
presents as red, velvety appearance with sharp margins, Fixed drug eruption
and a granular surface.
●● Fixed drug eruption is a peculiar cutaneous eruption
●● DD: Bowen’s disease, psoriasis, and intertrigo.
that characteristically recurs at the same site following
the administration of the offending drug or occasionally
Plasma cell balanitis (Zoon’s balanitis) a member of the same group of drug.
●● Plasma cell balanitis is an inflammatory disease ●● Genital lesions are commonly caused by tetracyclines.

affecting the glans and prepuce, with chronic-relapsing ●● The lesions may be single or multiple, round or oval, well-

course and benign behavior. marginated, erythematous, edematous patches that resolve
●● It is found at all postpubertal ages, and there seems to with a violaceous or hyperpigmented patches (Figure 19.6a).
be no racial predilection. Bullous lesions may develop occasionally (Figure 19.6b).
 Male genitalia  411

Figure 19.5  (a) Plasma cell balanitis presenting as well-demarcated, moist erythema over glans and inner prepuce.
(b) Another case of plasma cell balanitis.

●● They may be asymptomatic or accompanied by localized ●● Circinate balanitis is usually seen in

burning, stinging, or intense pruritus. association with other findings of reactive
●● DD: Erythema multiforme, genital herpes. arthritis (urethritis, conjunctivitis, and
arthritis). Rarely, it can present as an isolated
Circinate balanitis finding.
●● Circinate balanitis is one of the most common and ●● It starts as small vesicles or pustules in
diagnostically significant mucocutaneous findings in uncircumcised males that rupture, leaving painless
reactive arthritis (Reiter’s disease). superficial erosions that can coalesce to form a

Figure 19.6  (a) Fixed drug eruption presenting with ero-

sion covered by whitish slough. (b) Bullous fixed drug
eruption. (b – Courtesy: Dr. PC Das, Katihar, India.)
412  Male genitalia

Figure 19.7  Circinate balanitis presenting with erosion

with serpiginous margin.

sharply demarcated, characteristic serpiginous lesion

(Figures 19.7 and 19.8a,b).
●● In circumcised males, hyperkeratotic papules and
plaques are noted.
●● DD: Candidiasis, balanoposthitis, and pustular psoriasis.
Seborrheic dermatitis
●● Occasionally, its severe forms can involve the genital

skin. Spread of the eruption to the genitalia is much

more common in infants than in adults. Figure 19.8  (a) Circinate balanitis presenting with ero-
●● In addition, this condition is most severe in those who sion with serpiginous margin. (b) Circinate balanitis
cannot shampoo regularly, such as the homeless and presenting with annular erosion in HLA B27 positive male.
those with neurologic disease or other infirmities that (a,b – Courtesy: Soumyajit Roychoudhury, Consultant
Dermatologist, Berhampore, India.)
interfere with activities of daily living.
●● In the genital area, it is accentuated within the skin folds.

A classic but not unique characteristic of genital seborrheic

dermatitis is a glazed, shiny texture in the affected skin.
●● DD: Atopic dermatitis, allergic contact dermatitis,

irritant contact dermatitis, fungal infection, psoriasis,

and cutaneous candidiasis.
Irritant contact dermatitis
●● Irritant contact dermatitis (ICD) is an eczematous

reaction that develops in response to an exogenous

substance that can cause an inflammatory reaction
whenever applied to the skin of any individual.
●● Mild (and chronic) ICD is usually manifested by

irritation, pain, soreness, rawness, and poorly

demarcated, slightly scaly erythematous patches or
plaques with little or no edema. Often there is a dry,
glazed, fissured, chapped appearance.
●● Severe (and acute) ICD is manifested by a rapid

appearance of erythema, edema, and sometimes,

blistering (Figures 19.9 and 19.10).
●● Poorly keratinized skin, such as the modified mucous Figure 19.9  Mild irritant contact dermatitis presenting as
membranes of the glans penis and the inner aspect of erythema and erosions on the glans penis.
 Male genitalia  413

Figure 19.11  Umbilicated white papules of molluscum

contagiosum at the root of the penis.

nodules, often with overlying erosion that confers an

umbilicated appearance.
Figure 19.10  Severe irritant contact dermatitis with wide- ●● DD: Candidal intertrigo, psoriasis, seborrhea, atopic
spread erosion of penile, scrotal, and surrounding skin. dermatitis, allergic contact dermatitis, acrodermatitis
enteropathica, Langerhans cell histiocytosis, and
the prepuce, become very fragile, and blister roofs are granuloma gluteale infantum.
shed quickly, forming erosions or even ulcers.
●● On the more resilient keratinized skin of shaft of the Molluscum contagiosum
penis, blisters may remain visible for a day or two before ●● Molluscum contagiosum can be sexually transmitted or

they become unroofed, leaving erosions or ulcers. may be acquired non-sexually and affects the genitals of
●● DD: Allergic contact dermatitis, fixed drug eruption. children and adults frequently.
●● The lesions are characteristic, and patients present with
Diaper dermatitis smooth-surfaced, firm, dome-shaped papules with
●● Diaper dermatitis affects warm, moist areas of groin, central umbilication.
including genitalia and gluteals. The skin changes are ●● Their color can vary from pearly white or pink to yellow.

similar to those of ICD. Lesions are usually small, 2–5 mm in diameter, though
●● A severe form of irritant dermatitis is termed diaper occasionally much larger (giant mollusca more than 1.5 cm)
dermatitis of Jacquet, granuloma gluteale infantum, or lesions may be noted, especially in immunocompromised
pseudo wart, characterized by sharply marginated red persons (Figures 19.11 and 19.12a,b).

Figure 19.12  (a) Molluscum contagiosum on the penis and the scrotum. (b) Umbilicated papules of molluscum contagio-
sum on the penis and scrotum in a child. (b – Courtesy: Dr. Dependra Kumar Timshina, Remedy Clinics, Siliguri, India.)
414  Male genitalia

●● The lesions occur in clusters and may be noted in linear ●● DD: Lichen planus, lichen striatus, lichen spinulosus,
arrangement (pseudo koebnerization). bowenoid papulosis, and warts.
●● DD: Lichen nitidus, cutaneous cryptococcosis, and
verruca vulgaris. Lichen planus
●● Lichen planus is a skin disease that exhibits variable
Lichen nitidus morphologies on genital skin.
●● Lichen nitidus may involve genitalia, and the shaft of ●● Erosive lichen planus is a common non-infectious

the penis is characteristically involved. erosive condition occurring on the penis and almost
●● The primary lesions consist of multiple 1- to 3-mm, non-existent in circumcised men.
sharply demarcated, round or polygonal, flat-topped, ●● Itchy red papules are more common than erosions in

skin-colored shiny papules that often appear in groups men, and moist skin often exhibits white skin lesions
(Figure 19.13a,b). (Figure 19.14a–c).
●● They are most often asymptomatic, though some ●● Penile lichen planus are less often eroded, but when

patients report mild pruritus. erosions occur, they are very painful.
●● The condition remits spontaneously after several ●● Men with erosive penile lichen planus experience

years. scarring, with phimosis if uncircumcised, or

obliteration of the sharp distinction of the glans from
the shaft in the circumcised penis.

Figure 19.13  (a) Shiny papules of lichen nitidus on the

shaft of the penis. (b) Lichen nitidus on the glans penis Figure 19.14  (a) Violaceous papules of lichen
of a young boy. (a – Courtesy: Dr. Santoshdev P Rathod, planus. (b) Violaceous papules and plaque of lichen
Ahmedabad, India.) planus. (Continued)
 Male genitalia  415

Figure 19.14 (Continued)  (c) Violaceous papules and

plaque of lichen planus affecting the glans penis.

●● DD: Pemphigus vulgaris, cicatricial pemphigoid, Figure 19.15  (a) Smooth-surfaced pigmented papules
of the bowenoid papulosis. (b) Bowenoid papulosis
toxic epidermal necrolysis, severe erosive
with papules and plaques over penis, scrotum, and
candidiasis, psoriasis, and irritant contact dermatitis. groin. (a – Courtesy: Dr. Sushil S Savant, Mumbai,
India; b – Courtesy: Dr. Santoshdev P Rathod,
Bowenoid papulosis
Ahmedabad, India.)
●● Bowenoid papulosis represents an intraepithelial

neoplasia located on the shaft of penis, occurring mostly

in sexually active males aged 20 to 40 years. Human ●● Classical scabies presents with small, erythematous
papillomavirus (HPV) types 16, 18, 31, and 39 play an
papulovesicular eruptions and excoriated papules on
etiologic role.
the penis and scrotum. The lesions may get secondarily
●● The papules may occasionally become pruritic or
infected (Figure 19.16a–c). The pathognomonic lesions
inflamed, with pain and tenderness.
“burrows” may be found on the penis.
●● It presents with multiple, small, asymptomatic, well-
●● Nodular scabies is sequelae of hypersensitivity reaction
demarcated red, pink, or skin-colored papillomatous
to a mite and its products and presents as extremely
papules and plaques on shaft of penis (Figure 19.15a,b).
pruritic reddish brown nodules (Figure 19.16d).
●● DD: Condyloma acuminata, seborrheic keratosis, lichen
●● DD: Impetigo, insect bite, papular urticaria, and
planus, and Bowen’s disease.
lymphomatoid papulosis.

Scabies Dermatophytosis
●● Scabies is a parasitic infestation causing intense pruritus ●● External genital involvement is being seen with

that is worse at night. increasing frequency in dermatophyte infections.

416  Male genitalia

Figure 19.16  (a) Excoriated papules of scabies on the glans penis. (b) Excoriations with secondary infection in scabies.
(c) Widespread erosions in a child with scabies. (d) Erythematous nodular lesions of nodular scabies.
 Male genitalia  417

●● Its classical presentation is polycyclic annular lesions,

with papules, pustules, and scales at the margin, and
central clearing (Figure 19.17a,b).
●● Atypical manifestations include no central clearing,
presenting with only fine powdery scales, and tinea
pseudoimbricata (concentric rings) and is commonly seen in
people who have applied topical corticosteroids for a while.
●● DD: Nummular dermatitis, annular psoriasis, impetigo,
pityriasis rosea and allergic contact dermatitis.

Allergic contact dermatitis

●● Allergic contact dermatitis is a delayed type IV

sensitivity to specific sensitizing agents or allergens.

Only certain individuals exposed to a potential allergen
develop allergic contact dermatitis at the time of their
next contact with the allergen.
●● It is characterized by the presence of pruritic, poorly

marginated, bright-red, edematous patches and papules

(Figure 19.18). Sometimes minute vesicles stud the
surface of the erythematous patch. Oozing and scaling
are other variable findings.
●● Pruritus might result in the development of the itch–

scratch cycle and the clinical signs of lichen simplex

chronicus, especially seen on the scrotum.
●● Both the penis and the scrotum may be affected.

●● DD: Candidiasis, and dermatophytosis.

Hemangioma
●● Hemangioma is a true neoplasm of endothelial cells that

line the blood vessels and is the most common benign

soft-tissue tumor of infancy and childhood, occurring
in greater frequency in whites, premature infants, twins,
and children born to mothers of high maternal age.
●● Hemangiomas involving genitalia comprise a rare clinical

entity and have been mostly reported in the pediatric age

group and much more rarely reported in adults.
●● On genitals, these tumors can involve glans,

penile shaft, or scrotum. It usually manifests as an

Figure 19.17  (a) Multiple annular lesions of dermato-

phyte infection on the shaft of penis. Tiny pustules at
the margin may be identified. Note presence of tinea Figure 19.18  Allergic contact dermatitis presenting with
cruris. (b) Annular lesion with papules and scales at multiple erythematous papules and scaling involving the
the margin. glans and shaft of the penis.
418  Male genitalia

Figure 19.19  Hemangioma affecting the glans penis. Figure 19.20  Erythematous papular lesions of angiokera-
(Courtesy: Dr. Santoshdev P Rathod, Ahmedabad, India.) toma in a case with angiokeratoma corporis diffusum.

Sebaceous hyperplasia
asymptomatic pale patch, then grows rapidly and finally ●● Sebaceous hyperplasia is a benign hamartomatous
appears as a port-wine-stain–like lesion. condition of epidermal appendage tumors with
●● They are well-known for rapid growth during the sebaceous differentiation affecting mainly the adults of
first weeks to months of a child’s life followed by middle age or older.
a spontaneous but slow involution. As the lesion ●● Occasionally it occurs on scrotum, foreskin, or the shaft
regresses, the color of the lesion fades from a bright of penis.
red to a dull red and then a grey-white hue develops at ●● It consists of single or multiple, asymptomatic, small,
the center of the lesion and spreads to the periphery yellow papules, sometimes with a central depression
(Figure 19.19). At this stage, the tumor becomes soft in (Figure 19.21).
texture followed by a reduction in the volume.
●● Lesions may be single or multiple and are usually painless
but can be associated with pain, ulceration and bleeding.
●● DD: Angiokeratoma, verruca.

Angiokeratoma
●● Angiokeratomas are benign proliferations of dilated

thin-walled blood vessels in the upper dermis with

overlying epidermal hyperkeratosis.
●● The clinical variants of angiokeratoma can be divided into

a widespread form that includes angiokeratoma corporis

diffusum (Fabry disease), associated with deficiency of
Galactosidase A, and localized forms that include solitary
angiokeratoma, angiokeratoma circumscriptum neviforme,
angiokeratoma of Mibelli, and angiokeratoma of Fordyce.
●● The individual lesion presents as an asymptomatic, 2- to

5-mm, blue-to-red papule or plaque with scaly surface.

Early lesions are soft, red, and easy compressible
(Figure 19.20); later, they become blue, keratotic, and
non-compressible.
●● They may bleed spontaneously, after scratching, on

trauma, and following sexual intercourse.

●● Angiokeratoma of Fordyce is the most common type of

angiokeratoma, seen over the scrotum.

●● Malignant transformation has not been seen.

●● DD: Condylomata acuminata, cherry hemangioma,

Figure 19.21  Multiple yellowish papules of sebaceous
granuloma pyogenicum, and verrucous hemangioma. hyperplasia.
 Male genitalia  419

●● DD: Condyloma acuminate, furuncle, millium, lichen transmitted diseases and of interference with sexual
nitidus, and pearly penile papules. activity.
●● The lesions present as pink or white, smooth, dome-
Pearly penile papule shaped or filiform papules, orienting around the corona
●● Pearly penile papules are small, benign lesions that of the glans penis usually in one or more rows.
appear typically in parallel rows on the corona of the ●● They are most prominent along the dorsal surface
glans penis in late adolescence or early adulthood. of the corona and may encircle the glans entirely.
●● Older males and circumcised males have lower Rarely, lesions may be found on the shaft of penis
prevalence. (Figure 19.22a–c).
●● The lesions are asymptomatic, but patients seek ●● DD: Warts (Figure 19.22d), condyloma acuminata,
consultation because of concerns of sexually Tyson’s glands, and molluscum contagiosum.

Figure 19.22  (a) Flesh-colored papules arranged in paral-

lel rows on the corona of the glans penis (close-up image).
(b) Pearly penile papules on the ventral aspect (close-up
image). (c) Pearly penile papules on the ventral aspect of
the penis. (d) Warts arranged circumferentially around
the shaft of the penis (1) and pearly penile papules (2) in
a young male. (a,b – Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India; c – Courtesy: Dr. PC Das, Katihar, India.)
420  Male genitalia

Skin tag ●● While in uncircumcised males, the non-scaly

●● Genital skin tags are sessile or pedunculated fleshy plaques are most common under the prepuce and
growths may have several different shapes and colors, on the proximal glans, in circumcised ones, they are
including wrinkled or smooth textures, and dark or usually present on the glans and corona and may be
light hues. more scaly than those usually seen in genital skin
●● Lesions in men are typically around the scrotum, penile (Figure 19.23a–d).
shaft, or penis head. ●● Fissures, pruritus, and/or burning in the affected
●● Tags are benign and hidden away most of the time. area, ranging from minimal to marked, may be
●● DD: Anogenital wart. observed.
●● DD: Balanitis, lichen planus, candidiasis, and
Psoriasis extramammary Paget’s disease (Figure 19.23e).
●● The genital skin is involved in up to 40% of psoriasis

patients. Erythroplasia of Queyrat

●● In many cases, genital psoriasis is part of more ●● Erythroplasia of Queyrat is a premalignant

generalized plaque psoriasis or occurs in the setting of condition of the visible mucous membranes of
inverse psoriasis. penis and is usually seen in middle-aged and older
●● Lesions on the genital area are brightly erythematous, uncircumcised men. Etiology is not well known, but
often less well-demarcated and devoid of the typical chronic irritative triggers are believed to play an
psoriatic scales, due to maceration. important role.

Figure 19.23  (a) Erythematous, scaly plaque of psoriasis on the glans penis. (b) Flexural psoriasis in a child. (c) Dry, scaly
erythematous patch of psoriasis. (Continued)
 Male genitalia  421

Figure 19.23 (Continued)  (d) Psoriasis of the glans penis.

(e) Extramammary Paget’s disease presenting as ery-
thematous atrophic patch in the genitocrural region.
(b – Courtesy: Dr. Santoshdev P Rathod, Ahmedabad, Figure 19.24  (a) Erythroplasia of Queyrat presenting as
India; e – Courtesy: Dr. Sushil S Savant, Mumbai, India.) well-defined erythema. (b) Erythroplasia of Queyrat pro-
gressing to ulcer. (a – Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India; b –Courtesy: Dr. Santoshdev P Rathod,
●● It presents as asymptomatic, single erythematous Ahmedabad, India.)
patch, with a clear-cut border and lucent surface on
glans penis or inner foreskin, which shows slow growth Genitourinary tuberculosis
(Figure 19.24a,b). ●● Genitourinary tuberculosis is the second most common

●● DD: Allergic/irritant contact dermatitis, balanitis extra-pulmonary tuberculosis. An isolated involvement

xerotica obliterans, balanoposthitis, basal cell of genital organs has been documented in up to 30% of
carcinoma, cicatricial pemphigoid, drug-induced the cases.
bullous disorder, lichen planus, mucosal candidiasis, ●● Penile tuberculosis is usually primary and may be

and plaque psoriasis. acquired sexually from infected partner.

422  Male genitalia

●● Involvement of the testis results from direct extension

from the epididymis. Testicular tuberculosis may
present with painless or painful scrotal mass, abscess,
fistula, and multiple pus-discharging sinuses on the
posterior surface of the scrotum.
●● Penile tuberculosis usually presents with single or
multiple superficial ulcers with or without inguinal
lymphadenopathy. Nodules or fungating mass are rarer
presentations.
●● Papulonecrotic tuberculid is another form of penile
affection, which presents as asymptomatic, symmetric,
dusky-red papules and pustules over the glans penis.
The lesions occur in crops and heal with atrophic
scarring (Figures 19.25 and 19.26).
●● DD: Syphilitic ulcer, carcinoma, granuloma inguinale,
and human immunodeficiency virus (HIV) infection.

Leprosy (Hansen’s disease)

●● Leprosy may be missed in external genitals unless

specifically looked for. Figure 19.26  Papulonecrotic tuberculid lesions healing

●● Genital lesions are mostly observed in multibacillary with atrophic scars.
cases having extra-genital lesions too. Isolated genital
involvement is very rare in literature. ●● Lesions of erythema nodosum leprosum have been
●● Clinically, patches, plaques, and/or nodules may be reported in genital region too.
observed, depending on the spectrum of leprosy ●● DD: Psoriasis, sarcoidosis, and syphilis.
(Figure 19.27).
●● There are few cases of histoid leprosy in which nodular

lesions developed over the penile shaft.

Figure 19.25  Erythematous papule of papulonecrotic Figure 19.27  Erythematous nodules and plaques on the
tuberculid. penis in a case of lepromatous leprosy.
 Male genitalia  423

Lymphoma ●● Patients also present with non-healing ulcers, and male

●● B-cell non-Hodgkin lymphoma affects middle-aged and patients, rarely, present priapism.
older individuals and is the most common subtype of ●● DD: Squamous cell carcinoma.
lymphoma of the genitalia.
●● Other lymphoma types that may involve the genitalia
Lichen sclerosus et atrophicus (hypoplastic
include extranodal marginal-zone lymphoma, dystrophy)
follicular lymphoma, and anaplastic large cell
●● Lichen sclerosus et atrophicus occurs at all ages.
lymphoma.
●● Major symptoms are pruritus and eventual pain
●● The lymphoma can result in the formation of
with more advanced disease. Glans involvement may
a nodular mass and presents with an itching
result in narrower urine stream during micturition
sensation.
●● In some cases, pelvic pain and other general signs and
(Figure 19.28a).
●● It is most often characterized by well-demarcated,
symptoms, such as anemia, fatigue, and appetite loss,
hypopigmented plaques of fragile skin.
are noted.
●● Usually, at least some areas of the skin show fine

crinkling, and fragility is often manifested by purpura

or erosions (Figure 19.28b).
●● Uncircumcised males generally experience

phimosis as a presenting abnormality

(Figure 19.28c).
●● DD: Vitiligo, eczema, mucous membrane pemphigoid,

and erosive lichen planus.

Porokeratosis
●● Porokeratosis is clinically characterized by annular

plaques having central atrophy and raised keratotic

border with groove.
●● It usually presents as asymptomatic or, rarely, pruritic,

solitary or multiple annular skin lesions of a few

millimeters to centimeters in size.

Figure 19.28  (a) Lichen sclerosus affecting the skin surrounding urinary meatus. (b) Lichen sclerosus causing induration of
the prepuce resulting in inability to retract phimosis, fissures, and erosions. (c) Lichen sclerosus causing phimosis in a child.
(c – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India.)
424  Male genitalia

●● Most commonly, it affects the scrotum and penis Pseudoepitheliomatous, keratotic, and micaceous
(Figure 19.29a,b). balanitis
●● Porokeratosis ptychotropica is a rare clinical variant ●● Pseudoepitheliomatous, keratotic, and micaceous
commonly seen in the genitogluteal region and on balanitis is a rare condition affecting the glans penis
the buttocks. Clinically, it presents as itchy, solitary or and is usually seen in older, uncircumcised men.
multiple, coalescing verrucous or scaly plaques with ●● It presents as solitary, well-demarcated, hyperkeratotic
satellite lesions. plaque over the glans penis, with an overlying thick
●● DD: Annular lichen planus. adherent micaceous scale.
●● The condition is generally asymptomatic but fissuring,

maceration, irritation, and interference with sexual

activity may be associated (Figure 19.30a,b).
●● It needs close monitoring as progression to verrucous

carcinoma and squamous cell carcinoma is known.

●● DD: Penile horn, penile psoriasis, giant condyloma,

verrucous carcinoma, squamous cell carcinoma, and

keratoacanthoma.

Figure 19.30  (a) Well-demarcated, hyperkeratotic plaque

Figure 19.29  (a) Porokeratosis (Mibelli type) presenting of pseudoepitheliomatous, keratotic, and micaceous
with annular plaque with keratotic margin. (b) Porokeratosis balanitis. (b) Pseudoepitheliomatous, keratotic, and
(Mibelli type) presenting as dry erythematous plaque on micaceous balanitis with thick keratinous overgrowth on
the glans penis. (b – Courtesy: Dr. Santoshdev P Rathod, the glans penis. (b – Courtesy: Dr. Santoshdev P Rathod,
Ahmedabad, India.) Ahmedabad, India.)
 Male genitalia  425

Figure 19.31  Giant cauliflower-like plaques and nodules

with verrucous surface in Buschke-Lowenstein tumor.
Figure 19.32  Verrucous carcinoma on the glans penis.
(Courtesy: Dr. PC Das, Katihar, India.)
Buschke-Lowenstein tumor
●● Buschke-Lowenstein tumor is a localized variant

of verrucous carcinoma typically associated with The term “invasive” refers to tumors in which the
HPV types 6 and 11. The tumor is slow-growing and malignant cells have penetrated the underlying basement
highly destructive to contiguous tissues but seldom membrane and have infiltrated into the stroma.
metastasizes. ●● The risk factors include phimosis, chronic
●● It presents as an asymptomatic polypoid or cauliflower- balanoposthitis, chronic irritation, lichen sclerosus et
like growth on the genitals and surrounding areas that atrophicus, and lichen planus.
grows to a large size (Figure 19.31). Maceration and ●● It usually presents as an asymptomatic or itchy,
secondary infections are common. painful, indurated papule or nodule that may ulcerate
●● DD: Squamous cell carcinoma, genital warts, and (Figure 19.33a,b).
primary rectal adenocarcinoma. ●● Metastasis to regional lymph nodes has been reported
in 60% cases of penile SCC.
Verrucous carcinoma ●● DD: Basal cell carcinoma, Kaposi sarcoma,
●● Verrucous carcinoma is a relatively uncommon locally donovanosis, and pyoderma gangrenosum.
aggressive, low grade, slow-growing well-differentiated
squamous cell carcinoma with minimal metastatic Epidermoid cyst
potential. ●● Penile epidermoid cysts are uncommon.

●● It presents with a polypoid or exophytic nodule with a ●● They appear as solitary or, uncommonly, multiple,

verrucous surface. Long-standing cases may progress to round, usually small, mobile bumps. They occur in
an ulcer. various sizes.
●● It may progress locally to cause perianal infection, ●● They are usually white or yellow, though people with

fistula formation, and local lymphadenopathy. darker skin may have pigmented cysts.
●● Symptoms include pruritus, bleeding, pain, tenesmus, ●● Lesions may be asymptomatic, but rupture may result in

fecal incontinence, discharge, change in bowel habits, significant discomfort.

etc. (Figure 19.32). ●● DD: Cutaneous lipoma, steatocystoma multiplex.

●● DD: Genital warts.

Steatocystoma multiplex
Squamous cell carcinoma ●● Steatocystoma multiplex is an uncommon but distinct

●● Squamous cell carcinoma (SCC) is the commonest clinical condition characterized by the formation of
neoplasm of the genital tract and penis and may be numerous cutaneous sebum-containing cysts called
invasive cancer and noninvasive or preinvasive lesions. steatomas, steatocystomas, or sebocysts.
426  Male genitalia

Figure 19.34  Multiple tense cystic lesions on the scrotum.

●● The lesions are usually asymptomatic, but occasionally

they can become infected and painful (Figure 19.34).
●● DD: Epidermoid cyst, lipoma, calcinosis cutis.

Scrotal calcinosis
●● Idiopathic scrotal calcinosis is a benign condition,

mainly seen in men aged 20 to 40 years.

●● The pathogenesis of SC is controversial as to whether the

disease is idiopathic or the result of dystrophic calcification

of existing structures, including epidermal cyst, eccrine
epithelial cyst, and degenerated dartos muscle.
●● Clinically, it consists of hard, yellowish nodules within

the dermis of scrotal skin.

Figure 19.33  (a) Squamous cell carcinoma presenting with ●● Nodules vary in size (from 1 mm to several centimeters)
exophytic nodule on the glans penis. (b) Squamous cell
and number (solitary or multiple).
carcinoma presenting as noduloulcerative lesions on the
●● The nodules are usually asymptomatic, and patients
glans penis. (a – Courtesy: Dr. PC Das, Katihar, India.)
seek medical advice mainly for cosmetic reasons.
●● A few cases may have itching or discharge from the
●● In familial cases, it is an autosomal dominant calcified masses (Figure 19.35).
genodermatosis usually appearing at birth or early in ●● DD: Scrotal cyst.
life.
●● The cysts may be seen on the penis and scrotum. The Penile thrombophlebitis
cysts are always multiple and vary from 1 to 20 mm in ●● Superficial thrombophlebitis of the dorsal vein of

diameter. the penis (penile Mondor’s disease) is a rare disease

 Male genitalia  427

●● The cyst commonly presents as a small, soft, and freely

movable mass around the glans, mainly on the penile
ventral surface.
●● Mostly they are asymptomatic but can be complicated
by infection or trauma or can make coitus difficult.
●● DD: Epidermal cyst, median raphe cyst, lipomas,
steatocystoma, dermoid cyst, pilonidal cyst, Tyson’s
glands cyst.

Lymphangioma circumscriptum
●● Lymphangioma circumscriptum is a benign

proliferation of lymphatics and is broadly classified into

two major groups based on the depth of involvement
and size of the abnormal lymph vessels.
●● It can become evident at any age, but the greatest

Figure 19.35  Firm nodules of scrotal calcinosis. (Courtesy: incidence occurs at birth or early in life.
Dr. Deverashetti Srinivas, Nizamabad, India.) ●● Genitals, including scrotum and penis, may be affected.

Clinically, it presents as multiple discrete or grouped

vesicles that may be either translucent or red to blue (or
presenting with pain and induration of the dorsal part black). A verrucous surface may be seen.
of the penis. ●● Usually the condition is asymptomatic, but sometimes it

●● The possible causes comprise trauma, neoplasms, is associated with oozing of the lymphatic fluid from the
excessive sexual activity, or abstinence. This disease vesicle and with pain and pruritus.
almost always limits itself. ●● Complications include hemorrhage, ulceration,

●● The patient consistently presents with a rope-like cord secondary infection, and, rarely, malignant
on the dorsum of the penis. The cord is a thrombosed transformation such as lymphangiosarcoma and
dorsal vein that has become thickened and adherent to squamous cell carcinoma.
the overlying skin. ●● DD: Angiokeratoma, condylomataaccuminata,

●● The patients have a significant amount of pain, which molluscum contagiosum, bacillary angiomatosis and nevi.
can be either episodic or constant.
●● Some patients may develop irritative voiding Median raphe cyst
symptoms. ●● Median raphe cysts are considered to be a

●● DD: Peyronie’s disease, sclerosing lymphangitis. developmental anomaly resulting from abnormal or
incomplete development of paired genital folds and can
Peyronie’s disease present along the midline of the ventral side of the male
●● Peyronie’s disease is a superficial penile fibromatosis genital area, anywhere from tip of penis to anal orifice.
resulting from the growth of fibrous plaques in the Rarely, they may present as cordlike or canaliform
penile soft tissues. Specifically, scar tissue forms induration on the median raphe (Figure 19.36a,b).
in the tunica albuginea surrounding the corpora ●● They may present at birth or may remain asymptomatic

cavernosa. and unrecognized till early adulthood.

●● It is characterized by palpable nodules in the region of ●● The cyst grows in size with advancing age and may

the tunica albuginea enclosing the corpora cavernosa become symptomatic owing to infection and trauma.
and the penile septum, which may be associated ●● DD: Dermoid cyst, pilonidal cyst, epidermal inclusion

with pain and deformity on erection and a variable cyst, urethral diverticulum, steatocystoma.
degree of erectile dysfunction and decreased girth.
DD: Congenital penile curvature, penile dorsal vein Erythema multiforme
thrombosis, thrombophlebitis, systemic sclerosis, ●● Erythema multiforme is an acute, immune-mediated,

bubonulus, penile fracture. mucocutaneous condition commonly caused by herpes

simplex virus infection and certain medications,
Mucoid penile cyst characterized by classical iris or target lesions.
●● Mucoid penile cysts are uncommon benign lesions ●● Earliest lesions are usually bilaterally symmetrical, round,

affecting mainly young men and manifesting on the erythematous, edematous papules surrounded by areas of
ventral surface of glans penis. They most likely arise blanching that may resemble insect bite or papular urticaria.
from ectopic urethral mucosa sequestrated in the penile ●● These papules may enlarge and develop concentric alteration

skin during embryologic development. in morphologic features and color, resulting in target lesions.
●● Most of them are present at birth, but usually they are ●● Typical target lesions have a central dusky erythema,

detectable only in adolescence. purpura, or blister; a middle ring of pale edema; and
428  Male genitalia

symptoms (fever, malaise) and local symptoms (pain,

tenderness, burning and tingling) may precede
the development of genital lesions. Genital lesions
typically occur on the glans or shaft of penis and
start with painful, grouped vesicles appearing on
an erythematous base, which progress to pustules,
erosions, and/or ulcerations. Inguinal lymphadenopathy
may be associated. Crusting of lesions followed by
resolution of lesions occurs over the next couple of days
(Figure 19.37a,b).

Figure 19.36  (a) Median raphe cysts presenting as tense

cystic swellings on the glans penis. (b) Median raphe cysts
presenting as midline cord like swelling in the groin.

an outer well-defined ring of erythematous halo. In

atypical lesions, these areas manifest as round, palpable,
edematous lesions with only two zones or a poorly
defined border or both.
●● DD: Pemphigus vulgaris, bullous pemphigoid, and
paraneoplastic pemphigus.

Herpes genitalis
●● Genital herpes is a viral infection caused by a DNA

virus, Herpes simplex virus (HSV) type 2. Incubation

period is 5 to 14 days.
●● In primary infection, symptoms typically occur within

3 to 7 days after exposure but with a longer duration Figure 19.37  (a) Genital herpes presenting as grouped ves-
of 10 to 14 days. Before the onset of lesions, systemic icles and erosions. (b) Grouped erosions in genital herpes.
 Male genitalia  429

●● Genital herpes usually recurs. During reactivation, a Candidiasis

similar prodrome of fever and flu-like symptoms with ●● Candida species are normal flora and may cause
localized burning, itching, and tingling may occur, but symptomatic infection. Risk factors of candidiasis
these are often seen with decreased severity. The genital include older age, diabetes, prolonged antibiotic therapy,
lesions are usually fewer, and duration of disease is less and immunosuppression.
compared to those of primary infection. ●● Patients present with mild glazed erythema,
●● DD: Trauma, syphilitic chancre (non-purulent, usually satellite pustules and erosions, and moist curd-like
single, indurated, painless ulcers lymphadenopathy), accumulations, and sometimes ulceration of the glans
chancroid ulcers (purulent, often multiple painful penis and fissured prepuce are seen. Peripheral pustular
ulcers with soft, undermined edges), lymphogranuloma and papular lesions may be noted. Mild burning pain
venereum (transient papular, herpetiform vesicle, and pruritus are often associated (Figure 19.38a–e).
erosion, or ulcer). ●● DD: Pustular psoriasis, impetigo, dermatophytosis.

Figure 19.38  (a) Candidiasis presenting as whitish slough present on the glans and inner prepuce. (b) Candidiasis present-
ing as erosions and whitish membrane like structure. (c) Candidiasis presenting with phimosis, fissures, and accumulation
of whitish slough. (d) Numerous superficial pustules in candidiasis. (Continued)
430  Male genitalia

Figure 19.39  Erosions on the penis in a case of Stevens-

Johnson syndrome.

Stevens-Johnson syndrome and toxic epidermal

necrolysis
●● Stevens-Johnson syndrome (SJS) and toxic epidermal
Figure 19.38 (Continued)  (e) Whitish, easily scrapable
slough on the glans penis in candidiasis. (d – Courtesy: necrolysis (TEN) are severe life-threatening conditions
Dr. Santoshdev P Rathod, Ahmedabad, India.) after exposure to drugs (95% of cases) or infections.
●● The most common drugs implicated are sulfonamides,

Pemphigus vulgaris and vegetans penicillin, quinolones, cephalosporin, acetaminophen,

carbamazepine, phenobarbital, phenytoin, valproic
●● Penile involvement is usually seen along with
acid, oxicam group of non-steroidal anti-inflammatory
mucocutaneous disease, but it may occur as early
drugs, antitubercular, allopurinol, and antiretrovirals.
manifestation of the disease too, followed by ●● SJS and TEN manifest as erythematous or violaceous
development of cutaneous lesions in due course.
patches, atypical targetoid lesions, bullae, erosions,
●● Pemphigus vegetans is uncommonly seen and presents
and ulcers involving the skin and mucosa, including
as warty lesions in intertriginous areas, including genital
genitalia (Figure 19.39).
region. It has two clinical subtypes: the Neumann type, ●● DD: Staphylococcal scalded skin syndrome, drug-
with poor prognosis, usually begins with bullae; the
induced pemphigoid and pemphigus, acute graft versus
Hallopeau type, with excellent prognosis and longer
host disease, and paraneoplastic pemphigus.
remission, usually begins with grouped pustules.
●● DD: Bullous pemphigoid, Hailey-Hailey disease, Acrodermatitis enteropathica
cicatricial pemphigoid, fixed drug eruption, herpes ●● Acrodermatitis enteropathica is caused by zinc
genitalis, genital aphthosis, and Behçet’s disease for deficiency and may occur as a rare autosomal recessive
pemphigus vulgaris, (genital wart, herpes genitalis, and disease, in nutritional deficiency states or, less
condylomata lata for pemphigus vegetans). commonly, iatrogenically post-parenteral nutrition.
●● The classic presentation is a triad of periorificial and
Bullous pemphigoid acral dermatitis, diarrhea, and alopecia. The dermatitis
●● Localized variants with involvement limited to genital
consists of erythematous, well-demarcated, and scaling
region, including localized penile and scrotal bullous plaques or, sometimes, vesicopustular plaques and
pemphigoid, have been reported. crusting, which is most pronounced around the mouth,
●● DD: Pemphigus vulgaris, erythema multiforme, mucous
eyes, and genital area (Figure 19.40).
membrane pemphigoid. ●● Secondary infections with staphylococcus and candida

are common.
Cicatricial pemphigoid (mucous membrane ●● DD: Diaper dermatitis, flexural psoriasis, other
pemphigoid)
nutritional deficiency dermatosis.
●● Genital regions are involved in 50% of patients.

●● In the genital region, it may present as non-specific erosions, Chancre

skin fragility, and sometimes intact blisters over clinically ●● Chancre seen in primary syphilis is a sexually

hair-bearing skin. Scarring may result in phimosis. transmitted infection caused by a spirochaete bacterium,
●● DD: Erosive lichen planus, bullous pemphigoid, lichen Treponema pallidum, subspecies pallidum. Incubation
sclerosus et atrophicus. period is 9 to 90 days; the average is three weeks.
 Male genitalia  431

●● The primary syphilis presents as a single, indolent,

round or oval, indurated painless ulcer or chancre with
regional lymph nodes enlargement.
●● The lesion starts as a small red papule or crusted
superficial erosion that becomes a round or oval,
indurated, typically well-circumscribed ulceration and
which is firm on palpation (Figure 19.41a–c).
●● A hard chancre present on the inner surface of the
prepuce may snap back on a retracted prepuce (Dory
flop sign).
●● Common sites are the glans penis, coronal sulcus,
prepuce, frenulum, and shaft of penis.
●● DD: Herpes genitalis (recurrent painful vesicles, erosions
or ulcers), Fixed drug eruption, Behçet’s disease.

Aphthous ulcer (aphtha, aphthous stomatitis, or

canker sore)
●● Aphthous ulcer is encountered infrequently on the

genitalia.
●● Genital lesions that are seen in men are secondary and

usually occur in the setting of Behçet’s disease. Most,

but not all patients, with genital aphthous ulcers also
have a history of oral aphthae.
●● Aphthous ulcers of the genitalia tend to be larger and

deeper than those occurring in the mouth.

Figure 19.40  Acrodermatitis enteropathica with perianal, ●● The base of the ulcers can be bright red or may be
scrotal, and ventral penile involvement.
covered with either gray, necrotic material or heme-
colored eschar (Figure 19.41d).

Figure 19.41  (a) Chancre on inner prepuce presenting as solitary well-demarcated, ulcer with clean floor and indurated
base. (b) Chancre on the inner prepuce. (Continued)
432  Male genitalia

Figure 19.41 (Continued)  (c) Multiple chancres. (d) Large painful aphthous ulcer in a patient with Behcet’s disease.
(c – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India; d – Courtesy: Dr Rajeev Ranjan, Ara, India.)

●● Genital ulcers are extremely painful, and their ●● Common sites involved are external or internal surface of
appearance is sometimes preceded by, or accompanied prepuce, the frenulum, the coronal sulcus, and occasionally
by, low-grade fever, malaise, gastrointestinal, and/or the shaft of penis and external urinary meatus.
respiratory symptoms.
●● Larger genital lesions may heal with scarring.
●● It tends to occur more often on the scrotum than on the
penis.
●● DD: Behçet’s disease, primary syphilis, chancroid.

Chancroid (soft chancre, ulcus molle)

●● It is a sexually acquired infection caused by a

gram-negative bacillus, Haemophilus ducreyi.

Incubation period is three to ten days (average
seven days).
●● Chancroid presents as multiple painful ulcers on

genitalia.
●● A lesion starts with a small inflammatory papule

surrounded by erythema, rapidly progresses to a

pustule, and finally develops into a foul-smelling
painful ulcer.
●● Ulcers are sharply circumscribed, with a purulent

base, soft, undermined edges, and purulent exudates

present on the floor of the ulcer; removal of the exudate
may bleed on scrapping or gentle manipulation Figure 19.42  (a) Chancroid – solitary large ulcer with
(Figure 19.42a,b). hemorrhagic floor. Smaller shallow ulcer is present in the
●● The base of the ulcer is non-indurated. vicinity. (Continued)
 Male genitalia  433

●● The disease begins as single or multiple firm papule or

nodule that ulcerate eventually.
●● The ulcer is usually painless or mildly painful and
slowly progresses over weeks to months; it is beefy-
red in color and bleeds easily on touch. Foul-smelling
exudate due to extensive tissue damage is present.
●● The most common sites include the glans penis,
prepuce, frenulum, and coronal sulcus.
●● Complications are phimosis or lymphoedema of distal
tissue in active phase of the disease.
●● Recurrence in healed areas is common.
●● Morphological variants are classical granulomatous,
hypertrophic, sclerotic or cicatricial, destructive
necrotic (phagedenic).
●● DD: Syphilis (non-purulent, usually single,
indurated, painless ulcer, lymphadenopathy),
chancroid (purulent, often multiple painful ulcers,
soft, undermined edges, painful suppurative
lymphadenopathy), herpes genitalis (recurrent painful
vesicles, erosions or ulcers).

Crohn’s disease
●● Crohn’s disease is an inflammatory granulomatous

disease that can affect sites distant from the

gastrointestinal tract (non-contiguous form or
metastatic Crohn’s disease) genitalia.
●● Genital involvement can present in several different

forms. Interestingly, the severity of the cutaneous

findings may not correlate with the severity of the bowel
symptoms.
●● It can occur as a result of direct extension from areas

near the bowel, such as the anus or a colostomy site, or it

can appear on the genitalia as a solitary lesion.
●● Genital lesions usually appear as non-healing ulcers, but
Figure 19.42 (Continued)  (b) Solitary ulcer of chancroid on
they can present as a papule, plaque, or swelling.
the shaft of the penis. (c) Multiple dwarf type of chancroid
●● Penile and scrotal edema may occur.
ulcers.
●● Linear fissures can develop within intertriginous folds.

This sign, termed “knife cut,” is a very distinctive

●● If untreated, inguinal buboes may rupture to form finding.
inguinal ulcers. ●● DD: Hidradenitis suppurativa, Langerhans cell

●● Usually, unilateral painful inguinal lymphadenitis may histiocytosis, herpes genitalis in an immunosuppressed
develop along with the genital ulcer. patient, syphilis, granuloma inguinale, chancroid,
●● Clinical variants include giant, large serpiginous traumatic ulceration.
ulcer, phagedenic, transient, follicular, papular, dwarf
(Figure 19.42c), pseudo granuloma inguinale. Pyoderma gangrenosum
●● Complications include phimosis, paraphimosis, ●● It usually starts with one or more inf lammatory

urethral fistula, and phagedenic ulcerations. pustules, papules, or nodules that break down
●● DD: Genital herpes (recurrent painful vesicles, erosions, to form an ulcer. Most cases of genital localized
or ulcers), primary chancre (non-purulent, usually pyoderma gangrenosum are not complicated with
single, indurated, painless ulcers), chancroid (purulent, associated systemic diseases in both male and female
often multiple painful ulcers, soft, undermined edges). cases. But, genital cases with extragenital skin
lesions tend to have associated systemic diseases
Donovanosis (Figure 19.43a,b).
●● Donovanosis is a rare chronic, progressive ulcerative ●● Most commonly associated diseases are inflammatory

bacterial infection caused by a gram-negative bacilli, bowel disease (especially Crohn’s disease), rheumatoid
Klebsiella granulomatis. The incubation period is one arthritis, lupus erythematosus, hematopoietic
day to one year (average 17 days). malignancy, and various gammopathies.
434  Male genitalia

Figure 19.44  Ulceration with granulation tissue affecting

the shaft of the penis in circumferential manner in derma-
Figure 19.43  (a) Pyoderma gangrenosum of the penis pre- titis artefacta.
senting as crusted ulcers. (b) Rapid progression in the size
of ulcers in one month. (a,b – Courtesy: Dr. Santoshdev P
Rathod, Ahmedabad, India.) Malakoplakia
●● Malakoplakia is an acquired granulomatous disorder,

●● DD: Syphilis, herpes simplex, mycobacterial infection, which mainly occurs in the genitourinary tract,
amebiasis, squamous cell carcinoma, cutaneous Crohn’s accounting for 60% to 70% of the cases.
●● Its appearance is associated with organ transplantation,
disease, ulcerating sarcoidosis, aphthous ulceration,
Behçet’s disease, Fournier gangrene. connective-tissue disorders, neoplasm, diabetes
mellitus, and chronic debilitating/immunodeficiency
Dermatitis artefacta disorders such as HIV infection, hepatitis C,
●● Dermatitis artefacta is a factitious disorder in which the sarcoidosis, and chronic immunodeficiency.
patient creates skin lesions in order to satisfy an internal ●● The lesions may present as ulcerations, abscesses,

psychological need. erythematous papules, subcutaneous nodules, or masses

●● It has been associated with obsessive-compulsive and are associated with non-healing surgical wounds
spectrum disorder, depression, psychosis, and severe and draining fistulas.
personality disorders. ●● The lesions may present over the perianal area and

●● Its clinical expression is variable in morphology as well scrotum.

as distribution. ●● DD: Tuberculosis, Whipple disease, lepromatous

●● Inconclusive, bizarre, irregular rectilinear outlines and leprosy, fungal infection (cryptococcus), parasite
geographical patterning are cardinal (Figure 19.44). infection (leishmaniasis), Langerhans cell histiocytosis,
●● Simultaneous occurrence of artefactual genital ulcers in fibrous histiocytoma, lymphoma, granular cell tumor,
a couple, “folie à deux,” has been described. xanthoma, foreign body granuloma, hemophagocytic
●● DD: Neurotic excoriations, traumatic ulcer. syndromes, sarcoidosis.
 Male genitalia  435

Actinomycosis
●● Primary genital involvement is rare and follows trauma

or human bite.
●● The condition is characterized by the clinical triad of

painless subcutaneous nodules, sinuses, and discharge.

●● Penile lesions are mostly situated in the region around the

corona and are commonly associated with pilonidal sinus.

●● DD: Crohn’s disease, abscess, and tuberculosis.

Lymphedema
●● Genital lymphedema is an edematous disease of the

genitalia due to abnormal lymph circulation.

●● Congenital forms are associated with Turner, Noonan,

Klinefelter, yellow nail, and intestinal lymphangiectasia

syndromes.
●● Swelling due to lymphedema in the early phases is painless.

●● The tissue is soft and may pit when depressed.

●● As the condition becomes chronic, the skin and

subcutaneous tissue may have a woody, indurated

texture as the tissue becomes fibrotic. The penis may
assume a distorted shape. (Figure 19.45a,b).
●● Patients experience a sensation of heaviness and/or

tension, swelling, urinary troubles, and/or lymphorrhea

of the lesion.
●● The skin may exhibit warty excrescences or blisters, and

it may weep lymphatic fluid (Figure 19.45c).

●● If the cause is infection, subcutaneous abscesses and

sinus formation may be apparent.

●● DD: Venous thrombosis or insufficiency, Fournier

gangrene, lymphangioma circumscriptum, angioedema.

Priapism
●● Priapism is a persistent and often painful erection

that lasts for several hours, even in the absence of

stimulation or after stimulation has ended.

Figure 19.45  (a) Penile swelling (and deformity) and scrotal lymphangiectasia in filariasis. (b) Saxophone penis in lymphatic filaria-
sis. Note surface changes due to chronic lymphedema. (c) Lymphatic cysts in a case of acquired lymphedema due to surgery for
inguinal hernia. (b – Courtesy: Dr. PC Das, Katihar, India; c – Courtesy: Dr Hiral Shah, Baroda Medical College, Vadodara, India.)
436  Male genitalia

●● During priapism, the shaft of the penis is rigid and

inflexible, but the glans of the penis is usually soft.
●● DD: Peyronie’s disease, penile implant.

Penile fracture
●● Penile fracture is a tear in the tunica albuginea of the penis.

●● Its most common etiology is a direct blow to the erect

penis during intercourse or masturbation. Erection thins

out the tunica and makes it more susceptible to injury.
●● Patients complain of a cracking or popping sound,

immediate pain, rapid detumescence, penile swelling,

and penile deviation away from the injury.
●● Urethral injury may occur in 15–22% of fractures.

●● Location of the fracture can often be identified by

physical examination in which focal tenderness and

overlying hematoma with contralateral penile deflection
may be present.
●● DD: Congenital penile curvature, penile dorsal vein

thrombosis, thrombophlebitis, Peyronie’s disease.

Phimosis Figure 19.46  Phimosis in lichen sclerosus.

●● Phimosis is the inability of the prepuce to be retracted

behind the glans penis in uncircumcised males.

●● It is normally seen in younger male children and

resolves in most cases by three years of age.

●● Adult phimosis is usually pathological and is caused

by poor hygiene, trauma, neglect, and an underlying

dermatosis including candidiasis, lichen sclerosus et
atrophicus, non-specific balanoposthitis in diabetic
patient, penile lymphedema, and malignant conditions
such as erythroplasia of Queyrat and squamous cell
carcinoma.
●● Congenital or physiological phimosis is clinically

asymptomatic and is often associated with ballooning of

the foreskin during voiding.
●● Pathological phimosis is symptomatic, and skin

irritation, dysuria, bleeding, and occasionally urinary

retention are noted.
●● Prepucial skin appears inflamed and edematous and

sometimes shows white circumferential scarring and

fissures, and patients complain of pain during sexual
activity (Figure 19.46).
●● DD: Paraphimosis, squamous cell carcinoma.

Paraphimosis
●● Paraphimosis is an emergency condition in which the

retracted prepuce cannot be returned to its normal

anatomic position. It is important to recognize this
condition promptly, as delay in management can result
in gangrene and amputation of the glans penis.
●● It develops when the retracted prepuce is left retracted

for several hours. The common causes include trauma,

infections, and sexual activity.
●● Patients present with acute onset of painful swelling of the

glans penis and prepuce. A constricting band of edematous

prepuce is noted directly behind the swollen glans penis. The Figure 19.47  (a) Paraphimosis in a young male after sexual
rest of the penile shaft appears normal (Figure 19.47a,b). activity. (Continued)
 Male genitalia  437

Figure 19.47 (Continued)  (b) Edematous swelling in

paraphimosis.

●● If left untreated, necrosis and subsequent auto-

amputation of the distal penis may occur.
●● DD: Phimosis.

Hypospadias
●● Hypospadias is an abnormality of the anterior urethral

and penile development in which an abnormal urethral

opening is ectopically located on the ventral penis.
●● The urethral opening may be located as far down

as the scrotum or perineum, extending up to the

navicular fossa.
●● It is often associated with a ventral curvature of the

penis (chordee) and/or a defective ventral foreskin.

●● It is characterized by abnormal urethral opening on the

ventral penis and is usually asymptomatic.

●● Distal hypospadias without curvature does not cause

any functional limitations.

●● Proximally, one can impair control of the urine stream.
●● An accompanying curvature can hinder sexual intercourse.

●● DD: Trauma, ambiguous genitalia. Figure 19.48  (a) Gonorrhea presenting with purulent
discharge. (b) Gonorrhea with copious purulent discharge.
Gonorrhoea (a – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India.)
●● Gonorrhea is a sexually acquired infection caused by

Neisseriagonorrhoeae. The incubation period is two to

five days.
●● The most common clinical manifestation is acute gonococcal pyoderma, epididymitis, acute or chronic
anterior urethritis with dysuria and profuse purulent prostatitis, vesiculitis, and periurethral abscess.
urethral discharge (Figure 19.48a,b). ●● DD: Urinary tract infection.
●● It starts with scanty, mucopurulent or mucoid discharge.

As it progresses, the discharge becomes thick, purulent, Genital foreign body (corpus alienum)
and profuse, with intense burning and pain during ●● Foreign body or corpus alienum is the presence within

micturition as well as increased frequency and urgency. the body of an object that originates outside the
●● Occasionally it can present as a disseminated form organism.
in immunocompromised patients with fever, acral ●● Male genital foreign bodies include injections

cutaneous pustules, arthritis, and tenosynovitis. of paraffin, mineral oil, and silicone in the
●● Complications include posterior urethritis, subcutaneous tissue of the penis in order to increase
inflammation of Cowper’s and Tyson’s glands, the penile girth.
438  Male genitalia

Figure 19.49  Penile foreign body. (Courtesy: Dr. Santoshdev

P Rathod, Ahmedabd, India.)
Figure 19.51  Herpes zoster affecting the penis, scro-
tum, and perineum. (Courtesy: Dr. Santoshdev P Rathod,
●● It presents as irregular, indurated nodules with varying Ahmedabd, India.)
degrees of sclerosis (Figure 19.49).
●● The prepuce, penile shaft, scrotum, and pubis are the
●● DD: Other causes of subcutaneous nodules of the male
sites most commonly involved; however, oily substances
genitalia, scleroderma, infiltrative lesions in storage
can migrate to subcutaneous tissues, muscles, and
disease, malignancies such as squamous cell carcinoma,
lymph nodes.
and infectious disease.
●● Its complications include penile necrosis, infection,
phimosis, erectile dysfunction, penile deformity, Additional images – Figures 19.50a,b, 19.51, 19.52,
fistulization, and acute urinary retention. 19.53, and 19.54

Figure 19.50  (a) Bowen’s disease of the glans penis. (b) Bowen’s
disease of the glans penis. (a – Courtesy: Dr. Swetalina Pradhan,
AIIMS Patna, India; b – Courtesy: Dr. Santoshdev P Rathod,
Ahmedabd, India.)
 Male genitalia  439

Figure 19.52  Edematous, distorted penis in lymphogranu-

loma venereum. (Courtesy: Dr. Santoshdev P Rathod,
Ahmedabd, India.)

Figure 19.54  Penile warts.

2. Shim TN, Ali I, Muneer A, Bunker CB. Benign male genital derma-
toses. BMJ 2016;354:i4337.
3. Yura E, Flury S. Cutaneous lesions of the external genitalia. Med
Clin North Am 2018;102(2):279–300.
4. Bunker CB, Porter WM. Dermatoses of the Male Genitalia. In:
Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, editors.
Rook,s Textbook of Dermatology. 9th edition. West Sussex: John
Wiley & Sons; 2016. P 111.1–111.41.
Figure 19.53  Atrophy of the skin of glans penis due to
5. Bunker CB. Diseases and Disorders of the Male Genitalia. In:
topical corticosteroid abuse. (Courtesy: Dr. Santoshdev
Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K,
P Rathod, Ahmedabd, India.) editors. Fitzpatrick’s Dermatology in General Medicine. 8th edi-
tion. McGraw Hill; 2012; 852–877.
REFERENCES
1. Kumar P, Khare S, Rathod SP, Nimisha E, Khoja M, Kulkarni S,
Tiwary AK, Madke B. The Genital, Perianal, and Umbilical Regions.
In: Smoller BR, Bagherani N, editors. Atlas of Dermatology,
Dermatopathology and Venereology. 1st edition. Switzerland:
Springer Nature; 2020. P. 1–80.
 E28
Female genitalia

MADHULIKA MHATRE, ASEEM SHARMA

ABSTRACT
By convention, female genital dermatoses have been classified as venereal and non-venereal. This approach clubs entities with
totally different morphological presentations together (e.g., genital herpes, and genital warts) which does not help much in
making a clinical diagnosis. This chapter has classified female genital dermatoses based on morphology, according to the
theme of the atlas.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

440 DOI: 10.1201/9781351054225-50

 E29
Nipple areola complex

AMARKANT JHA AMAR, SHIVANI SHARMA

ABSTRACT
The nipple-areolar complex is best considered a separate anatomical region of the breast and may be affected by many devel-
opmental physiological variations and pathological conditions (including various benign or malignant lesions). Many clinical
conditions are unique to this anatomical region, and awareness of such conditions helps clinicians in making a diagnosis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-51 441

 E30
Scrotum

DILIP KUMAR SA

ABSTRACT
This is an overview of various dermatoses that are predominantly seen on the scrotum. Clinical classification has been done
according to the types of skin lesions. The salient features of both common and rare conditions of scrotum have been described.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

442 DOI: 10.1201/9781351054225-52

 E31
Seborrheic area

POOJA NUPUR

ABSTRACT
Seborrheic areas include the scalp, eyebrows, nasal alae, chin and beard area, center of the chest, axilla, back, and groins. These
areas contain numerous sebaceous glands, which secrete sebum and contribute to the skin surface lipids. Any perturbation
of the sebaceous lipid barrier makes these areas susceptible to various infections (e.g., pityriasis versicolor) and inflammatory
conditions (e.g., seborrheic dermatitis). Some other conditions too show a predilection for seborrheic area. This chapter dis-
cusses dermatoses showing predilection for seborrheic areas.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-53 443

 E32
Intertriginous areas

POOJA NUPUR, SHAHID HASSAN

ABSTRACT
Intertriginous areas are some of the most commonly affected sites for dermatologic disorders of the face for which the major-
ity of patients consult a physician. A variety of skin diseases may involve these areas as a part of primary cutaneous diseases
or secondary to the underlying systemic diseases. At first glance, many of the intertriginous dermatoses may appear similar.
However, a thorough history and physical examination may further narrow the differential diagnosis considerably or help us
reach a specific diagnosis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

444 DOI: 10.1201/9781351054225-54

 20
Nails

PIYUSH KUMAR

INTRODUCTION
Nail
The nail unit is a specialized structure situated on the dor- plate
sal aspect of the distal-most part of each finger and toe. Lateral
The nail unit consists of a nail plate, four modified epithe- nail fold
lial tissues (proximal nail fold, nail matrix, nail bed, and
Lunula
hyponychium), lateral nail folds, and dermal tissue under
Proximal
the nail bed (Box 20.1 and Figures 20.1 and 20.2). The nail nail fold
is firmly anchored to the underlying bone of the digit with Cuticle
the help of the nail folds, dermal tissue of the nail bed, and
fibrous tissues merging with the periosteum and tendons.
The detailed discussion on the anatomy is beyond the scope
of this chapter.
Nail involvement in various diseases results in recog- Surface View
nizable abnormalities of one or more components of the
nail unit in varying combinations. The diagnosis of nail Figure 20.1  Surface view of the nail unit. (Courtesy:
diseases rests on identification of these abnormalities. Nail Dr. Sunil Kothiwala; Reprinted by permission from
Taylor and Francis Group LLC Books Nail Disorders: A
unit abnormalities (nail unit signs) have been discussed
Comprehensive Approach by Archana Singal, Shekhar
as abnormalities of nail plate (size, thickness, curvature, Neema, Piyush Kumar [COPYRIGHT] 2019.)

BOX 20.1: The nail apparatus

●● Nail plate – It is a horny rectangular translucent plate made up of hard keratin plate which appears pink due to
vascular structures underneath. The proximal semilunar whitish area is termed as lunula. The surface of nail plate is
generally convex in both longitudinal and horizontal directions. Histologically, nail plate has three horizontal layers –
thin dorsal lamina, thick intermediate layer and thin ventral lamina.
●● Nail folds – The nail folds (proximal and paired lateral nail folds) are soft tissues that partially cover the nail plate.
The proximal nail fold keratinizes to produce cuticle.
●● Cuticle – It is a thin, translucent fold of skin extending onto proximal nail plate.
●● Hyponychium – It is an epithelial tissue underlying the free edge of the nail plate and is distally continuous with the
volar skin of the digit.
●● Matrix – It is the germinal part of the nail unit and is mostly covered by the proximal nail fold (except for lunula). It
keratinizes to produce the major bulk of the nail plate – the proximal part of the nail matrix gives rise to the ventral
nail plate, and the distal nail matrix contributes to the intermediate nail plate (Figure 20.3). Understanding this fact
is crucial in determining the exact site of nail matrix pathology and nail matrix biopsy.
●● Nail bed – The nail bed consists of specialized epithelium, two to three cells thick, with underlying connective tis-
sue in close proximity to the periosteum. The connective tissue is devoid of subcutaneous fat but is rich in nerves,
blood vessels, and glomus bodies.

DOI: 10.1201/9781351054225-55 445

446 Nails

Dorsal
Intermediate Nail
Nail Proximal Plate
Ventral
matrix
Distal

Nail bed

Figure 20.3  Formation of nail plate by nail matrix and nail bed.

surface changes, adhesion, and color), and surrounding soft

tissues Table 20.1.1–6

Figure 20.2  The longitudinal section of the nail unit:

Abnormalities of nail plate size
(a) Nail plate, (b) Cuticle, (c) Proximal nail fold,
Anonychia/Micronychia
(d) Hyponychium, (e) Nail bed, (f) Terminal phalanx
●● Anonychia is complete absence of nails in single or
bone, (g) Nail matrix. (Courtesy: Dr. Sunil Kothiwala.)
multiple digits (Figure 20.4).

Table 20.1  Various nail signs

Affected part of the

nail unit Features Nail sign
Nail plate Absent Anonychia
Abnormalities of Small Micronychia, onychoatrophy
nail-plate size Large Macronychia
Width > length Brachyonychia (racquet nail)
Deformed Onychodystrophy
Abnormalities of Increased • Complete nail involvement – pachyonychia,
nail-plate thickness onychochauxis, onychogryphosis
• Partial nail involvement – onychomatricoma (usually single
nail)
Decreased • Complete nail affected – nail-plate thinning, haplalonychia
• Partial nail involvement – traumatized nails, worn-down nails
Abnormalities of Flat (loss of normal curvature) Platonychia
nail-plate curvature Concave/spoon-shaped (reversal Koilonychia
of normal curvature)
Increased transverse and Clubbing
longitudinal curvatures
Increased transverse curvature Pincer nails, Tile-shaped nails, plicated nails
Increased longitudinal curvature Parrot beak nails
Abnormalities of Longitudinal indentation • Single/few – Longitudinal groove, median canaliform
nail-plate surface dystrophy of Heller
• Numerous (affecting entire nail plate) – onychorhhexis
Longitudinal elevation • Single/few – Longitudinal ridge (complete length of nail
plate affected) and beads (partial nail plate affection)
• Numerous – trachyonychia
Transverse indentation • Single or few – Beau’s line
• Numerous – habit tic deformity
Focal defect/indentation Pits
Nail plate fragility Increased Brittle nails
Abnormalities of Distal separation of different layers Onychoschizia
nail-plate adhesions of nail plate
Distal separation of nail plate from Onycholysis
nail bed
Proximal separation of nail plate Onychomadesis
from nail bed
Abnormal adhesions Dorsal and ventral pterygium
(Continued)
 Nails 447

Table 20.1  Various nail signs (Continued)

Affected part of the

nail unit Features Nail sign
Abnormalities of Nail plate loses translucency and Chromonychia
nail-plate color appears colored
White Leukonychia (true, apparent, and pseudo)
Red Erythronychia
Brown-black Melanonychia
Others Yellow nails, green nails
Alterations in lunula • Red lunula
• Macro Lunula
• Triangular lunula
Nail folds changes Inflamed Paronychia, onychocryptosis
Pigmentation • Hutchinson’s sign
• Pseudo-Hutchinson’s sign
Subungual changes Collection of keratin material Subungual hyperkeratosis
Collection of blood • Large – subungual hematoma
• Tiny, linear – splinter hemorrhage

●● It could be congenital or acquired; congenital forms

may be a syndromic (e.g., ectodermal dysplasia) or
isolated disorder.
●● Acquired forms may be permanent or transient. Transient
anonychia can occur in various inflammatory processes.
●● Micronychia (Figure 20.5) is too-small nail plates.
●● Some important acquired causes of anonychia/
micronychia include burn, trauma/surgery, lichen
planus, Stevens-Johnson syndrome, and epidermolysis
bullosa (junctional and dystrophic types).

Onychoatrophy
●● Onychoatrophy is faulty underdevelopment of the nail

in which there is reduction in size and thickness of the

nail unit (Figure 20.6). Figure 20.5  Micronychia of middle finger in leprosy.

Macronychia
●● The nail plate is too large (Figure 20.7).
●● Macronychia occurs from an underlying bone

abnormality.

Figure 20.4  Anonychia of great toenails in epidermolysis

bullosa. Figure 20.6  Onychoatrophy in a case of lichen planus.
448 Nails

Figure 20.7  Macronychia.

Brachyonychia/racquet nail
●● Racquet nails refers to short nails in which the width

of the nail plate and nail bed is greater than the length.
It occurs due to shortening of the bone of the terminal
phalanx.
●● Usually thumbs are affected (Figure 20.8).

Onychodystrophy/dystrophic nail
●●Onychodystrophy refers to nails that become misshapen
or thickened or that exhibit a partially destroyed nail
plate (Figure 20.9a,b).

Figure 20.9  (a,b) Onychodystrophy of toenail.

Abnormalities of nail plate thickness

Nail-plate thickening
●● Normal nail plate gains thickness as it grows distally.

The vascular supply, subungual hyperkeratosis, and

drugs may influence nail thickening.
●● Nail-plate thickening may be congenital or acquired.

Figure 20.8  Racquet nail – short nail plate with length less Acquired thickening may occur as a result of reduced
than the width. nail growth or inflammatory disorders of the nail
 Nails 449

bed. Subungual hyperkeratosis due to onychomycosis, ●● Onychochauxis – It is characterized by a thickened

pachyonychia congenita, pityriasis rubra pilaris, or nail with yellowish discoloration without
psoriasis also results in nail-plate thickening. any change in the curvature of the nail plate
●● Nail thickening is also commonly seen in elderly and (Figure 20.10b).
in patients with foot deformities due to chronic trauma ●● Onychogryphosis – It is characterized by nail-
and friction. plate thickening with gross hyperkeratosis
●● Thickened nail plates may assume different and increased curvature of the nail plate
morphologies. (Figure 20.10c,d). It most commonly affects the
●● Pachyonychia – It refers to a thickened nail with an great toes of elderly patients with poor foot care
increased transverse curvature (Figure 20.10a). and podiatric abnormalities.

Figure 20.10  (a) Pachyonychia. (b) Onychochauxis. (c) Oyster-like onychogryphosis. (d) Ram’s horn dystrophy (onychog-
ryphosis). (Continued)
450 Nails

Figure 20.10 (Continued)  (e) Longitudinal yellow

discoloration and thickening of the nail plate in
onychomatricoma.

●● Partial nail-plate thickening of a single digit may be

seen in nail matrix tumors such as onychomatricoma
(Figure 20.10e).

Nail-plate thinning
●● Nail-plate thinning is defined as thickness of the nail

plate less than 0.5 mm. Nail-plate thinning results

from diseases affecting the proximal nail matrix
and is often associated with fissuring, ridging, and
onychorrhexis. They break easily or split at the free
edge of the nail plate.
●● They are seen in amyloidosis, hemiplegia, occupational

contact with chemicals, trachyonychia, and lichen

planus (Figure 20.11a).
●● In lichen planus, uniform thinning is due to atrophy of

proximal nail matrix and is referred to as “angel wing”

deformity.
●● In hapalonychia (egg shell nails), nail plates become

thin and assume a semitransparent, bluish-white

hue (Figure 20.11b). Such nails may be observed in
malnutrition, chronic arthritis, leprosy, myxoedema,
acroasphyxia, peripheral neuritis, hemiplegia, and
cachexia etc.
●● Focal nail plate thinning may be seen in traumatized

nails, onychoschizia, and worn-down nails Figure 20.11  (a) Nail plate thinning in lichen planus. (b) Egg
(Figure 20.11c). shell nail. (c) Distal triangular thinning in worn-down nails.
 Nails 451

Table 20.2  Koilonychia

Koilonychia Causes
Physiological • Healthy infants
• Old age
Familial • Syndromic – LEOPARD syndrome,
nail–patella syndrome
• Isolated
Acquired • Dermatological diseases – lichen
koilonychias planus, psoriasis, lichen striatus,
keratoderma, Darier disease,
Raynaud’s disease
• Systemic diseases – iron deficiency
Figure 20.12  Flat nail plates.
anemia, hemochromatosis,
polycythemia vera, malnutrition,
Abnormalities of nail plate curvature systemic lupus erythematosus,
pellagra, thyroid diseases
Platonychia Occupational • Toenails – rickshaw pullers, farmers
●● The transverse curvature of the nail plate is lost, koilonychias • Fingernails – dentists, construction
resulting in an abnormally flat nail (Figure 20.12). It workers, butchers, and automotive
may be an early feature of koilonychia. workers
Others • Trauma
Koilonychia (spoon nails) (derived from Greek word
koilos meaning hollow)
●● It is a common nail dystrophy in which the dorsal

surface of nail plate becomes flat or truly concave. It is ●● Various causes of koilonychias have been summarized
believed that anoxia and atrophy of the distal matrix are in Table 20.2.
responsible for development of koilonychias.
●● Koilonychia is the converse of clubbing. It is more Clubbing (Hippocrates fingers, acropachy)
visible when viewed from the side. When a drop of ●● Clubbing refers to increased transverse and

water is put on the surface, it will not roll off. It should longitudinal curvatures of the nail plate along
be noted that nails in koilonychia are brittle. It is with hypertrophy of the soft tissue of nail pulp
commonly seen in fingernails rather than toenails. (Figure 20.14a).
●● Physiological koilonychias is seen in healthy infants ●● It can be hereditary (autosomal dominant with variable

and children who have thin and soft nails and improves penetrance) or acquired and may be unilateral or
spontaneously in due course. bilateral (Figure 20.14b, Table 20.3).
●● Two types of koilonychia are described – familial form ●● Clinical changes are present in nails and fingertips.

and acquired form. Nails bulge along both the longitudinal as well as
●● Iron deficiency is the most common cause of acquired transverse axes. Changes are prominent in the three
koilonychias. It may develop before clinical or radial digits. Tissues around the terminal phalanx
laboratory signs of anemia manifest (Figure 20.13). become hypertrophied and appear as “drum sticks”
(Figure 20.14c). It is insidious in onset. Sometimes
abrupt clubbing can be seen in lung carcinoma.
●● Three geometric assessments exist, namely Lovibond’s

angle, Curth’s angle, and Schamroth’s window

(Figure 20.14d).
●● Lovibond’s angle is found at the junction between
the nail plate and proximal nail fold. Normally it
is less than 160°. It is altered to more than 180° in
clubbing.
●● The angle at the distal interphalangeal joint is called
Curth’s angle and is normally 180°. It is reduced to
160° in clubbing.
●● Schamroth’s window is formed when the dorsal
aspects of fingers on opposite hands are apposed. In
Figure 20.13  Koilonychia in iron deficiency anemia. clubbing, this window is closed (Figure 20.14e).
452 Nails

Lovibond
Curth angle
angle >160°
<160°
180° 160°

Normal finger Clubbed finger

A B
(d)

●● Clubbing should be differentiated from pseudoclubbing,

in which Lovibond’s angle is preserved. Pseudoclubbing
may be seen in nail bed tumors or in diseases of distal
phalangeal bone.

Transverse overcurvature of the nail

●● Nail curvature may exceed the normal limits, resulting

in nail-plate abnormalities. These deformities may be

associated with ingrowing nails, but inflammatory
edema is absent. Three types have been recognized.

Figure 20.14  (a) Clubbing of finger nails. (b) Familial

clubbing. (c) “Drum sticks” appearance in clubbing.
(d) Geometric assessments for clubbing. (e) Closed
Schamroth’s window in clubbing. (b – Courtesy: Dr. PC
Das, Katihar, India.)
 Nails 453

Table 20.3  Clubbing

Unilateral
clubbing Bilateral clubbing
• Idiopathic • Pulmonary disease – lung cancer,
• Arterial cystic fibrosis, empyema, pleural
aneurysms mesothelioma, lung hydatid cysts,
and fistula pulmonary metastases
• Hemiplegia • Cardiac disease – Cyanotic
• Pancoast congenital heart disease, other
tumor causes of right-to-left shunting, and
• Local injury bacterial endocarditis
• Gastrointestinal disease – ulcerative
colitis, Crohn’s disease, primary
biliary cirrhosis, cirrhosis of the liver,
hepatopulmonary syndrome
• Dermatological disease –
pachydermoperiostosis
• Malignancies – thyroid cancer;
Hodgkin disease; chronic myeloid
leukemia; polyneuropathy,
organomegaly, endocrinopathy,
monoclonal gammopathy, and skin
changes (POEMS) syndrome
• Endocrinological diseases –
acromegaly, thyroid acropachy,
secondary hyperparathyroidism
• Other conditions – pregnancy,
sickle cell disease, alcoholism, HIV
infection
• Drugs – hypervitaminosis A, heroin
abuse, laxative abuse, heavy metal
poisoning, angiotensin II receptor
blocker

●● Pincer nail (trumpet, arched, or omega nail): This

is characterized by transverse overcurvature, the
degree of which increases distally (Figure 20.15a,b),
resulting in pinching of the underlying nail bed and
pain, even at rest. This condition may be inherited
or acquired after prolonged use of ill-fitting shoes
or high-heeled shoes. Acquired pincer nails usually
affect great toes. However, if fingernails are affected,
inflammatory osteoarthritis or subungual exostosis
should be looked for.
●● Tile-shaped nail: There is an increase in the transverse
curvature, but, unlike with pincer nails, lateral margins
remain parallel (Figure 20.15c). This is an acquired
form and associated with degenerative osteoarthritis
affecting nails of fingers and toes.
●● Plicated nail: The surface is flat, but the lateral margins
are sharply angled, forming vertical sides that are
parallel (Figure 20.15d). This is due to injury to the Figure 20.15  (a,b) Pincer nail. (c) Tile-shaped nails show-
distal phalanx and nail unit by psoriasis and total ing increased transverse curvature. Note the parallel
destructive onychomycosis. lateral margins. (Continued)
454 Nails

Abnormalities of nail-plate surface

Longitudinal grooves
●● Longitudinal grooves (LG) are canal-like longitudinal

defects on the nail surface (Figure 20.17a). The depth

of the groove may be complete or partial. It should be
differentiated from ridges, which are linear elevations
on the surface.
●● Longitudinal grooves occur due to long-standing

nail abnormalities, causing defects in the nail matrix

growth. LG can be solitary or multiple, with different
clinical implications.
●● Single (or a few) longitudinal groove affecting one or
multiple nails – This occurs due to the pressure effect
of space occupying lesions (SOL) like wart, myxoid
cyst, or periungual fibromas (Koenen tumor) on the
nail matrix. The groove is deep and wide. SOL can
be visualized at the proximal portion of longitudinal

Figure 20.15 (Continued)  (d) Plicated nail surface due to over

curvature of nail plate. Vertical growth of lateral margins is
evident. (c,d – Courtesy: Dr. Balachandra S. Ankad.)

Longitudinal overcurvature of the nail plate

Nail beaking (Parrot beak nails)
●● It is a condition where the distal nail plate bends around

the tip of the digit; it is usually seen in fingernails

(Figure 20.16).
●● It is usually seen in systemic sclerosis and leprosy, where

there is shortening of the terminal phalanges.

Figure 20.17  (a) Longitudinal grooves on the nail plate.

Figure 20.16  Nail beaking in a case of leprosy, with short- (b) Solitary longitudinal groove with an angiofibroma
ening of the digit. visible at its proximal end. (Continued)
 Nails 455

Figure 20.17 (Continued)  (c) Median canaliform deformity of Heller. (d) Onychorrhexis.

canal (Figure 20.17b). It may also result from repeated Longitudinal ridges and beads
cuticle manipulation as in habit tic deformity. ●● Longitudinal ridges (continuous elevations running
●● Multiple longitudinal grooves – Multiple LG may be from the cuticle to the free edge of nail plates) and beads
observed in various physiological (young children, (elevations running for a short distance on the nail plate
old age) and pathological conditions (lichen planus, surface) are minor changes and may not indicate any
Darier disease, rheumatoid arthritis). disease (Figure 20.18a,b). However, they may become
●● Some specific examples of longitudinal grooves include prominent with age. Some known associations include
the following: leprosy and other neuropathies, rheumatoid arthritis,
●● Median canaliform dystrophy of Heller (MCDH)
is most distinctive form of LG. This uncommon
condition consists of central or paracentral,
longitudinal groove or split that starts at the cuticle
or mid-way along nail plate and extends up to free
edge of nail plate (Figure 20.17c). This longitudinal
groove is associated with multiple small transverse
fissures or grooves extending from the groove
towards the periphery (but do reaching the edge of
nail plate) – a “fir-tree” appearance. Usually thumbs
and toes are affected symmetrically, but other
digits too may be affected. The exact etiology of this
condition is not known; it may be idiopathic or may
develop after repeated self-inflicted trauma (impulse
control disorder).
●● Onychorrhexis is another LG in which a series of
shallow and narrow furrows are present, running
parallel to each other and involving the entire surface
of the nail (Figure 20.17d). The common conditions
causing onychorrhexis are included in Table 20.4.

Table 20.4  Onychorrhexis

Etiology Diseases
Medical causes Hypothyroidism, bulimia, anemia,
anorexia nervosa
Dermatological Psoriasis
Trauma Repeated injuries like keyboard players
Chemical Excessive exposure to soaps and
trauma detergents,
Nail polish removers
Miscellaneous Exposure to cold, genetic, old age
Figure 20.18  (a) Longitudinal ridges. (Continued)
456 Nails

nails.” The term “twenty-nail dystrophy” is misleading,

as any number of nails may be affected and hence,
trachyonychia is the preferred term (Figure 20.19a).
●● Two clinical types are recognized. Both types may
have associated koilonychia, onychoschizia, and
hyperkeratosis of the cuticles.
●● Opaque trachyonychia – It is a severe type and
is characterized by rough, thin, brittle nails with
excessive longitudinal ridging (Figure 20.19b).

Figure 20.18 (Continued)  (b) Longitudinal beads.

(c) Chevron nail.

collagen vascular diseases, systemic amyloidosis, and

nutritional deficiency.
●● Chevron nail/herringbone nail is an uncommon
example of patterned longitudinal ridges seen in
children; they resolve in adulthood. The ridges arise
from the proximal nail fold and converge in a V-shaped
pattern towards the midpoint distally (Figure 20.18c).

Trachyonychia (rough nails, twenty-nail dystrophy,

from the Greek word trakos for rough)7
●● Trachyonychia refers to gray, rough nail plates that lose Figure 20.19  (a) Multiple, but not all, digits affection in
luster and appear as “sandpapered nails” or “sand-blasted trachyonychia. (b) Opaque trachyonychia. (Continued)
 Nails 457

Figure 20.19 (Continued)  (c) Shiny trachyonychia.

●● Shiny trachyonychia – It is a less severe type and

presents as shiny nails with superficial ridging
and numerous pits. The nail is less thin and fragile
compared to opaque trachyonychia (Figure 20.19c).
●● The common causes of trachyonychia include alopecia
areata, lichen planus, and psoriasis.
●● It is more common in children, where it may show Figure 20.20  (a,b) Multiple Beau’s lines.
spontaneous resolution over years, and is mostly
idiopathic. In adults, the disease runs a longer course.
Table 20.5  Beau’s lines
However, it does not cause permanent nail changes
or scarring even when it is associated with lichen Single or a
planus. few digits Multiple digits affected

Transverse grooving (TG) • Trauma • Physiological – infants, cyclically with

●● Transverse grooves are horizontal indentations on
• Paronychia menstruation
• Psoriasis • Systemic diseases – coronary
the nail surface, caused by injury to the nail matrix,
• Eczema thrombosis, pulmonary embolism,
resulting in transient cessation of nail growth. TG
renal failure, Kawasaki disease, zinc
moves distally when the nail matrix resumes its
deficiency, malnutrition
function, and it ultimately disappears.
●● Beau’s lines are transverse grooves that may affect
• Dermatological diseases – autoimmune
vesicobullous diseases (pemphigus
a few, multiple, or all digits (Figure 20.20a,b and
vulgaris, epidermolysis bullosa
Table 20.5). The depth and width of the groove
acquisita, pemphigoid gestationis,
give clues to the severity and duration of illness
linear IgA disease, etc.), pustular
respectively. The distance of the groove from
psoriasis, Stevens-Johnson syndrome
the proximal nail fold gives an estimate of the
• Infections – measles; mumps;
timing of injury in the past. Multiple Beau’s lines
pneumonia; hand, foot, and mouth
indicate a repetitive injury to the nail matrix, as in
disease
chemotherapy. These lines are non-specific but serve as
• Drugs – retinoids, metoprolol,
“retrospective indicators” of many pathological states.
anti-mitotic agents, dapsone
The thumb nail supplies information for the previous
hypersensitivity syndrome
five to six months, whereas the great toe may give
• Others – severe exposure to cold
evidence of past disease for up to two years.
458 Nails

Figure 20.21  (a) Habit tic deformity of both thumb nails caused by proximal nail fold pushed back repeatedly. (b) Thumb
nail showing severe changes of habit tic deformity.

●● Sometimes a very deep Beau’s line may develop as a Pitting (pits, erosions, onychia punctata)
result of complete cessation of nail matrix activity (for ●● Pits are appreciable as punctate indentations on the
more than two weeks duration) and cause total division nail plate and occur due to defective keratinization
of the nail plate, resulting in latent onychomadesis. of the proximal matrix, with persistence of foci of
parakeratotic cells in the nail plate (Figure 20.22a).
Habit tic deformity
As the nail plate grows beyond the proximal nail
●● Habit tic deformity (washboard nails) is caused by
fold, abnormal parakeratotic keratinocytes are
repetitive external trauma to the nail matrix and is lost, leaving behind tiny depressions or pits on the
clinically characterized by transverse grooves and surface. Pits commonly affect fingernails more than
parallel ridging, both running from the nail fold to the toenails.
distal edge of the nail; the surface changes resemble a ●● Pits are seen in various inflammatory conditions
washboard (Figure 20.21a,b). (Table 20.6). Trauma is the most common cause of pits
●● Thumbs are most commonly affected.
affecting a single digit.

  
Figure 20.22  (a) Nail pitting in a case of erythroderma.
(b) Coarse, irregularly arranged nail pits in psoriasis.
(Continued)
 Nails 459

Figure 20.22 (Continued)  (c) Shallow, regularly arranged nail pits. (d) Elkonyx in psoriasis.

●● Pits vary in depth, size, shape, and number, depending ●● In diabetes mellitus, pits are seen as small craters
on the etiology. on the middle and ring fingers and are referred to as
●● In psoriasis, they are irregular, coarse, and randomly Rosenau’s depressions.
placed on the surface. The presence of more than 20 ●● Occasional pits may appear in normal individuals.
pits is suggestive of psoriasis (Figure 20.22b).
●● In alopecia areata, pits are small and shallow, Increased nail plate fragility
arranged in a regular and uniform pattern or in
a grid-like pattern (Glen-plaid or Scotch-plaid Brittle nails
pattern) (Figure 20.22c). ●● Brittle nails are clinically characterized by
●● Sometimes a single, larger and deeper pit is noted in onychoschizia and onychorrhexis (Figure 20.23).
psoriasis and is called an elkonyxis (Figure 20.22d).
Occasionally, it is also seen in secondary syphilis,
Reiter’s syndrome, after etretinate, and in
isotretinoin therapy.
●● In eczema, coarse and irregular pits are seen in
affected fingernails.
●● In Reiter’s syndrome and secondary syphilis, pits
are seen in lunula, giving a mottled appearance to
the lunula.
Table 20.6  Nail pitting

Etiology Diseases
Dermatological • Single (/few) digit – chronic
diseases paronychia, lichen nitidus, lichen
striatus, parakeratosis pustulosa
• Multiple digits – psoriasis, alopecia
areata, eczema, lichen planus, Reiter’s
disease, pityriasis rosea, pityriasis
rubra pilaris, pemphigus vulgaris,
chemical dermatitis, dermatomyositis
Systemic Diabetes mellitus, systemic lupus
diseases erythematosus, sarcoidosis,
rheumatoid arthritis
Others Drug induced erythroderma, trauma
Figure 20.23  Brittle nails in a homemaker.
460 Nails

Figure 20.24  (a) Onychoschizia of finger nail. (b) Onychoschizia of toe nail.

●● It may be idiopathic and is common in women. layers, and in this condition it is split horizontally into
Local causes including damage by alkalis, solvents, layers (Figure 20.24a,b).
sugar solutions, and hot water may be ●● Repeated soaking of nails in water followed by drying
contributory. is considered crucial in development of onychoschizia.
●● Dermatological diseases like eczema, lichen planus, and Hence, it is commonly seen in those who are involved
onychomycosis may present with brittle nails. in such activities – house cleaners, nurses, hairdressers,
●● Systemic causes include endocrinopathies, tuberculosis, etc. Nail cosmetics and exposure to chemicals (solvents,
Sjögren’s syndrome, malnutrition, anemia, and thioglycolates) also predispose a person to development
peripheral neuropathy. of onychoschizia.

Onycholysis
Abnormalities of nail plate adhesions
●● Onycholysis is the separation of the nail plate

Onychoschizia (Lamellar nail dystrophy, peeling of nails) from the underlying nail bed and/or lateral nail
●● It is splitting of nail plate at the free edge in fingers folds, starting at the distal end and progressing
and toes. The nail is formed in layers analogous to skin proximally (Figure 20.25a). The detached part of

Figure 20.25  (a) Onycholysis. (b) Onycholysis in nail psoriasis. Note distal nail plate appearing white (area of onycholysis)
and erythema proximal to it.
 Nails 461

the nail plate loses translucency and appears white

(or yellowish) (Figure 20.25b). Sometimes, the area
may be colonized by Pseudomonas spp, producing a
greenish hue.
●● Additional findings provide important diagnostic
clues and include proximal erythematous border
(psoriasis vide Figure 20.25b), yellowish discoloration
(onychomycosis vide Figure 20.25a), green
discoloration (Pseudomonas spp. colonization) and red
discoloration (photo-onycholysis).
●● The causes of onycholysis are summarized in
Table 20.7.

Onychomadesis
●● It is spontaneous separation of nail plate from the

nail bed, starting from its proximal portion. It

Table 20.7  Onycholysis

Single nail
affection Few nails/Multiple digits affected
• Trauma • Dermatological causes –
• Foreign body psoriasis, onychomycosis,
implantation eczema, pachyonychia
• Nail bed tumors congenita, lichen planus,
– both benign pemphigus vulgaris and
and malignant vegetans, reactive arthritis,
lichen striatus, contact
dermatitis from various nail
cosmetics
• Systemic causes – anemia (iron
deficiency), diabetes mellitus,
hyperthyroidism and
hypothyroidism, peripheral
arterial diseases, pellagra,
erythropoietic porphyria,
porphyria cutanea tarda,
histiocytosis X, sarcoidosis,
scleroderma, amyloid and Figure 20.26  (a,b) Onychomadesis.
multiple myeloma
• Trauma – pedicure/manicure,
mechanical trauma
results from complete cessation of growth of the
• Chemical exposure – paint
proximal nail matrix lasting for more than two weeks
removers, dicyandiamide,
(Figure 20.26a,b).
thioglycolate, solvents, color ●● Onychomadesis and Beau’s lines are caused by the same
developers
set of conditions; the difference lies in severity and
• Photo-onycholysis –
duration of the insult.
tetracyclines, psoralens, ●● Sometimes total loss of nail is observed due to
fluoroquinolones, oral
destruction of the matrix following trauma and lichen
contraceptives, griseofulvin
planus.
• Drugs (not through photo-
onycholysis) – chemotherapy, Pterygium (from the Greek word pterygion
retinoids, hydroxylamine meaning wing)
• Others – pregnancy, prolonged ●● Pterygium refers to a nail abnormality caused by
maceration abnormal adhesions between two components of
• Idiopathic and familial forms the nail units. Two types are recognized – dorsal
462 Nails

(pterygium unguis) and ventral (pterygium inversus

unguis).
●● Dorsal pterygium: It is characterized by linear or
“wing-like” fibrotic tissue that causes the proximal
nail matrix to adhere to the nail matrix and nail
bed. Usually the nail plate gets divided into two
halves, and sometimes, in severe cases, the whole
nail plate is lost (Figure 20.27a,b). It indicates
severe nail disease and is due to destruction of nail
matrix. It is a specific, end-stage finding in lichen
planus and a hallmark of severe disease. However,
it is also observed in other conditions, such as
burns, trauma, radiation dermatitis, cicatricial
pemphigoid, graft-versus-host disease, and toxic
epidermal necrolysis.
●● Ventral pterygium: In this condition,
hyponychium gets attached to the undersurface
of the nail plate, causing obliteration of distal nail
groove (Figure 20.27c). It is usually asymptomatic
but may cause pain during trimming of the
affected nails. The common causes include
trauma, systemic sclerosis, lupus erythematosus,
and leprosy. Rarely, the condition may be
idiopathic or familial.

Abnormalities of nail plate color

Chromonychia
●● The nail plate normally appears as a “pink translucent”

structure, and any deviation from the normal

appearance is termed chromonychia. The complete nail
plate or its part may be affected.
●● Chromonychia can be exogenous or endogenous.

Exogenous chromonychia (Figure 20.28a) occurs

after exposure to dyes (e.g., silver nitrate, potassium
permanganate) and decorative use of henna, and the

Figure 20.27  (a) Dorsal pterygium due to trauma. (b) Dorsal pterygium in nail lichen planus. (c) Ventral pterygium in
multiple digits (circled) in a case of systemic sclerosis.
 Nails 463

Figure 20.28  (a) Exogenous chromonychia after henna application. Note that proximal margin of chromonychia follows the
shape of proximal nail fold. (b) Endogenous chromonychia due to subungual hematoma. Note that proximal margin of chrom-
onychia follows the lunula. (c) Punctate leukonychia in a child. (d) Mees’ lines. (e) Total leukonychia. (f) Terry’s nails. (Continued)
464 Nails

Figure 20.28 (Continued)  (g) Terry’s nails. (h) Half

and half nails. (i) Half and half nails. (j) Muehrck’s
nails. (k) Apparent leukonychia in onychomycosis.
(g,i – Courtesy: Michela Starace, Aurora Alessandrini,
and Bianca Maria Piraccini.)

proximal margin of chromonychia follows proximal ●● Various clinical forms of chromonychia have been
the nail fold. In contrast, the proximal margin of established – white nails (leukonychia), yellow nails,
endogenous chromonychia (Figure 20.28b) parallels green nails, red nails (erythronychia), and brown-black
the lunula. nails (melanonychia) (Tables 20.8 and 20.9).

Table 20.8  Leukonychia

Types Features Examples

True Nail matrix diseases result in Four types are known
leukonychia parakeratotic nuclei retained within the • Punctate – idiopathic, trauma, psoriasis) (Figure 20.28c)
nail matrix. • Transverse – trauma, heavy metal poisoning (e.g., Mees’
True leukonychia (partial) lines – single or multiple, transverse, narrow whitish line
moves distally as the nail grows. running along the width of the nail and parallel to lunula,
It does not fade on pressure. Figure 20.28d), chemotherapy
• Longitudinal – Darier disease, Hailey-Hailey disease
• Total – congenital (Figure 20.28e)
(Continued)
 Nails 465

Table 20.8  Leukonychia (Continued)

Types Features Examples

Apparent Whitening of nail plate is because of • Terry’s nails describes a nail with a proximal white and
leukonychia abnormal subungual tissues. distal normal-appearing (or brown) nail plate. It is seen
Apparent leukonychia develops because in liver cirrhosis and congestive heart failure
of pallor of nail bed due to anemia and (Figure 20.28f,g).
vascular impairment. • Half and half nails (Lindsay’s nails) are seen in uremic
It disappears or fades on pressure. patients in whom proximal white and distal brownish
discoloration is noted (Figure 20.28h,i).
• Muehrcke’s nails are seen in hypoalbuminemia (< 2gm/
dl) and in patients receiving chemotherapy as paired (or
multiple) bands of white lines. A pink line lies between
the white lines (Figure 20.28j). Usually, the second,
third, and fourth fingernails are affected, and thumbs
are spared. Correction of the hypoalbuminemia reverses
the sign.
Pseudo Pseudo leukonychia is whitening of nail • Onychomycosis (Figure 20.28k), keratin granules.
leukonychia plate and nail bed due to the
external origin.

Table 20.9  Chromonychias

Chromonychia Features Diseases

Erythronychia Focal Glomus tumor
(Figure 20.29a)
Longitudinal Onychopapilloma
band (single nail), Darier
disease (alternate
longitudinal
leukonychia and
erythronychia
(Figure 20.29b),
Hailey-Hailey
disease
Yellow nails Distal, focal, Onychomycosis
varying (Figure 20.29c)
shades
Complete Yellow nail syndrome
(Figure 20.29d,e)
Green nails Associated Pseudomonas
onycholysis colonization
(Figure 20.29f)
Brown-black Longitudinal Splinter hemorrhage
color 1- to 3-mm (Figure 20.29g)
lines
Focal or Subungual hematoma
complete (Figure 20.29h),
Fungal
melanonychia
Figure 20.29  (a) Focal erythronychia and swelling in glo-
Band-like Melanonychia (Figure
mus tumor of nail bed. (b) Longitudinal erythronychia in
pigmentation 20.29i) Darier disease. (Continued)
466 Nails

Figure 20.29 (Continued)  (c) Onychomycosis with yellow and other discolorations. (d) Yellow nail syndrome. (e) Yellow nail
syndrome. (f) Pseudomonas spp colonization causing green nails. (g) Splinter hemorrhage in a case of herpes zoster.
(h) Subungual hematoma following intramatricial injection. (Continued)
 Nails 467

Table 20.10  ABCDEF rule

ABCDEF
mnemonic Features
A • Age peak – elderly population
(40–70 years of age)
• Predisposed race – Asians, Africans,
African-Americans, and Native
Americans
B • Brown-black pigment
• Breadth (width) – > 3–5 mm
• Border – irregular
C • Change – rapid increase in width and
growth rate of nail band; nail dystrophy
does not improve with “adequate
treatment”
D • Digit involved – single more often than
Figure 20.29 (Continued)  (i) Multiple bands of longi- multiple, dominant hand, thumb more
tudinal melanonychia. (a – Courtesy: Dr. Rajesh Kumar often than big toe, which is more often
Mandal, North Bengal Medical College and Hospital, than index finger
Darjeeling, India; d – Courtesy: Michela Starace, Aurora
E • Extension – pigmentation of proximal and/
Alessandrini, and Bianca Maria Piraccini; e – Courtesy:
Dr. Anil Patki, Pune, India.) or lateral nail folds (Hutchinson’s sign)
F • Family history – previous melanoma or
dysplastic nevus syndrome
Longitudinal melanonychia
●● Longitudinal melanonychia appears as linear streaks or ●● Hutchinson’s sign should be differentiated from pseudo-
bands of brown-black pigmentation (Figure 20.30). Hutchinson’s sign, in which pigmentation of periungual
●● Nail melanocytes are quiescent and generally do not
tissue occurs due to conditions other than melanoma.
produce melanin. Nail pigmentation may occur as a ●● Dermoscopy picks up early pigmentation of the
result of activation of melanocytes (trauma- or drug- proximal nail fold and is referred to as “micro-
induced), nevus (benign proliferation of melanocytes), Hutchinson’s sign.”
or melanoma. Hence, pigmentation of the nail is a
warning and should be critically evaluated.
●● Clinical suspicion of nail unit melanoma (NUM) is
Alterations in lunula (Table 20.11)
raised by ABCDEF rule (Table 20.10).
Table 20.11  Lunula – change in color and shape

Changes in lunula Diseases

Red lunula (Figure 20.31a) Alopecia
Lichen planus
Blue lunula (Figure 20.31b) Raynaud’s disease
Macro Lunula Habit tic deformity
Triangular lunula (Figure 20.31c) Nail patella syndrome

Nail folds changes

Paronychia
●● Paronychia is inflammation of the nail folds, with

erythema, swelling, and pain. Two types are recognized

●● Acute paronychia is usually infective in nature
and is most commonly caused by Staphylococcus
aureus, followed by streptococci and pseudomonas
organisms. Other responsible agents include
gram-negative organisms, herpes simplex virus,
Figure 20.30  Longitudinal melanonychia affecting mul- and yeasts. Acute paronychia develops following
tiple nails in a young lady. trauma/injury to the nail folds and presents as
468 Nails

Figure 20.31  (a) Red lunula. (b) Blue lunula. (c) Triangular
lunula.

a painful, erythematous swollen nail fold with

collection of pus (Figure 20.32a).
●● Chronic paronychia (persisting more than six
weeks) is usually multi-factorial in origin and
presents as periodically inf lamed and painful
nail folds. The cuticle is usually lost, and in
long-standing cases, the nail plate becomes Figure 20.32  (a) Acute paronychia with pus formation.
dystrophic, with pronounced transverse ridges (b) Chronic paronychia. Note loss of cuticle. (c) Long
(Figure 20.32b,c). standing chronic paronychia with dystrophic nails.
 Nails 469

Figure 20.33  (a) Ingrown nails affecting both great toes. Note overgrowth of lateral nail folds. (b) Ingrown nail with serosangui-
nous discharge and swelling of lateral nail folds. (b – Courtesy: Dr. Dependra Kumar Timshina, Remedy Clinic, Siliguri, India.)

Ingrown nails/onychocryptosis folds (Figure 20.34). It is an important indicator of

●● In onychocryptosis, the lateral edges of nail plate penetrate subungual melanoma.
into the soft tissue of the lateral nail folds, causing
Pseudo-Hutchinson’s sign
periungual inflammation and pain (Figure 20.33a,b).
●● It refers to the pigmentation of nail folds (along with

nail-plate pigmentation) in non-melanoma conditions

Hutchinson’s sign (e.g., ethnic pigmentation, Laugier-Hunziker-Baran
●● Hutchinson’s sign refers to the periungual extension syndrome, Bowen’s disease, minocycline-induced
of brown-black pigmentation from longitudinal pigmentation, etc.) (Figure 20.35). In pseudo-
melanonychia onto the proximal and lateral nail Hutchinson’s sign, the cuticle remains translucent.

Figure 20.34  Subungual melanoma with pigment spread- Figure 20.35  Pseudo-Hutchinson’s sign in Laugier-
ing to surrounding tissue. Hunziker-Baran syndrome.
470 Nails

Figure 20.36  Subungual hyperkeratosis.

Subungal changes
●● Subungal hyperkeratosis results from accumulation of
keratin material under the nail plate as a result of nail bed
hyperkeratosis. As a result of subungual hyperkeratosis,
the nail plate loses its translucency and appears yellowish-
white in color (Figure 20.36). Common causes include
psoriasis, dermatophyte infection, and eczema.
●● Subungual hematoma is a transient condition
characterized by a painful collection of blood under the
nail plate and occurs as a result of traumatic injury to
the digit. The nail plate loses its translucency, and the
Figure 20.38  Onychoscopic view of distal splinter
color changes over time from the initial red-purple to hemorrhage.
dark brown and black as the blood clots (Figure 20.37).

Gradually, the color fades and the nail plate assumes its
normal appearance.
●● Splinter hemorrhage refers to a micro-hemorrhage
from nail bed capillaries due to local trauma or
microemboli. It presents as longitudinal 1- to
3-mm red, brown, or black lines under the nail
plate (Figure 20.38). It may be solitary or multiple,
and asymptomatic or tender. Splinter hemorrhage
may be caused by trauma (usually distal in
location) or may be seen in various dermatological
(psoriasis) and systemic (proximal in location,
infective endocarditis, meningococcal disease,
antiphospholipid syndrome, systemic lupus
erythematosus, Raynaud’s disease, and cutaneous
vasculitis) conditions.
●● Glomus tumor is a painful tumor of the nail unit
that usually affects the first and second finger
nails. The pain is disproportionate to the clinical
signs and is typically paroxysmal in nature, with
associated cold sensitivity. There is often a pinpoint
tenderness. Glomus tumor may be visible as a red-
purple subungual mass, while bigger masses may be
associated with onycholysis, nail plate thinning, and
Figure 20.37  Traumatic subungual hematoma of great toe. fissuring (Figure 20.39).
 Nails 471

Singal (Figures 20.10a, 20.10c, 20.10d, 20.11b, 20.13, 20.17d,

20.19c, 20.23, 20.28c, 20.28d, 20.28f, 20.28h, 20.28j, 20.31b,
20.34); Dr. Michela Starace (Figures 20.7, 20.18c, 20.29d,
20.31a, 20.31c); Prof. Balachandar Ankad (Figures 20.12,
20.18b, 20.24a, 20.28e), Prof. Bela J Shah (figure 20.14c); and
Prof. Eckart Haneke (Figure 20.29f) for their invaluable
contributions.

REFERENCES
1. de Berker D. Nail anatomy. Clin Dermatol 2013;31:509–515.
2. Lal NR, Kumar P, Barkat R. Nail Unit Signs. In: Singal A, Neema
S, Kumar P, editors. Nail Disorders: A Comprehensive Approach.
1st edition. New York: CRC Press/Taylor & Francis Group; 2019.
P. 21–48.
3. Ankad BS, Beergouder SA. Nail Plate Abnormalities. In: Singal
A, Neema S, Kumar P, editors. Nail Disorders: A Comprehensive
Approach. 1st edition. New York: CRC Press/Taylor & Francis
Group; 2019. P. 147–164.
4. Starace M, Alessandrini A, Piraccini BM. Chromonychia. In: Singal
A, Neema S, Kumar P, editors. Nail Disorders: A Comprehensive
Approach. 1st edition. New York: CRC Press/Taylor & Francis
Group; 2019. P. 165–175.
5. Rich P. Overview of Nail Disorders. In: Stratman E, Corona R, edi-
Figure 20.39  Large nail bed glomus tumor with nail
tors. UpToDate [Internet]. Waltham (MA): UpToDate Inc; 2019 [cited
plate splitting. (Courtesy: Soumyajit Roychoudhury, 2019 Dec 10]. Available from: https://www.uptodate.com/contents/
Behrampore, India.) overview-of-nail-disorders
6. Singal A, Arora R. Nail as a window of systemic diseases. Indian
ACKNOWLEDGMENT Dermatol Online J 2015;6:67–74.
7. Haber JS, Chairatchaneeboon M, Rubin AI. Trachyonychia: Review
The author would like to thank the editors and publisher and Update on Clinical aspects, histology, and therapy. Skin
(CRC Press) of the book Nail Disorders: A Comprehensive Appendage Disord 2017;2(3–4):109–115.
Approach for allowing the use of images and content from
the book. The author would like to thank Prof. Archana
   SECTION 4
Skin in systemic diseases

PC DAS, PIYUSH KUMAR

Skin is the interface between the internal milieu of the body have some form of skin involvement at some stage of the
and the environment. The health of an individual can be eas- disease process. Many of the systemic diseases – involving
ily judged by their skin, and it is rightly said to be the mirror systems ranging from the central nervous system to kidneys
of the human body. Hence, careful dermatological exami- and internal malignancies – have dermatological manifes-
nation can provide important clues to various systemic dis- tations that not only help in diagnosis of internal diseases
eases. Dermatologists need to develop a keen eye to look for but also cause suffering to patients and require treatment
and identify these clues. There are numerous dermatological in their own right. The treatment of systemic diseases also
signs that are harbingers, or telltale signs, of various systemic results in dermatologic toxicity, which requires expert man-
diseases. Some of these dermatological signs, such as pallor, agement by dermatologists.
jaundice, cyanosis and clubbing, are non-specific, but they Dermatologists should be aware of dermatologic mani-
are still useful as they always trigger a search for systemic festation of internal disease, dermatological manifestation
illness. Some other cutaneous signs are specific enough to of internal malignancy, and cutaneous toxicity of various
allow a clinical diagnosis. Some notable examples include drugs. In-depth knowledge of cutaneous manifestations
pretibial myxoedema in Graves’ disease, Gottron papules in and a high index of suspicion can place dermatologists in a
dermatomyositis, hypertrichosis lanuginosa acquisita, and unique position where they can diagnose systemic diseases
erythema gyratum repens in internal malignancy, etc. early and prevent needless suffering. The ensuing few chap-
Most of the systemic diseases, be they infectious, meta- ters emphasize the dermatological presentations of systemic
bolic, genetic, neoplastic, autoimmune, or environmental, diseases.

DOI: 10.1201/9781351054225-56
 21
Nutritional deficiency disorders

CHIRAG DESAI, PIYUSH KUMAR

INTRODUCTION mucocutaneous lesions consistent with nutritional disor-

ders (vide Table 21.2).1–4 Some of the nutrient deficiency
The human body requires many nutrients (Table 21.1) states do not have a prominent cutaneous involvement
on a regular basis for a variety of biological functions. (e.g., thiamine deficiency causing beri beri) and will not
Though many or all organ systems are affected when one be discussed here.
or more nutrients are deficient, the skin (and its append-
ages) and mucosa are affected early in most cases. These Atrophic glossitis
skin and mucosal changes are “tell-tale” signs of nutri- ●● Atrophic glossitis (AG) refers to the absence of
tional deficiency, and some of them are specific enough filiform or fungiform papillae on the dorsal surface
to allow a clinical diagnosis of deficiency of a particular of the tongue, causing a soft and smooth appearance
nutrient. Thus, recognition of mucocutaneous, hair, and (Figure 21.1a).
nail changes can be an important tool for diagnosing vari- ●● This change is non-specific and may be seen in the

ous nutritional deficiencies. This chapter discusses clini- deficiency of various nutrients, usually several of them
cal diagnosis of various nutritional deficiencies based on rather than one at a time. Deficiency of riboflavin
presentation (Table 21.2) and other clues (Table 21.3). (vitamin B2), niacin (vitamin B3), pyridoxine (vitamin
As the causative factors responsible for various nutrient B6), folate, cobalamin (vitamin B12), iron, and zinc may
deficiencies are common (Box 21.1), multiple nutritional cause atrophic glossitis.
deficiencies tend to co-exist. This should be kept in mind ●● Affected patients complain of pain and a burning
while dealing with a patient with suspected nutritional sensation.
deficiency. Also, these dermatoses mimic many common ●● Clinically, the tongue appears beefy red with a smooth,
dermatoses and should be considered as clinical differ- glossy surface. Angular cheilitis is frequently associated.
entials, especially in people who are at risk of developing ●● DD: Candidiasis, lichen planus, median rhomboid

nutritional deficiencies (vide Box 21.1) and who develop glossitis, migratory glossitis.

Table 21.1  Different nutrients required for humans

Macronutrients Energy-yielding • Carbohydrates

• Proteins
• Fats (saturated fats, monounsaturated fats,
polyunsaturated fats, essential fatty acids)
Non-energy–yielding • Water
• Fiber
Micronutrients Vitamins • Water soluble – vitamins B complex and vitamin C
(all are non-energy– • Fat soluble – vitamins A, D, E and K
yielding) Minerals Sodium, potassium, calcium, magnesium, chloride
and phosphorus
Trace elements Zinc, selenium, molybdenum, chromium, copper,
iodine, iron, manganese and fluorine

DOI: 10.1201/9781351054225-57 473

474  Nutritional deficiency disorders

Table 21.2  Clinical approach to dermatoses seen in various nutritional disorders

Nutrient excess (toxicity)/

Presenting feature Deficient nutrient/condition condition
Glossitis (red) Niacin, pyridoxine, riboflavin, vitamin B12, folate, vitamin B1,
biotin, iron
Angular stomatitis Riboflavin, niacin, pyridoxine, folate, vitamin B12, Iron, zinc, marasmus Vitamin A
Xerosis Vitamin A, essential fatty acids, Hematochromatosis, selenium
Scaling/exfoliation • Generalized – PEM, vitamin A Vitamin A
• Acral – niacin
• Scalp – biotin (early),
• Periorificial – biotin (early)
Follicular papules Vitamin A, vitamin C, EFA, copper Vitamin A
Skin and mucosa; Vitamin B12 (Addisonian pattern), folate, vitamin A (face and Hemochromatosis, vitamin A
hyperpigmentation neck), kwashiorkor (extremities)
Hypopigmentation of skin Copper, selenium
Eczematous changes • Acral areas – zinc, biotin • Scalp – selenium toxicity
• Seborrheic areas – niacin, riboflavin, pyridoxine • Genitals – selenium
• Periorificial areas – zinc, biotin toxicity
• Intertriginous areas – biotin
• Sun exposed areas – niacin
Purpuric lesions Vitamin C (perifollicular petechiae), vitamin K, Essential fatty Vitamin E
acids (traumatic)
Edema Protein (generalized), thiamine (pedal edema), vitamin C
(“woody edema” of lower legs), niacin (sunburn like), iron
Photosensitivity Niacin Pyridoxine (acral blistering)
Yellowish discoloration β-carotene

Table 21.3  Clues for the diagnosis of nutritional disorders.

Signs Deficient nutrient

Pallor Iron, folate, vitamin B12, copper
Atrophic lingual papillae Niacin, iron, riboflavin, folate, vitamin B12, pyridoxine
Transversely depigmented hairs PEM (flag sign), copper, EFA
Easily pluckable hairs PEM
Sparse, thin hairs PEM, zinc, biotin, vitamin A
Corkscrew hairs (flattened and curled) Vitamin C
Koilonychia Iron
Beau’s lines PEM
Brittle nails EFA, Iron, (selenium excess)
Bleeding gums Vitamin C, riboflavin, Vitamin K
Reduced taste sensation Vitamin A, zinc
Muscle pain Vitamin C, thiamine, selenium
Muscle wasting PEM
Bone tenderness Vitamin C, vitamin D, calcium, phosphorus
Delayed wound healing Zinc, PEM, vitamin C
Genital lesions Zinc, riboflavin, vitamin B3, folate
Night blindness Vitamin A
Conjunctival erythema and Riboflavin
Corneal vascularization
Conjunctival xerosis Vitamin A
Hemorrhagic disease of the Vitamin K
newborn (HDN)
Peripheral neuropathy Vitamin B1, pyridoxine (both excess and deficiency), Vitamin B12, Zinc
Abbreviations: EFA, essential fatty acids; PEM, protein energy malnutrition.
 Nutritional deficiency disorders  475

BOX 21.1: Factors responsible for deficiency of nutrients

●● Inadequate intake (poverty, old age, alcoholics, anorexics, people with psychiatric morbidities, etc.)
●● Impaired absorption (inflammatory bowel disease, celiac disease and tropical sprue, short bowel syndrome, etc.)
●● Impaired metabolism
●● Increased requirement (infancy and adolescence, pregnancy, lactation, concurrent infection, malignancy)
●● Increased excretion
●● Increased destruction

Angular cheilitis (AC) (Angular stomatitis, perlèche/ mouth (Figure 21.1b). AC due to nutrient deficiency is
perlèche) associated with atrophic glossitis.
●● Angular cheilitis is an inflammatory condition that
Riboflavin (vitamin B2) deficiency (oral-ocular-genital
affects either or both angles of the mouth.
●● AC may develop due to inadequate dentures,
syndrome)
●● Risk factors – pregnancy, lactation, phototherapy
loss of vertical dimension of the mouth in older
people, contact allergy, nutritional deficiencies, for hyperbilirubinemia (in premature infants),
hypersalivation, marionette lines, and atopic advanced age, low income, depression, alcoholics,
or seborrheic dermatitis. Candida albicans, hypothyroidism, and phenytoin therapy.
●● The clinical findings include the following:
Staphylococcus aureus, and β-hemolytic streptococci
infection too may cause AC. ●● Oral – tongue (atrophic and magenta in color) and
●● Patients may complain of discomfort, pain while lips (chapping and fissuring of the lips, angular
opening the mouth, and a burning sensation. cheilitis) involvement.
●● AC typically presents with erythema, painful cracking,
●● Ocular – photophobia and blepharitis angularis;
scaling, bleeding, and ulceration at the corners of the cataracts may develop.

Figure 21.1  (a) Loss of papilla in the central part of the tongue. (b) Erosion and crusting at the angle of mouth in angular cheilitis.
476  Nutritional deficiency disorders

BOX 21.2: Genital involvement in riboflavin deficiency

●● Mild: Pruritic dermatitis on sweating

●● Moderate: Acute dry phase with erythema, confluent red papule that spreads to involve the perianal area and inner
thighs
●● Severe: Balanitis and phimosis

●● Genital – males affected more than females; clinical AG, AC, and dry, chapped lips are commonly associated
findings vary according to the severity of the findings. Eyes may show xerophthalmia.
condition (Box 21.2).
●● Cutaneous features include seborrheic dermatitis-like Vitamin A deficiency
changes and dyssebacia. ●● Risk factors include persons with fat malabsorption,

●● Extracutaneous findings include anemia, peripheral cystic fibrosis, pancreatic insufficiency, cholestasis,
neuropathy, depression. people with small-bowel bypass surgery, vegans,
●● DD: Erythema multiforme, fixed drug eruption, zinc refugees, recent immigrants, persons with alcoholism,
deficiency. preschool children with low socioeconomic status.
●● The clinical findings include diffuse dryness of skin
Protein energy malnutrition (PEM) with pruritus and fine scaling, phrynoderma, dry hair
●● PEM is a group of malnutrition disorders that include with hyperpigmentation of the hair cast, increased nail
marasmus, kwashiorkor, and marasmic kwashiorkor fragility, and keratinization of the mucosa.
(having features of both). ●● Phrynoderma presents as asymptomatic or mildly

●● Marasmus is primarily caused by a deficiency in calories pruritic, discrete but grouped follicular papules of
and energy, whereas kwashiorkor is thought to result various sizes with central keratotic plugs that block the
from protein deficiency. Protein deficiency results in follicle openings. The lesions usually appear first on the
characteristic edematous appearance of kwashiorkor
and is absent in marasmus.
●● PEM is common in the developing countries of Asia Table 21.4  Protein energy malnutrition: kwashiorkor and
and Africa, and is mostly seen in children and older marasmus
population. Features Kwashiorkor Marasmus
●● Predisposed groups include children from low

socioeconomic status, adults with HIV, alcohol and Age group 6 months–5 years Less than 1 year
substance abuse, history of gastric bypass surgery, and Predominant Protein deficiency > Energy
anorexia nervosa. etiology Energy deficiency deficiency
●● In marasmus, the child appears lethargic and cachetic, Dermatosis, Flaky-paint dermatosis, Wrinkled, dry
with marked loss of subcutaneous fat and muscle flaky-paint crazy pavement skin
wasting. Patients have dry, wrinkled, loose skin and dermatitis
have a “Monkey facies” due to loss of buccal fat. Adults Hair changes Common, “Flag sign” Less common
with marasmus may develop additional follicular and “salt and
hyperkeratosis and folliculitis. pepper” appearances
●● In kwashiorkor, the child appears edematous, with Subcutaneous Reduced Extreme loss
a moon facies and a swollen abdomen. The typical fat
skin change is termed “crazy pavement dermatosis, or Pitting edema Present on extremities Absent
mosaic skin” – the skin becomes pigmented and dry, and face
and develops irregular fissures revealing pale areas. The Face May be edematous, Drawn in,
fragile skin peels away in large sheets in an irregular moon face monkey-like
pattern (flaking paint appearance). These changes are Growth Present (–2 SD) Present (–3 SD)
characteristically noted over friction- and trauma-prone retardation
areas. Other cutaneous findings include erythema, Wasting Present but not severe Severe
thinning, petechiae, ecchymoses, and purpura. The Appetite Poor Usually good
child is often irritable (Table 21.4, Figure 21.2a–d).
Mental Very common Uncommon
●● In PEM, hairs lose pigmentation and become dry,
changes (irritability, apathy)
lusterless, sparse, and brittle. Also, they can be pulled
Anemia Severe (sometimes) Present, less
out easily. In patients who have periods of adequate
severe
nutrition interspersed with malnutrition, hairs may
Response to Relatively faster Slower
show alternating dark and pale areas (flag sign). Nail
treatment
plates become thin and soft and may show Beau’s lines.
 Nutritional deficiency disorders  477

Figure 21.2  (a) Generalized exfoliation in a boy with bulging belly, and edema of hands. (b) Red-brown areas of epidermal
damage and exfoliation in the same patient (Figure 21.2a). (c) Skin exfoliation as flaky paint dermatosis and erosions on
the legs. (d) Close-up of the lesions. (e) Follicular papules of phrynoderma on the elbow. (f) Bitot’s spots. (e – Courtesy:
Dr. Anup Kumar Tiwary, Consultant Dermatologist, Yashoda Hospital and Research Center, Ghaziabad, India; f – Courtesy:
Dr. Pradeep Kumar Jha, Consultant Ophthalmologist, Darbhanga, India.)
478  Nutritional deficiency disorders

BOX 21.3: Bitot’s spots

●● Bitot’s spots are slightly elevated, white lesions seen on the bulbar conjunctiva near the limbus, at the three o’clock
or nine o’clock positions. Bitot’s spots are more common on the temporal side (Figure 21.2f).
●● It does not disappear completely, even after the treatment of vitamin A deficiency. Hence, it is not a sign of active
VAD.

back of the elbows (Figure 21.2e) and the front of the Vitamin B12 deficiency
knees, and can spread to involve the extremities, upper ●● Risk factors include strict vegetarianism and veganism,
forearms, and thighs. Occasionally the abdomen, back, pernicious anemia, malabsorption, atrophic gastritis,
and buttocks may be affected. The face is rarely affected, surgical resection of the terminal ileum, celiac disease,
and hands and feet are spared. bariatric surgeries, long-term use of drugs such as
●● It may be seen in the deficiency of vitamin A metformin, proton-pump inhibitors, and gastric acid-
(commonly), vitamin E and essential fatty acids. blocking agents, recreational nitrous oxide use.
●● DD: Keratosis pilaris, lichen spinulosus. ●● The clinical findings include atrophic glossitis (Hunter’s
●● Ocular findings include poor adaptation to darkness glossitis), angular cheilitis, lemon-yellow waxy
(nyctalopia), xerophthalmia, Bitot’s spots (Box 21.3), pallor, hyperpigmentation especially in dark-skinned
keratomalacia, corneal perforation, and blindness. patients, and brittle hair with premature whitening
(Figure 21.3a,b).
Essential fatty acids deficiency ●● Hyperpigmentation is usually generalized with
●● Essential fatty acids (EFA), linoleic acid (LA) and alpha-
accentuation in flexural areas, palms, and soles, and in
linolenic acid (ALA), deficiency is rare and is usually areas of pressure, such as the terminal phalanges, knees,
seen in infants and children with low EFA in their diet. and elbows. Mucosal and nail pigmentation too may be
EFA deficiency is frequently seen with PEM. observed (Figure 21.3c,d).
●● Other risk factors include malabsorption states, ●● Extracutaneous findings include anemia (macrocytic
pancreatic insufficiency, bariatric surgery, long-term type), neurological findings (Box 21.4) (peripheral
parenteral nutrition, and extreme dietary fat restriction. nervous system and spinal cord involvement),
●● Skin is dry and scaly with some degree of erythema.
psychiatric manifestations (major depressive disorder,
Intertriginous areas may develop erosions. Hairs may be personality changes and psychosis, mild cognitive
less pigmented or alopecia may develop. impairment or dementia), optic nerve atrophy.
●● Systemic features include growth failure, poor wound ●● Subacute combined degeneration of the spinal cord
healing, increased capillary fragility, and increased (Lichtheim’s disease) characterized by patchy losses of
susceptibility to infections. myelin in the dorsal and lateral columns may develop in
●● DD: PEM, vitamin A deficiency, atopic dermatitis.
untreated cases.

Hypervitaminosis A Folate deficiency

●● Risk factors inclute hepatic and renal compromise, ●● Folate and vitamin B play their roles in a common
12
excessive intake of preformed vitamin A. biochemical pathway, homocysteine remethylation, and
●● Patients complain of joint and bone pain and headache. hence the clinical picture of their deficiency states is
●● Patients develop loss of hair and coarseness of overlapping.
remaining hair, loss of eyebrows, exfoliative cheilitis, ●● Risk factors include infancy, pregnancy, lactation,

fissuring of corner of mouth and nostrils, generalized old age, alcoholism, smoking, renal and liver
exfoliation, pigmentation of face (and neck), and disease, malabsorption states, folate antimetabolites
follicular keratotic papules. (methotrexate and trimethoprim), malignancy, vitamin
●● Chronic cases may develop clubbing. B12 deficiency (increased urinary loss of folate).

BOX 21.4: Neurological manifestations in vitamin B12 deficiency

1. Sensory impairment in gloves-and-stocking pattern

2. Diminished vibration sense and proprioception in the legs, varying degrees of ataxia, positive Romberg sign
3. Deep tendon reflexes (DTRs) that may be exaggerated, diminished, or absent
4. Motor involvement and spastic paraparesis autonomic bladder, bowel, or sexual symptoms
5. Positive Babinski sign
 Nutritional deficiency disorders  479

Figure 21.3  (a) Atrophic glossitis and angular cheilitis in

vitamin B12 deficiency. (b) Diffuse pigmentation of both
palms in vitamin B12 deficiency. (c) Vitamin B12 deficiency in
two sisters with diffuse pigmentation of palms. (d) Same
patients (Figure 21.3c) with increased pigmentation over
the knuckles. (c – Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India; d – Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India.)

●● Patchy hyperpigmentation of the skin and mucous initially. Angular stomatitis is often co-existing.
membranes is present, particularly at the dorsal Perineal eczematous lesions and ulcerations may
surfaces of the fingers, toes, and creases of palms and develop.
soles. ●● Systemic features include gastrointestinal (anorexia,
●● Patients may complain of unexplained temperature nausea, vomiting, abdominal pain, and diarrhea after
elevation (< 102°F) and significant weight loss. meals) and neuropsychiatric (cognitive impairment,
●● The tongue is swollen and sore and appears beefy- dementia, and depression) manifestations.
red or shiny, usually around the edges and tips ●● DD: Vitamin B12 deficiency, Addison’s disease.
480  Nutritional deficiency disorders

Hemochromatosis Hypozincemia (Zinc deficiency)5

●● Hereditary hemochromatosis (HH) is an adult-onset ●● Acquired form is mostly seen where there

disorder characterized by inappropriately high iron are predisposing factors such as pregnancy,
absorption resulting in progressive iron overload, lactation, extensive cutaneous burns, generalized
causing dysfunction of certain organs – the liver, heart, exfoliative dermatoses, food faddism, parenteral
pancreas, pituitary, joints, and skin. nutrition, anorexia nervosa, intestinal
●● HH shows a clear male predilection. In addition, malabsorption syndromes (inflammatory
males present earlier and develop more severe bowel disease), cystic fibrosis, alcoholism, HIV
disease. infection, malignancy, uremia, and chronic renal
●● The cutaneous hallmark is skin hyperpigmentation, disease.
which starts early in the disease and may be a ●● The inherited form (acrodermatitis enteropathica, AE)

presenting feature. Patients develop a diffuse is mostly seen in children.

brownish-bronze or, at times, slate-gray ●● AE typically presents at weaning, with a clinical

pigmentation pronounced over sun-exposed areas triad of dermatitis, diarrhea, and alopecia.
like the face. Hyperpigmentation may affect mucosa, Initially, the patients develop erythematous,
teeth, and scars too. dry, and scaly patches (scald-like appearance)
●● Other cutaneous findings include ichthyosiform around orifices (perioral and perianal area) and
changes (over sun-exposed areas), skin atrophy, over trauma-prone sites (hands and feet). If left
koilonychia, and hair loss (including body hair and untreated, lesions progress to vesiculobullous,
pubic hair). erosive, and crusted lesions. Mucosal findings
●● Extracutaneous findings include liver disease include angular cheilitis, and glossitis. Patients may
(hepatomegaly, and cirrhosis), diabetes mellitus, develop paronychia and nail dystrophy, and loss of
arthropathy (most commonly affected joints are MCP scalp hair, eyebrows, and eyelashes in severe cases.
joints, proximal interphalangeal joints, knees, and Children are usually irritable and inconsolable
wrists), cardiomyopathy, amenorrhea, impotence, (Figure 21.4a–c).
hypogonadism. ●● Ocular involvement is common and manifests as
●● DD: Drug-induced pigmentation, actinic reticuloid, conjunctivitis, blepharitis, punctate keratopathy,
beta thalassemia. and photophobia.

Figure 21.4  (a) Scald-like erythema and scaling of the buttocks

in acrodermatitis enteropathica. (b) Perioral crusted lesions on an
erythematous skin. (Continued)
 Nutritional deficiency disorders  481

Figure 21.4 (Continued)  (c) Dusky erythematous patches

with central erosions on the foot. (d) Erythematous, scaly and
crusted plaques over trauma prone areas in a case of hypoz-
incemia due to Nephrotic syndrome. (e) Close up of lesions
over upper thigh- central area heal while the peripheral area
shows erythema, scaling and crusting. Without treatment
central area, once healed, develops erythematous crusted
lesions again. (f) Scaly crusted lesions on the buttock and
lower extremities in a chronic alcoholic patient. In alcoholic
patients, multiple nutritional deficiencies often co-exist and
hence, dermatoses cannot be attributed to the deficiency of
any one nutrient. (g) close up of lesions. (f,g – Courtesy:
Dr. Deverashetti Srinivas, Nizamabad, India.)
482  Nutritional deficiency disorders

●● The skin changes in acquired form are usually similar tryptophan and niacin. Hence, patients with prolonged
but milder and of slower onset. The patients develop pyridoxine deficiency invariably develop features of
eczematous scaly plaques that can develop into vesicles, pellagra.
bullae, or pustules. Periorificial and acral trauma-prone ●● DD: Pellagra, folate deficiency, isoniazid toxicity.
areas are typically affected (Figure 21.4d–g).6
●● Long-standing cases may show frequent infections, Pellagra (Vitamin B3 or Niacin deficiency)
delayed wound healing, growth retardation, anorexia, ●● Risk factors include alcohol abuse; diet composed

anemia, photophobia, hypogonadism, delayed puberty, mainly of corn, millet, or sorghum; carcinoid tumors;
and altered mental status. Hartnup disease; Crohn’s disease; anorexia nervosa,
●● DD: Biotin deficiency, essential fatty acid (EFA) restricted diet in atopic dermatitis for food allergy;
deficiency, vitamin B2 (riboflavin) deficiency, necrolytic drugs (isoniazid, 5-fluorouracil).
migratory erythema (NME), pseudo glucagonoma ●● The clinical presentation encompasses four Ds:

syndrome. diarrhea, photosensitive dermatitis, and dementia.

Death (a fourth D) occurs in untreated cases.
Biotin deficiency ●● Cutaneous lesions are typically present on photo-

●● Nutritional deficiency of biotin is uncommon. The exposed areas. The dorsum of the hands with lesions
clinical manifestations of biotin deficiency result extending over forearms (glove or gauntlet pattern),
more commonly from the deficiencies of enzymes feet (usually not extending proximal to malleoli-
involved in biotin homeostasis (e.g., biotinidase boot pattern), neck (casal necklace – broad collar-
deficiency). like lesions in cervical dermatomes with C3 and C4
●● Risk factors include chronic alcoholism, smoking, innervation, sometimes with a cravat-like extension
inflammatory bowel disease, long-term anticonvulsants anteriorly over the sternum to the level of the nipples),
therapy, and consumption of excessive amounts of raw and face (in distribution of the trigeminal nerve)
eggs. are most commonly affected. Pressure-prone areas
●● Cutaneous findings include dry skin, seborrheic like shoulders, elbows, forearms, and knees may be
dermatitis-like eruptions and erythematous eczematous involved in some cases. The symmetry and the line
rash in periorofacial locations. Hairs become fine and of demarcation from unaffected skin are particularly
brittle, and total alopecia may develop. Nails may be striking.
brittle. ●● The early skin lesions mimic sunburn. The lesions

●● Patients may exhibit increased susceptibility of fungal appear as bilaterally symmetrical erythematous,
infections, especially candidiasis. edematous lesions on photo-exposed areas. Severe cases
●● Prominent systemic symptoms include neurological may develop blisters that may rupture, leaving large
findings (developmental delay, hypotonia, seizures, denuded areas. The lesions eventually heal with a dusky,
progressive spastic paresis, myelopathy, encephalopathy, brown-red pigmentation. Additionally, patients may
ataxia, etc.), gastrointestinal features (nausea, anorexia), develop erythematous, scaly lesions on trauma- and
and sensorineural hearing loss. friction-prone areas (Figure 21.5a–d).
●● DD: Zinc deficiency. ●● The late lesions are characterized by thickened,

pigmented skin with a dry, scaly, and fissured surface

Pyridoxine (vitamin B6) deficiency (parchment-like appearance). Blisters and other acute
●● Risk factors include old age, cirrhosis, chronic renal changes may recur. Healing takes place in a centrifugal
failure, hemodialysis, peritoneal dialysis, phototherapy manner.
for hyperbilirubinemia, drugs (hydralazine, isoniazid, ●● The tongue may show changes, such as

D-penicillamine, pyrazinamide), alcoholism, tobacco pseudomembranous furs, erosions, and ulcers, but
smoking, and pregnancy. later becomes atrophic. Other findings include angular
●● The clinical findings include stomatitis, atrophic stomatitis and bleeding from the gingiva. The lips may
glossitis, angular cheilitis, and seborrheic dermatitis- be inflamed, chapped, or fissured. Genitalia may show
like eruption. erosions and ulcerations.
●● Extracutaneous findings include neurological ●● Gastrointestinal involvement results in diarrhea, loss

findings (distal-limb numbness and weakness, of appetite, nausea, vomiting, epigastric discomfort,
impaired vibration and proprioception, preserved abdominal pain, and increased salivation.
pain and temperature, sensory ataxia, generalized ●● Neuropsychiatric manifestations include headache,

seizures), anemia, cardiovascular (atherosclerosis, irritability or apathy, anxiety, delusions, hallucinations,

early myocardial infarction, recurrent venous stupor, photophobia, tremor, ataxia, spastic paresis,
thromboembolism), psychiatric (depression). fatigue, depression and dementia.
●● Secondary niacin deficiency can result from a deficiency ●● DD: Phototoxic reaction, zinc deficiency, Hartnup

of pyridoxine, which is essential for metabolism of syndrome, porphyria cutanea tarda.

 Nutritional deficiency disorders  483

Figure 21.5  (a) Bilateral, symmetrical involvement of extensor forearms and dorsum of hands in pellagra. (b) Bilaterally
symmetrical erythematous, scaly patch in pellagra palms. (c) Erythematous patch with crusting at places. (d) A severe
case of pellagra with extensive involvement of trunk and upper extremities. (a – Courtesy: Dr. Dependra Kumar Timshina,
Remedy Skin Clinic, Siliguri, India; b – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India; c – Courtesy: Dr. Hiral Shah,
Baroda Medical College, Vadodara, India; d – Courtesy: Dr. Santoshdev Rathod, SVPIMSR Smt. NHL Municipal Medical
College, V.S. Hospital, Ahmedabad, India.)

Vitamin K deficiency massive transfusion, chronic kidney disease, and

●● Risk factors include neonates, malabsorption hemodialysis.
(biliary cirrhosis, cystic fibrosis, malabsorption ●● Vitamin K has its major roles in coagulation, bone
syndromes), anorexia nervosa, drugs (coumarins, development, and cardiovascular health.
salicylates, cephalosporin, cholestyramine, warfarin, ●● There is often a history of profuse bleeding on trivial
salicylates, anticonvulsants), chronic illness, trauma, epistaxis, hematoma, gastrointestinal bleeding,
malnutrition, alcoholism, parenchymal liver disease, menorrhagia, hematuria, and gum bleeding.
484  Nutritional deficiency disorders

●● Petechiae and ecchymosis, hematomas, and/or oozing of ●● Other important cutaneous findings include alopecia,
blood at surgical and puncture sites may be evident on increased capillary fragility, poor wound healing, and
clinical examination. breakdown of old scars.
●● Infants may develop intracranial bleeding signs – ●● Extracutaneous findings include hypotension,
vomiting, poor intake, and seizures. hemopericardium (may cause sudden death),
●● DD: von Willebrand disease, deficiency of various intraocular and retrobulbar bleeding, spongy
clotting factors, scurvy, immune thrombocytopenia friable gum with bluish-purple hue, and loose teeth.
(ITP) and thrombotic thrombocytopenic purpura Scorbutic rosary (beading at the costochondral
(TTP). junctions) and sternum depression are other
characteristic bony features.
Scurvy
●● Predisposed groups include alcoholics, psychiatric

patients with restricted diet, patients on dialysis,

people who eat primarily at fast food restaurants,
cigarette smokers, refugees and people of low
socioeconomic status, pregnant and lactating women,
people with type 1 diabetes or with disease of the
small intestine.
●● The cardinal clinical features consist of four Hs –

hemorrhage, hyperkeratosis, hypochondriasis, and

hematologic abnormalities.7,8
●● Hemorrhages: The bleeding may be superficial
(typically perifollicular in location and affects the
legs and buttocks where hydrostatic pressure is the
greatest) or deep (subcutaneous bleeds presenting
as nodules). Spontaneous bleeding may occur from
the gums or may be subconjunctival, intra-articular,
subungual, and intramuscular hemorrhages.
Periosteal hemorrhages in long bones are very
painful and cause pseudoparalysis. Affected babies
present in a typical immobilized posture with
semiflexion of the hips and the knees (“pithed
frog”).
●● Hyperkeratotic papules: Perifollicular keratotic
follicular papules and curling of central hairs
(corkscrew like hair) are other characteristic
cutaneous findings (Figure 21.6a,b).
●● Hypochondriasis.
●● Haematological abnormalities: anemia.

Figure 21.6  (a) Perifollicular petechiae in scurvy. (b) Perifollicular hemorrhagic lesions on leg of 17-year-old
girl. Note twisted broken “corkscrew hairs” (arrow). (a – Courtesy: Dr. Carolina Rincon-Cordoba and Prof Pablo
Fernandez-Penas, Westmead Hospital, Sydney; b – With permission from Journal of Pakistan Association of
Dermatologists 2011; 21 (3): 202–206.)
 Nutritional deficiency disorders  485

●● DD: Child abuse. bleeding disorders, hematologic

malignancies.

Carotenemia and Carotenoderma

●● Risk factors include excessive ingestion (mango, orange,

papaya, carrots, green beans, asparagus, broccoli,

cucumber, butter, eggs, milk, etc.), hypothyroidism,
diabetes mellitus, pancreatic insufficiency, hepatic
disorders, anorexia nervosa, and renal diseases
(nephrotic syndrome).
●● Patients develop yellow-orange coloration of the

skin (carotenoderma), particularly noticeable in

palms, soles, forehead, the tip of the nose, and
nasolabial folds. Mucosa and sclera are typically
spared (Figure 21.7).
●● Female patients may develop amenorrhea.

●● DD: Jaundice (sclera is involved).

Figure 21.7  Orange palm in carotenemia. (Courtesy: Dr.
Carolina Rincon-Cordoba and Prof Pablo Fernandez-
Minerals Penas, Westmead Hospital, Sydney.)
Minerals of dermatological significance have been summa-
rized in Table 21.5.
Table 21.5  Minerals of dermatological importance

Mucocutaneous Hair and nail

Mineral Risk groups features finding Systemic findings
Zinc Risk group – Premature • Mucosa – angular • Hair – alopecia, • Gastrointestinal – diarrhea,
infants cheilitis, stomatitis • Nails – nail dysgeusia
• Inherited (Onset – • Cutaneous – dystrophy, • Neuropsychiatric – irritability,
days to weeks after impaired wound paronychia emotional, apathetic, listless
birth if bottle-fed, healing, secondary • Ocular – photophobia,
or after weaning if infection (Candida blepharitis, conjunctivitis,
breastfed) albicans, abnormal dark adaptation
• Acquired – Low zinc Staphylococcus • Others – growth retardation,
level in mothers aureus), periorificial hypogonadism in males, fatal if
milk, alcohol, and acral dermatitis, untreated
malabsorption, psoriasiform
inflammatory bowel dermatitis on
disease, HIV extremities
infection, anorexia
nervosa,
medications like
penicillamine,
diuretics, valproate
Selenium Patients receiving total Keshan disease Keshan disease Keshan disease
parenteral nutrition, (selenium deficiency) • Hair – • Cardiovascular system –
low soil content, • Cutaneous – hypopigmentation myocarditis, cardiomyopathy,
low-birth-weight hypopigmentation of the hair cardiomegaly, chronic
infants of skin congestive heart failure
Thinning and curling
• Musculoskeletal – muscular pain
(pseudo-albinism)
and weakness
Nails – white nail
• Gastrointestinal tract – hepatic
beds, terry-like
congestion, liver necrosis
nails, Leukonychia
mesenteric lymphadenosis
• Hematology – erythrocyte
macrocytosis
(Continued)
486  Nutritional deficiency disorders

Table 21.5  Minerals of dermatological importance (Continued)

Mucocutaneous Hair and nail

Mineral Risk groups features finding Systemic findings
Selenium toxicity Selenium toxicity Selenium toxicity:
• Cutaneous – • Hair – dry, brittle • Neuropsychiatric – depression
reddish hue, rapid loss of hair dizziness, peripheral
swelling, blisters, • Nails – brittle with anesthesia hyperreflexia,
occasionally ulcer white horizontal seizures
which heal slowly streaking on the • Gastrointestinal tract –
surface, nail loss nausea, vomiting,
diarrhea hypersalivation,
garlic breath odor,
hemorrhagic gastritis
• Kidney – acute tubular
necrosis
Copper Risk group – infants • Cutaneous – • Hair – • Musculoskeletal – osteoporosis,
• Causes – low hypopigmentation hypopigmentation fracture, periosteal reaction,
copper level in of skin of hair • Myeloneuropathy
milk/diet, protein • Ocular – permanent vision loss
energy malnutrition
(PEM) and
excessive zinc
intake

Menkes disease Menkes disease Menkes disease Menkes disease

(kinky-hair disease) – • Cutaneous – • Hair – alopecia • Musculoskeletal – depressed
infants after 2–3 follicular with hair-shaft nasal bridge, bony abnormalities
months hyperkeratosis, soft abnormalities like osteoporosis, diaphyseal
inelastic including pili torti, periosteal reaction, subperiosteal
depigmented skin monilethrix, and new bone formation, increased
at the nape of trichorrhexis risk of fracture
neck, pudgy • Horizontal • Ocular – ptosis
cheeks, a Cupid’s eyebrows • Central nervous system –
bow of the upper growth retardation, lethargy,
lip hypothermia, seizures,
developmental delay, failure to
thrive
• Hematology – anemia,
• Renal – hydronephrosis,
• Others – high-arched
palate, delayed tooth
eruption, reduced facial
movements
Iron Active menstruating • Mucosa – glossitis, • Hair – telogen • Ocular – blue sclerae
women, vegetarians angular cheilitis, effluvium,
aphthous ulcer lusterless dry
• Cutaneous – focally narrow and
pruritis split hair shaft
• Nails –
koilonychia,
fragile
longitudinal
ridging, brittle
nails
(Continued)
 Nutritional deficiency disorders  487

Table 21.5  Minerals of dermatological importance (Continued)

Mucocutaneous Hair and nail

Mineral Risk groups features finding Systemic findings
Plummer-Vinson Plummer-Vinson Plummer-Vinson syndrome
syndrome syndrome • Hematology – microcytic
• Mucosa – glossitis, • Nails – koilonychia anemia
smooth atrophy of • Orogastric – dysphasia
tongue, thin lip,
reduced mouth
opening
• Cutaneous – dry
and wrinkled skin

Iron overload- Iron overload

• Cutaneous – • Cardiovascular
bronze system-Cardiomyopathy
pigmentation of • Liver – cirrhosis
the skin, ichthyosis • Endocrinology – diabetes
mellitus

REFERENCES 5. Kumar P, Lal NR, Mondal AK, Mondal A, Gharami RC, Maiti A. Zinc
and skin: A brief summary. Dermatol Online J 2012 Mar 15;18(3):1.
1. Galimberti F, Mesinkovska NA. Skin findings associated with nutri- 6. Kumar P, Anand V, Mallik SK. Hyperkeratotic scaly lesions. Indian
tional deficiencies. Cleve Clin J Med 2016;83(10):731–739. Pediatr 2012;49: 935.
2. Heath ML, Sidbury R. Cutaneous manifestations of nutritional 7. Kumar P, Debbarman P, Mondal AK, Lal NR, Mondal A, Gharami
deficiency. Curr Opin Pediatr 2006 Aug;18(4):417–422. RC, Maiti A. Perifollicular hemorrhagic lesions and broken twisted
3. Lekwuttikarn R, Teng JMC. Cutaneous manifestations of nutritional hairs on legs. J Pak Assoc Dermatol 2011;21(3):202–206.
deficiency. Curr Opin Pediatr 2018 Aug;30(4):505–513. 8. Tiwary AK, Kumar P. Nutritional Disorders of Skin. In: Lahiri K,
4. Jen M, Yan AC. Syndromes associated with nutritional deficiency De A, editors. Postgraduate Dermatology. New Delhi: Jaypee
and excess. Clin Dermatol. 2010 Nov-Dec;28(6):669–685. Brothers;2021. pp. 571–86.
 E33
Endocrine disorders

VIKAS PATHANIA, SUNMEET SANDHU

ABSTRACT
Endocrine diseases cause alterations in cutaneous biology because of deficiency or excess of a particular hormone. The muco-
cutaneous changes may be specific to a particular endocrine disease or may be non-specific, but the constellation of cutaneous
and systemic manifestations guide the physicians to the diagnosis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

488 DOI: 10.1201/9781351054225-58

 E34
Renal disorders

DEPENDRA KUMAR TIMSHINA, VISHAL GOLAY

ABSTRACT
Cutaneous manifestations are not uncommon in renal dysfunction, ranging from non-specific hyperpigmentation or pruritus
to specific skin or nail changes. Chronic kidney diseases are very common in the population. Careful examination of the skin
and nails of kidney patients can provide timely clinical clues about underlying kidney diseases. In this chapter, we discuss the
clinical features and differential diagnosis of common cutaneous conditions seen in patients of various renal disorders.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-59 489

 E35
Autoimmune rheumatic diseases

ANUP KUMAR TIWARY

ABSTRACT
Autoimmune rheumatic disorders are a vast group of chronic inflammatory conditions that characteristically and invariably
involve joints, ligaments, tendons, muscles, blood vessels, subcutaneous tissue, and skin to a variable extent. Though autoim-
mune rheumatic diseases can present with a diverse range of cutaneous and systemic manifestations, skin manifestations can
guide treating physicians in making a clinical diagnosis. This chapter aims at discussing cutaneous signs that can be useful in
making clinical diagnosis of various autoimmune rheumatic diseases.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

490 DOI: 10.1201/9781351054225-60

 E36
Hereditary disorders of connective tissue

RAVI HIREMAGALORE, PIYUSH KUMAR

ABSTRACT
Inherited-connective tissue disorders arise from mutations in genes coding for molecules such as collagen, elastin, fibrillin,
etc., which form the bulk of connective tissues of various organs such as skin, bones, joints, heart, blood vessels, etc. Thus, such
disorders can present with a myriad of clinical features, depending on the major organs involved. This chapter discusses the
clinical approach to the diagnosis of various hereditary disorders of connective tissues.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-61 491

 E37
Hepatic diseases

GAUTAM KUMAR SINGH

ABSTRACT
Mucocutaneous changes are quite common in patients with liver diseases. Many of these changes, though common, are non-
specific; not only can they be seen in patients without liver diseases, but also they generally do not point to a specific underly-
ing liver disease. However, they can be telltale signs of underlying liver disease, and they contribute to morbidity, requiring a
physician’s attention. This chapter discusses various dermatoses associated with underlying liver diseases.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

492 DOI: 10.1201/9781351054225-62

 E38
Cardiovascular diseases

PREEMA SINHA, ANWITA SINHA

ABSTRACT
Many cardiovascular diseases are associated with both non-specific and specific cutaneous findings and signs. For example,
pedal edema is a non-specific sign, raising the possibility of underlying cardiovascular disease. On the other hand, Osler nodes
and erythema marginatum are characteristic of infective endocarditis and acute rheumatic fever respectively. Awareness and
identification of dermatoses associated with cardiovascular diseases are useful for both dermatologists and internists.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-63 493

 E39
Neurocutaneous conditions

SATYAKI GANGULY

ABSTRACT
Both the central nervous system and skin are derived from embryonic ectoderm. Mutations affecting the formation, migration,
and differentiation of these cells give rise to a group of diseases, collectively called neurocutaneous syndrome. Mucocutaneous
manifestations are telltale signs and provide crucial clinical clues to the diagnosis of these conditions. This chapter discusses
the clinical approach to the diagnosis of various neurocutaneous syndromes based on predominant cutaneous findings.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

494 DOI: 10.1201/9781351054225-64

 22
Psychocutaneous disorders

SWETALINA PRADHAN, GAURAV DASH

INTRODUCTION ●● The trunk and extensor aspects of the extremities are

common sites. Other areas include the face, neck, and
Psychocutaneous disorders are commonly encountered buttocks (Figure 22.2a,b).
in dermatology. Patients usually have a primary psy- ●● The primary lesion is an erythematous papule with a
chiatric disease with cutaneous manifestations. Most tiny vesicle in the center. The lesions appear in crops
patients are apprehensive about visiting a psychiatrist, and tend to be symmetrically distributed. The lesions
but they do visit a dermatologist for treatment of their are so pruritic that they are immediately attacked and
skin problems. Therefore, dermatologists can play a key are rarely observed by physician.
role in the management of these disorders. Most of the ●● Secondary lesions are what one usually observes, which
time clinical diagnosis is challenging, as these disorders include excoriations and crusts. Lesions heal with
mimic a variety of dermatological conditions. In this
chapter we discuss clinical clues to diagnose such condi-
tions and how to clinically differentiate them from other Psychocutaneous disorders
disease entities.

When to suspect psychocutaneous disorders? 1,2 With lesions Without lesions

●● Psychocutaneous disorders may be suspected when the

history of the patient does not match the lesions.

●● They are mostly seen in emotionally unstable Skin Hair Nail

patients, more commonly in adolescent or middle-

• Papules – Prurigo Nail avulsion- Psychogenicpruritus*
aged females. simplex
Trichotillomania
Trichoteiromania Factitious Delusional parasitosis*
●● They are commonly seen in patients with other • Plaque – Lichen Trichotemnomania dermatitis Cutaneous sensory
simplex chronicus, Onychotillomania disorders*
psychiatric illnesses or with a history of emotional or Pseudoknuckle pads Onychophagia Body dysmorphic
• Nodule – Prurigo disorders
physical abuse. nodularis
●● They are clinically suspected when there are bizarre • Vesicles/bullae –
Factitious dermatitis
lesions at various stages of healing over approachable • Crusts – Dermatitis
areas without proper history. neglecta
• Ulcer – Factitious
dermatitis
• Excoriations –
The clinical approach to various psychocutaneous disor- Neurotic excoriations,
ders is summarized in Figure 22.1, and some of these con- acne excoriée,

ditions are discussed here.1–4 • Purpura –

Psychogenic
purpura,
Munchausen
Prurigo simplex (chronic prurigo of adults, prurigo syndrome
mitis, subacute prurigo, and Hebra prurigo) • Variable
presentations –
●● Prurigo simplex is characterized by intense itching that Factitious dermatitis,
malingering,
is relieved only when the patient scratches the lesion Munchausen
until it bleeds. syndrome and
Munchausen
●● The condition is mostly seen in middle-aged and elderly syndrome by proxy
* These conditions may have excoriations on clinical examination.
persons of both genders. Most patients have neurotic
behavior patterns and overreact to the pruritus. Figure 22.1  Clinical approach to psychocutaneous disorders.
DOI: 10.1201/9781351054225-65 495
496  Psychocutaneous disorders

Figure 22.2  (a) Excoriated papules of prurigo simplex in

a middle aged lady. (b) Prurigo simplex affecting exten-
sor aspect of upper extremity. (a,b – Courtesy: Dr. Piyush
Kumar, Katihar, India.)

hypopigmented, atrophic scars with hyperpigmented

margins.
●● DD: Scabies, atopic dermatitis, insect-bite reactions,
papular urticaria, dermatitis herpetiformis, contact
dermatitis, pityriasis lichenoides et varioliformis acuta
(PLEVA).

Lichen simplex chronicus

●● Well-developed lesions present as circumscribed

lichenified plaques (pigmented, thickened, and

slightly scaly) with excoriations on the surface with
characteristic accentuation of normal skin marking.
●● Lesions are mostly seen over approachable areas such as

the scalp, the nape of the neck, legs, extensor aspects of

the arms, scrotum (in males) and vulva (in females), and
anogenital areas (Figure 22.3a–c).
●● These lesions are characterized by paroxysmal itching

and scratching.
●● DD: Psoriasis (well-defined raised erythematous plaques

with loose whitish scales on the surface), hypertrophic

lichen planus (well-defined lichenified plaque with
central depigmentation and atrophy).

Figure 22.3  (a) Lichenified plaque on the back. The margin is well defined and hyperpigmented, and the surface is remark-
able for erosions. (b) Lichen simplex chronicus over extensor aspect of ankle and dorsum of foot. (Continued)
 Psychocutaneous disorders  497

Figure 22.4  A young girl with factitious cheilitis present-

ing with erythema and scaling of the lips and surrounding
area. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● In purging types of anorexia and bulimia nervosa,

callosities develop over the knuckles of the dominant
hand due to constant friction with the incisors during
induced vomiting. This is known as Russell’s sign.
●● DD: True knuckle pads (unrelated to trauma).

Factitious cheilitis
●● Factitious cheilitis is commonly seen in children with

compulsive disorders.
●● The main mechanism is repeated licking of lips, with or

without biting.
●● Patient presents with irritant contact dermatitis,

crusting, and ulceration of lips (Figure 22.4).

●● DD: Actinic cheilitis.

Prurigo nodularis
●● Nodules are distributed chiefly on extremities,

Figure 22.3 (Continued)  (c) Lichen simplex chronicus especially on the anterior aspect of the thighs and legs.
affecting the scrotum. Note the well-defined margin of ●● Hyperpigmented, hyperkeratotic nodules and/or

the lesion. (d) Unilateral lichenified plaques of pseudo plaques with injury or excoriation occur on the surface.
knuckle pads. (a,b,d – Courtesy: Dr. Piyush Kumar, The margins of individual lesions are often pigmented
Katihar, India; c – Courtesy: Dr. Deverashetti Srinivas, (Figure 22.5a,b).
Nizamabad, India.) ●● Lesions heal with scarring, and new lesions keep

appearing insidiously.
●● There is paroxysmal itching severe enough to be relieved
Pseudo-knuckle pads only by scratching to the point of damage.
●● Pseudo-knuckle pads occur after repeated trauma or ●● Background skin has excoriation and lichenified
friction. changes.
●● Commonly seen in children with obsessive behavior as ●● DD: Lichen planus hypertrophicus (Table 22.1).
“chewing pads” and in adults as an occupational disorder.
●● Common sites are the dorsal surface of the fingers Obsessive-compulsive disorder (OCD)
(knuckles) and hand joints. ●● It is a common anxiety disorder in which patients have
●● It presents as a hypertrophic lesion on the joints, usually repeated and unwanted thoughts or ideas that compel
unilaterally (Figure 22.3d). them to act upon to provide a sense of temporary relief.
498  Psychocutaneous disorders

Table 22.1  Lichen planus hypertrophicus and prurigo

nodularis

Lichen planus
Features hypertrophicus Prurigo nodularis
Excoriations Not seen Present
Itching Patients usually Very severe itching,
rub the may lead to injury
lesions marks on the
surface
Perilesional Perilesional No such lesions
lesions papules of seen
lichen planus
may be seen
Background skin Appears Has excoriations
normal and lichenified
changes

Figure 22.5  (a) Multiple nodules of prurigo nodularis

on extensor aspect of both shins. (b) Prurigo nodularis
presenting as well-defined plaques with eroded top and
pigmented margin. (b – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

●● Obsession of cleanliness leads to repeated hand washing,

using detergents and other chemicals to clean the house,
repeated bathing, excess use of soap and shampoo.
●● Commonly seen in young and middle-aged women.
●● Excess hand washing and use of water often leads to
Figure 22.6  (a) A middle-aged lady with compulsive
candidiasis or irritant contact dermatitis due to overuse hand washing presenting as candidiasis- dorsal aspect.
of soaps and detergents (Figure 22.6a,b). (b) Candidiasis of the palmar aspect. Note preferential
●● Irritant contact dermatitis due to OCD of cleanliness is involvement of creases. (a,b – Courtesy: Dr. Piyush Kumar,
also known as “washing eczema.” Katihar, India.)
 Psychocutaneous disorders  499

Figure 22.7  (a) Keratinous debris on the glans penis and inner prepuce in a case of dermatitis neglecta. (b) Clearance of
keratinous material after cleaning with ethyl alcohol swab.

Dermatitis passivata (dermatitis neglecta) psychological need of which they are usually not
●● It is commonly seen in geriatric or demented patients consciously aware.
who suffer from self-neglect. ●● Usually seen in adolescent women.
●● Lesions are usually found on the upper central chest, ●● Patients present with various kinds of lesions,
over the back. such as purpura, blisters, ulcers, and erythema
●● The cessation of normal skin cleansing will produce an only over approachable sites. These lesions are
accumulation of keratin and dirty debris that forms a polymorphic and bizarre in shape with geometric
thick carapace with time (Figure 22.7a,b). borders and angulated edges surrounded by
●● It’s extreme form has been called the Diogenes syndrome. normal skin, or they assume patterns that do not
conform to any recognized skin disease morphology
Dermatitis artefacta (Figure 22.8a–c).
●● It is a factitious disorder in which patients inflict ●● The patient usually gives a hollow history with unclear
cutaneous lesions upon themselves to satisfy a initiation or evolution of lesions.

Figure 22.8  (a) Geographic-shaped ulcer on the upper

back. The patient injured himself with scissors and other
sharp objects. (b) Sharp objects and medicinal herbs
found in possession of the patient. (Continued)
500  Psychocutaneous disorders

●● Multiple lesions are seen in various stages of evolution.

There may be small superficial erosions to deep
ulcerations with linear/angular or circular/oval shapes
varying from 2 mm to several cm caused by repeated
picking or scratching.
●● The lesions heal with post-inflammatory
hyperpigmentation and leave behind hypo- or
hyperpigmented scars.
●● DD: Prurigo simplex, atopic dermatitis.

Acne excoriée
●● Patients usually have mild acne, but they pick and

squeeze the lesions, leading to scarring.

●● It presents as excoriations on a background of acne

lesions (Figure 22.9a,b).

●● Most frequently seen in young women.

●● DD: Facial picking disorder (where there are no

acneiform lesions originating the picking habit),

trigeminal trophic syndrome, dermatitis artefacta.

Figure 22.8 (Continued)  (c) Ulcers of different depths in

dermatitis artefacta. The guy developed these lesions
after getting separated from his girlfriend. (c – Courtesy:
Dr. Piyush Kumar, Katihar, India.)

Neurotic Excoriations
●● It is an obsessive-compulsive disorder characterized by

a repetitive and compulsive desire to pick, scratch, or

rub the skin.
●● It is most commonly seen in middle-aged women.

●● It is associated with depression.

●● The face is the most commonly affected site. Other sites

include accessible areas like the extensor aspects of the

forearms, arms, legs, thighs, face, and upper trunk. Figure 22.9  (a) Punctate excoriations on the face in a young
Unreachable areas such as the central mid-upper back female with acne vulgaris. Older lesions have healed with
are spared. post-inflammatory hyperpigmentation. (Continued)
 Psychocutaneous disorders  501

Figure 22.9 (Continued)  (b) Acne excoriae presenting as

erosions and crusting. (a,b – Courtesy: Dr. Piyush Kumar,
Katihar, India.)

Psychogenic purpura syndrome

●● Also known as Gardner-Diamond syndrome,

autoerythrocyte sensitization, or painful bruising

syndrome.
●● It occurs more common in emotionally unstable

middle-aged females.
●● Patients present with bizarre, recurrent, tender purpuric

or ecchymotic lesions spontaneously or after mental or

physical stress (Figure 22.10).
●● Extremities are a common site.

●● Lesions are often preceded by a tingling or burning

sensation.

Munchausen syndrome
●● It is a factitious disorder.

●● Munchausen syndrome has also been called “hospital

addiction,” “polysurgical addiction,” and “professional

Figure 22.10  Painful purpuric lesions on the thigh of a
patient syndrome.” lady. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
●● It is seen mostly among medical students and health

care professionals.
●● Essential features are recurrent nature of illness, controlling, hostile, angry, disruptive, or attention-
similarity in pattern of presentation, visiting different seeking behavior during hospitalization.
hospitals with the same complaint, dropping off ●● Patients are pathological liars and have pseudologia
treatment once deception is discovered. fantastica (uncontrollable lying characterized by the
●● Patients mostly present with abdominal or neurological fantastic description of false events).
complaints. Dermatological complaints are rare. ●● On the skin, patients present with bizarre lesions on
●● Symptoms or behaviors are present only when the accessible areas, usually self-inflicted by sharp objects or
patient is aware of being observed. There may be chemicals. Lesions may be ulcerative, erythematous, or
502  Psychocutaneous disorders

gangrenous; there may be subcutaneous emphysema or of traumatic alopecia, with the patch containing hair of
lymphedema. uneven length described as “irregularly irregular” in a
●● Other symptoms may be unexplained bleeding, wave-like or centrifugal pattern (Figure 22.11a).
repetitive urinary tract infection, non-healing wounds, ●● In extreme cases only hair on the occiput is seen,
repeated infections at different sites, and skin and which is known as a Friar Tuck or tonsure pattern
genital injuries. (Figure 22.11b).
●● Multiple hospitalizations often lead to iatrogenic ●● Clinically, three zones are seen on scalp:
general medical conditions (e.g., multiple scars because Zone 1 – uninvolved hair
of unnecessary surgeries or adverse drug reactions). Zone 2 – missing hair due to recent pulling
Individuals with the chronic form of this disorder can Zone 3 – regrowing short hair of various lengths
have a “gridiron abdomen” caused by multiple surgical ●● The eyelashes, eyebrows, and facial and pubic hair may
scars. also be primarily affected.
●● Trichotemnomania is another psychocutaneous
Munchausen by proxy disorder that involves intentional cutting of hair.
●● The victims are usually infants or toddlers.

●● Usually the mother or caregiver inflicts lesions on

another person in order to satisfy a subconscious

psychological need.
●● Risk factors include single parent, previous abuse,

disturbed family, frequent visits to the doctors, frequent

accidents, poverty, overcrowding, and young parents.
●● Skin lesions in form of non-healing wounds,

hemorrhages, blisters, bruising, and excoriations may

be seen.
●● Associated systemic features, such as fever, abdominal

pain, diarrhea, vomiting, and septicemia from infection

of induced cutaneous ulcerations, may be seen.
●● Bruises at “punishment” sites such as the face, ears, and

head of a child less than one year old, black eyes, and
bruising on the buttocks/lower back and outer thighs
should raise suspicion.
●● The pattern of bruising in form of fingertip marks,

striate finger stripe effects from slapping, or pinch

marks as opposing crescent-shaped lesions can give a
clue to diagnosis.
●● Kicks to the lower body show as irregular, large, deep

bruises. Belt and strap damage appears on the trunk as

parallel curved marks.
●● The people administering the harm refuse treatments

and hospital admission and may become hostile and

abusive.

Trichotillomania, trichotemnomania,
trichoteiromania
●● Trichotillomania is a body-focused repetitive behavior

disorder, better termed trichotillosis.

●● Two peaks of preponderance are seen: one in childhood,

mainly between the ages of 5 and 12 years, and other

as chronic cases who present as adults but who started
hair-pulling activities in adolescence or early adult life.
●● There is equal sex predilection in children; in

adolescents and adults, female predominance is seen.

●● There is an irresistible compulsion of hair pulling,

resulting in a short-lived sense of relief and gratification

when hair has been pulled out. Figure 22.11  (a) Trichotillomania in a young girl. Note
●● The hair is twisted or plucked out in patches, mainly in varying length of the hairs. (b) Trichotillomania – Friar
areas accessible to the dominant hand. It leads to a kind Tuck sign. (Continued)
 Psychocutaneous disorders  503

Figure 22.11 (Continued)  (c) Trichoteiromania pre-

senting as localized alopecia. Note lichenified scalp
in the affected area. (b – Courtesy: Dr. Piyush Kumar,
Katihar, India; c – Courtesy: Dr. Deverashetti Srinivas,
Nizamabad, India.)

●● In trichoteiromania the patient manipulates the hair

by rubbing or scratching, leading to frictional damage,
split ends, and traumatic alopecia (Figure 22.11c).
●● DD: Alopecia areata (localized area of hair loss without Figure 22.12  (a) Onychophagia in a young male. Note
any textural change; new hairs are fine and exclamation damage to proximal nail fold and hang nails. (b) Loss of
cuticle, proximal nail fold pigmentation and onychopha-
mark hairs present), tinea capitis (localized loss of
gia. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
hair with surface change in the form of scaling and
dermatitis).

Onychophagia, onychotillomania, ●● Onychotemnomania involves nail cutting to the point

onychotemnomania of pain or injury to the surrounding area.
●● Onychophagia is a body-focused repetitive behavior

disorder that involves chronic, seemingly uncontrollable Delusional parasitosis

nail biting and chewing. ●● It is also known as Ekbom syndrome or
●● It usually begins in childhood and is most commonly pseudoparasitica dysaesthesia.
seen during adolescence. ●● It is a primary psychiatric condition in which the
●● Onychophagia may be associated with thumb- patient believes they are infested with mites, parasites,
sucking or secondary irritant dermatitis, bacteria, worms, insects, or animate material even
infection, inflammation, or malformation of digits though no infesting organism or material can be found
(Figure 22.12a,b). by the clinician. This may disable the patient to such
●● Onychotillomania involves nail pulling, which may be an extent that they often find themselves unemployed,
so severe as to lead to complications like paronychia, end up in debt (in an attempt to rid their home of the
trauma of cuticles. infesting organisms), repeatedly buy new furniture and
504  Psychocutaneous disorders

carpets, and repeatedly wash or clean their bodies to get Table 22.2  Psychogenic dysesthesias
rid of infections.
Dysethesias Clinical features
●● Most of the patients are more than 50 years old, with a
female preponderance. Vulvodynia • Patients complain of pain and
●● Patients complain of localized or generalized itching, burning in the vulva in the
burning, or crawling felt on the skin, most often absence of organic causes.
persistent in nature and disabling. • Patient may even abstain from
●● Skin manifestations are variable, ranging from none to sexual relations.
excoriation, lichenification, prurigo nodularis, erosions, Glossodynia • Burning sensation in the mouth
and sometimes ulcerations. without organic causes, such as
●● Patients often bring evidence of the parasitic infection diabetes mellitus, iron
in the form of clothing, lint, skin crusts, or other deficiency, deficiency of vitamins
debris, which is delusionally misinterpreted as entire B2, B6, and B12, folic acid, or
organisms, body parts, larvae, or ova. This is often allergy to dental material.
collected in a matchbox. This is called “matchbox sign” • The symptoms are aggravated
or specimen sign. by foods and liquid and during
●● A variant of delusional parasitosis is Morgellons the end of the day.
disease, in which the patient has a fixed belief that Trichodynia • Unexplained pain of hair is
fibers are embedded in their body. It presents as an almost always associated with
itching, pricking, or biting sensation. The patients sleep dysesthesia.
dig and pick their skin with nails and tweezers • It is commonly seen in people
(tweezers sign) and bring the debris in a match box who complain of hair loss.
(matchbox sign). Coccydynia • Chronic and disabling pain
●● DD: A genuine infestation, generalized pruritus, occurs in or around the coccyx.
dermatitis herpetiformis. Notalgia • There is itching and paresthesia
paresthetica in the interscapular region,
Psychogenic pruritus (Figure 22.13a,b) which may extend to the
●● This is also known as somatoform pruritus or shoulders, chest, and back.
functional itch disorder.
●● Patient presents with intense chronic (more than six
●● There is an imagined defect in appearance in the face
weeks) pruritus not associated with any known cause. (excessive facial hair, presumed scarring, wrinkling,
●● There are no primary lesions. Secondary lesions such as
excessive facial redness associated with burning
excoriations, scars, pigmentation, and lichenification sensation, large nose, wrinkles) or scalp (excessive hair
may be present. loss, thinning hair), shrinking of genitals, or discomfort
●● The intensity of pruritus may vary with stress and
in the genital areas, extending to the thigh.
usually increases during rest or inaction.

Psychogenic dysesthesia
●● This is also known as persistent somatoform pain

disorder of regional cutaneous/mucosal dysesthesia.

●● There is burning, stinging, or itching in skin and

mucous membrane without any visible lesions or

systemic abnormalities (Table 22.2).
●● Associated psychiatric disturbances like depression or

anxiety may be seen.

Body dysmorphic disorder

●● It is also called dermatologic hypochondriasis, beauty

hypochondriasis, or dysmorphophobia.
●● It is usually seen in adolescent women.

●● It is described as “rich in symptoms but poor in signs.”

●● There is preoccupation with a real or an imagined defect

in physical appearance; or if there is a slight physical

anomaly, concern is out of proportion to the anomaly.
●● Females are often more obsessed with facial defects and

weight issues, whereas males are more concerned with Figure 22.13  (a) Unilateral, pigmented patch in the scapu-
genitalia and muscle mass. lar region in notalgia paresthetica. (Continued)
 Psychocutaneous disorders  505

●● The affected individuals often spend an excessive

amount of time in front of a mirror or repeatedly
checking for perceived imperfections.
●● This causes significant distress, frequently leading to
social isolation or functional impairment.

REFERENCES
1. Kuhn H, Mennella C, Magid M, Stamu-O’Brien C, Kroumpouzos
G. Psychocutaneous disease. J Am Acad Dermatol
2017;76(5):779–791.
2. Yadav S, Narang T, Kumaran MS. Psychodermatology: A
comprehensive review. Indian J Dermatol Venereol Leprol
2013;79:176–192.
3. Al Hawsawi K, Pope E. Pediatric psychocutaneous disorders: A
review of primary psychiatric disorders with dermatologic manifes-
tations. Am J Clin Dermatol 2011;12(4):247–257.
4. Vhiriac A, Brzezinski P, Pinteala T, Chiriac AE, Foia L. Common
psychocutaneous disorders in children. Neuropsychiatr Dis Treat
2015;11:333–337.

Figure 22.13 (Continued)  (b) Unilateral erythematous and

pigmented patch on the back in notalgia paresthetica.
(a,b – Courtesy: Dr. Chirag Desai, Mumbai, India.)
 E40
Dermatoses in HIV-infected persons

SANTOSHDEV P RATHOD

ABSTRACT
Human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) has a great potential to affect skin, mostly
due to immunosuppression and antiretroviral therapy. It may range from non-specific pruritus to extensive/disseminated skin
infections threatening life. Immunosuppression can alter the typical morphologies of various infectious skin lesions such as
molluscum contagiosum, warts, deep mycosis, tuberculosis, syphilis, chancroidal genital ulcers, and herpes. Non-infectious
dermatoses, such as psoriasis, seborrheic dermatitis, granuloma annulare and skin malignancies, may have a more rapid
course, generalized distribution, and resistance to treatment. Occasionally, immune restoration after starting antiretroviral
drugs may reactivate varicella zoster virus and exaggerate the inflammation in various other dermatoses. Thus, it is imperative
to know the common dermatoses and their altered clinical course observed in AIDS patients. Knowing the expected cuta-
neous side effects of antiretroviral drugs is another prerequisite to allow the replacement of one drug with another without
compromising the efficacy.

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 E41
Internal malignancy

SHEKHAR NEEMA, DIPALI RATHOD

ABSTRACT
Underlying systemic changes may be often signaled by a skin manifestation. Any symptomatic and non-metastatic condi-
tion associated with a neoplasm constitutes a paraneoplastic syndrome. Also, some neoplastic diseases affecting the internal
organs may further trigger several cutaneous manifestations. However, recognition of some typical paraneoplastic dermatoses
may lead to the early diagnosis of a neoplasm and in future may determine a better prognosis. According to the various clinical
presentations, age, and gender of patients, the underlying neoplasms may be different. This places great responsibility on the
dermatologists in diagnosing malignancies depending upon the presenting cutaneous signs and symptoms and referring the
affected patient to the concerned specialty for appropriate management. Although these dermatoses are relatively unusual, in
this chapter we discuss the various paraneoplastic cutaneous manifestations. We aim to highlight the clinical manifestations
of these dermatoses to provide assistance in the early diagnosis and management of these rare, but life-threatening conditions.
Careful evaluation of the clinical features and performing the necessary investigations related to respective neoplasms is of
utmost importance for the diagnosis of the paraneoplastic conditions.

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DOI: 10.1201/9781351054225-67 507

 E42
Nails in systemic disease

BALACHANDAR S ANKAD, BALAKRISHNA NIKAM

ABSTRACT
Different parts of the nail unit exhibit specific changes in many internal diseases, and hence, certain nail changes may serve
as indicators of systemic diseases. Thus, knowledge of nail changes can equip dermatologists to suspect and diagnose unap-
parent systemic disorders.

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508 DOI: 10.1201/9781351054225-68

 5
Section    

Miscellaneous

DOI: 10.1201/9781351054225-69
 E43
Head and neck mass

SASI KIRAN ATTILI

ABSTRACT
The head and neck form a distinct area of body incorporating numerous important anatomic structures. Space-occupying
lesions arising out of these structures are considered under the topic “head and neck mass.” Space-occupying lesion means any
nodule, tumor, plaque, or cyst of significant dimension causing disfigurement. Obviously, such masses may have their origin
not only in the skin but rather more frequently in the subcutaneous structures and eye, nose, oral cavity, etc. The former group
of cutaneous conditions is considered in this chapter.

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510 DOI: 10.1201/9781351054225-70

 E44
Red face

ABHISHEK JHA, SHIVANI SHARMA

ABSTRACT
Dermatoses of the face presenting with prominent erythema are called “red face.” Apart from redness, other morphologies of
skin lesions are usually evident. Analysis of these skin lesions in the background of facial erythema helps to reach differentials.

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DOI: 10.1201/9781351054225-71 511

 E45
Leonine facies

SOURABH JAIN

ABSTRACT
Leonine facies can be seen in a wide range of benign and malignant conditions, associated with chronic disease course and/or
bad prognosis. Rarely, leonine facies can be a presenting feature of previously undiagnosed critical conditions such as Sézary
syndrome or leukemia cutis. Thus, recognizing leonine facies can be an important, easily appreciable clinical clue to the diag-
nosis of various conditions needing immediate medical attention. This chapter discusses the clinical approach to the diagnosis
of various conditions commonly associated with leonine facies.

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512 DOI: 10.1201/9781351054225-72

 E46
Linear lesions

ANUPAM DAS, PREETI SHARMA

ABSTRACT
Linear lesions are frequently encountered in the day-to-day practice of dermatology. These lesions may follow the lines of
Blaschko, lymphatics, blood vessels, or dermatomes. They may vary in morphology from a papule to vesicle to plaque, etc.
Many common dermatoses such as lichen planus, psoriasis, etc. may sometimes present in a linear distribution. Knowledge
about common linear lesions as well as atypical presentation of common dermatoses may help in making a faster and more
accurate diagnosis. In this chapter we have tried to classify linear lesions on the basis of the morphology, and the common
ones have been discussed in detail.

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DOI: 10.1201/9781351054225-73 513

 23
Annular lesions

PIYUSH KUMAR, RASHMI ROY

INTRODUCTION The chapter aims to focus on entities commonly presenting

with annular lesions.
Clinical diagnosis is an art of identifying, compiling, and The clinical diagnosis of annular lesions rests on a
analyzing clues present in history and clinical examination particular shape (Box 23.1) and clinical characteristics of
findings. Recognition of additional clues such as annular the border (Table 23.1). 2–5 Certain additional clues have
configuration may help a physician in arriving at a clinical been included in Table 23.2. Various dermatoses that
diagnosis or bring a physician closer to the diagnosis. commonly present with annular lesions are discussed
below. 2–5
“The next time you see a circle in the sky, in a
drawing, or on the skin, pause and consider its Actinic lichen planus (lichen planus actinicus,
significance.” lichen planus subtropicus, lichen planus tropicus,
summertime actinic lichenoid eruption)
—Robert Jackson1 ●● This is Common in Africa, the Middle East, and the

Indian subcontinent.
The list of dermatoses assuming an annular configura- ●● There is no gender predilection, and it affects adults as

tion is exhaustive. A detailed discussion on the pathomech- well as children.

anisms behind the formation of annular configuration is ●● The disease is exacerbated in spring and summer.

beyond the scope of this chapter, and readers are requested ●● The lesions present in four morphologic patterns:

to go through the excellent texts by R Jackson and Sharma annular, dyschromic, classic plaque-like, and
et al.1,2 Certain dermatoses appear annular because of dif- pigmented.
ferent stages of the disease process at different sites of a ●● It presents as hyperpigmented plaques surrounded by
particular lesion. For example, healing lesions of discoid hypopigmented border over sun-exposed sites such as
lupus erythematosus may have an atrophic center while the the face, V area of the chest, neck, back of the hand,
margin is still active and erythematous (or sometimes, pig- lower extensor forearms (Figure 23.1a,b).
mented). Similarly, the center of bullous impetigo may heal ●● Pruritus is mild or absent.

with epithelization while the margin is still active as erosion ●● The disease may be seen with lichen planus

or pustule. The discussion on dermatoses that appear annu- pigmentosus, which does not involve sun-exposed
lar during the evolution of lesions or that assume an annu- areas. Mucous membrane involvement is much less. The
lar morphology only occasionally has not been attempted. Koebner phenomenon is not seen.

BOX 23.1: Annular and related morphology

Annular: Ring-shaped lesion. Usually, plaques present with annular configuration, but other lesions, such as macular,
papules, nodular, vesicles, or pustules, may present in annular configuration.
Arcuate: Incomplete annular lesion, arc-shaped lesion.
Polycyclic: Polycyclic lesions are formed by coalescing of the adjacent annular or arcuate lesions.
Target/Iris lesions: The lesion has three zones – a central dusky-red macule (or vesicle) surrounded by pallor, which in
turn is surrounded by erythema. Typically seen in erythema multiforme.

514 DOI: 10.1201/9781351054225-74

 Annular lesions  515

Table 23.1  Clinical diagnosis of annular lesion

Border Features Dermatoses

Patch Hypopigmented Actinic lichen planus (perilesional halo with pigmented center), halo nevus, Wornoff
ring, steroid-induced perilesional hypopigmentation
Hyperpigmented Discoid lupus erythematosus (during healing)
Erythematous Dermatophyte infection (steroid-modified), figurate erythemas, fixed drug eruption,
bullous impetigo (healing phase), necrolytic migratory erythema, livedo reticularis,
circinate balanitis (vulvitis), annular lichenoid dermatitis of youth
Papule Scaly Dermatophyte infection
Non-scaly Granuloma annulare
Keratotic Elastosis perforans serpiginosa
Plaque Erythematous • Scales present – annular psoriasis, pityriasis rosea, subacute cutaneous lupus
erythematosus, neonatal lupus erythematosus, secondary syphilis
• Scales absent – urticaria and urticaria multiforme, granuloma annulare, annular
giant cell elastolytic granuloma, necrobiosis lipoidica, annular tufted angioma
• Variable scaling – Hansen’s disease, Jessner’s lymphocytic infiltration, annular sarcoidosis
• Crust/hyperkeratotic areas – lupus vulgaris
Pigmented Lichen planus, basal cell carcinoma
Keratotic • With a groove/canal – porokeratosis
• without a groove – annular lupus vulgaris, annular tuberculosis verrucosa cutis
Targetoid appearance • Acute hemorrhagic edema of infancy
Nodules Keratoacanthoma centrifugum marginatum
Vesicles & Linear IgA dermatosis/chronic bullous disease of childhood, cutaneous larva migrans,
Bullae neutrophilic figurate erythema, necrolytic migratory erythema
Pustules Dermatophyte infection, bullous impetigo, subcorneal pustular dermatosis, pustular
psoriasis, circinate balanitis, pseudo-circinate balanitis
Scales • Leading scale – dermatophyte infection
• Trailing scale – pityriasis rosea, erythema annulare centrifugum (figurate erythema)
• Double edged scales – ichthyosis linearis circumflexa
• Transient lesions – erythrokeratodermia variabilis
Purpura Purpura annularis telangiectodes of Majocchi, acute hemorrhagic edema of infancy,
Henoch-Schönlein purpura, suction (traumatic) purpura, neutrophilic figurate erythema

Table 23.2  Clinical clues for the diagnosis of annular lesions

Clue Dermatoses
Arcuate lesions Elastosis perforans serpiginosa, lupus vulgaris, Jessner’s lymphocytic infiltration, keratoacanthoma
centrifugum marginatum, circinate balanitis, ichthyosis linearis circumflexa
Serpiginous lesions Cutaneous larva migrans
Polycyclic lesions Dermatophyte infection
Predominantly affected sites • Photoexposed area – actinic lichen planus, subacute cutaneous lupus erythematosus,
neonatal lupus erythematosus, basal cell carcinoma
• Flexures – subcorneal pustular dermatosis, necrolytic migratory erythema, annular
lichenoid dermatitis of youth
• Genitalia – erythema multiforme, circinate balanitis
• Acral areas – necrolytic migratory erythema, granuloma annulare (dorsal surface),
secondary syphilis (palmar/plantar surface)
• Lower legs – necrobiosis lipoidica, purpura annularis telangiectodes of Majocchi,
Henoch-Schönlein purpura
• Trauma prone areas – lupus vulgaris and tuberculosis verrucosa cutis, necrolytic
migratory erythema, cutaneous larva migrans
Rapidly spreading lesions Dermatophyte infection, granuloma annulare, figurate erythema, necrolytic migratory
erythema
Transient/Migratory lesions Urticaria, cutaneous larva migrans, circinate balanitis, ichthyosis linearis circumflexa,
erythrokeratodermia variabilis
Reverse Koebnerization Granuloma annulare
516  Annular lesions

Figure 23.1  (a) Actinic lichen planus seen as pigmented

patch and plaque with palo halo. (b) Actinic lichen pla-
nus on the forehead.

●● DD: Discoid lupus erythematosus (active lesion – scales), granuloma annulare, Hansen’s disease (annular
erythematous plaque with adherent scales; healing lesions of borderline tuberculoid and mid-borderline),
lesion – depigmented atrophic center with pigmented erythrokeratodermia variabilis (transient gyrate or circinate
margin), sarcoidosis, halo nevus, steroid-induced erythematous patches which fade or migrate within few
perilesional hypopigmentation. hours, additional fixed erythematous hyperkeratotic
plaques), purpuric annular dermatoses (Table 23.4).
Figurate erythema (gyrate erythema)
●● These are a group of reactive conditions seen in a variety Erythema annulare centrifugum (EAC)
of benign or malignant conditions and are clinically ●● It is the most common type of figurate erythema and

characterized by annular erythema with trailing scales. may affect any age group, with a peak incidence in the
●● Classically, this group includes erythema annulare fifth decade of life.
centrifugum, erythema marginatum, erythema migrans ●● It has been reported in many infectious entities such as

and erythema gyratum repens (Table 23.3). dermatophytes, candida, Epstein-Barr virus, pox virus, HIV,
●● DD: Tinea corporis (itchy, polycyclic lesions with varicella, parasites and bacteria. Certain drugs (cimetidine,
papules, pustules, and scales at the margin), annular NSAIDS, antimalarials), Crohn’s disease, hypereosinophilic
psoriasis, annular urticaria (transient lesions without any syndrome and pregnancy also act as causative factors.

Table 23.3  Figurate erythemas

Progression of Associated diseases

Diseases Age of presentation Common sites lesion (rate) Course of disease (selected)
Erythema Affect any age group Favors thighs, hips, Up to 6 cm in Lesion persists for Crohn’s disease,
annulare Peak at fifth decade and trunk diameter or weeks to months autoimmune
centrifugum of life more in 1–2 Total duration endocrinopathies,
weeks ranges from days hypereosinophilic
to decades syndrome, neoplasm
Erythema Bimodal age of onset Lower extremities, Lesion begins Lesion lasts for Borrelia burgdorferi
migrans One peak at axillae, groin, and after tick 4–6 weeks infection
5–19 years and popliteal fossa in bite and can
second at adults and trunk progress up
55–70 years in children to 15 cm
Erythema Children 5–15 years Trunk, axillae, and 2–12 mm over Lesion lasts from Rheumatic fever
marginatum Can also affect the proximal 12 hours hours to a few
adult population extremities days
Erythema Commonly affects Trunk and other 1 cm per day Lesion resolves Lung, breast, and
gyratum adults large body after treatment esophagus cancer
repens surface areas of the underlying pulmonary
malignancy tuberculosis
 Annular lesions  517

Table 23.4  Purpuric annular lesions

Annular purpuric Commonly affected age group and

conditions site Clinical features
Purpura annularis • Mostly in young adults • Annular lesions with pinpoint petechiae at the margin. The
telangiectodes • Common in females center may show atrophy and telangiectasia.
of Majocchi • Bilateral lower legs affection is • The lesion may persist for years.
common
• Arms and trunk may be involved
Acute • Typically affects children • Triad of fever, large purpuric plaques, and acral edema.
hemorrhagic between 4 and 24 months of • Bilaterally symmetrical purpuric plaques on the extremities
edema of age and face, with relative sparing of trunk.
infancy • Purpuric plaques have targetoid appearance.
• Systemic features are minimal.
• The disease runs an acute self-limiting course.
Henoch- • Commonly affects children • Papular, purpuric, and urticarial lesions on lower legs. The
Schönlein between 3 and 6 years of age lesions appear in crops.
purpura • Visceral involvement manifests as abdominal pain, vomiting,
diarrhea, intussusceptions, bloody stools, joint pain (knees
and ankles) and nephritis.
• The disease runs an acute self-limiting course, but recurrence
may occur.
Suction • May affect any age group • Localized suction to the skin usually produces purpura or
(traumatic) • Any site affected sometimes, blisters.
purpura • History of suction toys, vacuum • Acute self-limiting condition.
cleaners, gas masks, therapeutic
cups, and sucking while kissing
Neutrophilic • Any age group affected • Annular lesions with erythema, vesicles, or purpura at the
figurate • Face, trunk, or extremities margin.
erythema affected • The disease runs a self-limiting course with relapse in summers.

●● A rare autosomal dominant form of EAC has been

described, which is referred to as “familial annular
erythema.”
●● The initial lesions present as firm pink papules that
expand centrifugally, with central clearing in an
annular pattern but polycyclic. An incomplete arc
pattern is also seen when the expansion is not uniform
(Figure 23.2).

Figure 23.2  (a) Erythema annulare centrifugum with annular erythema and trailing scale. (b) Multiple lesions of erythema
annulare centrifugum on the trunk.
518  Annular lesions

BOX 23.2: Clinical forms of Erythema annulare centrifugum

Superficial form – It shows minimally elevated lesions with trailing scales at the inner border of the annular erythema
due to desquamation. Scaling may not be seen in all of the lesions. Pruritus may be associated. Vesicles may
develop occasionally at the margin.
Deep form – It shows lesions with elevated edges without scales. Pruritus is absent.

●● An individual lesion can expand up to 6 cm or more in annular erythematous plaque around the papule, a
diameter in a period of one to two weeks. It can persist raised advancing border without scales, and a central
for weeks to months without any associated systemic light-colored area resembling a bull’s-eye. The lesion
manifestations. is warm to the touch. The color may vary from red to
●● The lesion can be localized or generalized, rarely bluish-red. The lesion can become indurated or develop
involving the palm, sole, mucous membrane, and scalp. vesicles and may undergo necrosis.
●● Two forms of EAC are known (Box 23.2). ●● It commonly affects the lower extremities, axilla and
●● The lesions resolve with post-inflammatory groin, and popliteal fossa in adults and affects the trunk
hyperpigmentation without any scarring. But in children.
recurrences are known. The total duration of disorder ●● The annular erythema can develop up to a diameter of
ranges from days to decades. 15 cm.
●● The lesion is associated with burning in some cases.
Erythema marginatum (erythema marginatum ●● Pruritus and pain are absent. Localized alopecia may
rheumaticum or erythema annulare rheumaticum) develop at the site of the lesion.
●● This disorder is a cutaneous manifestation of rheumatic ●● The lesions last four to six weeks when left untreated.
fever. Not all patients with rheumatic fever develop ●● Multiple lesions can occur in the same patient due to
erythema marginatum. multiple tick bites.
●● Mostly children between 5 to 15 years are affected, but ●● Disseminated lesions can appear days to weeks after the
adults can also develop the disease. appearance of the primary lesion and are smaller in size
●● The lesion begins as erythematous macules that spreads and with a less indurated center.
peripherally and forms annular or polycyclic patches or ●● Other features of Lyme disease, such as fever, arthralgia,
plaques without scaling. The lesions are asymptomatic malaise, fatigue, headache, neck stiffness, myalgia, and
and can migrate from 2–12 mm over a period of lymphadenopathy, are also present.
12 hours. The skin appears pale or lightly pigmented ●● DD: Southern tick-associated rash illness. Presents with
in areas of previous involvement. The lesions are more similar lesions but have negative serology for Lyme
prominent in afternoon. disease.
●● It commonly involves trunk, axillae, and proximal

extremities. Erythema gyratum repens

●● The lesions last from hours to a few days. Recurrent ●● It is a rare and often paraneoplastic eruption associated

crops can occur over a number of weeks. with lung, breast, esophagus, and stomach cancer.
●● Carditis, migratory polyarthritis, subcutaneous The cutaneous lesion can develop prior to or after the
nodules, and sydenham chorea are the associated diagnosis of neoplasm.
findings. ●● It commonly affects adults.

●● The patient presents with pruritic, multiple, concentric

Erythema migrans (Erythema chronicum migrans) polycyclic, annular or circinate erythematous plaques
●● It is seen in Lyme disease, an infectious disease with scales at the advancing age. The lesion assumes a
following the bite of infected tick. The disease is characteristic “wood grain” appearance (or zebra-like
caused by Borrelia burgdorferi spirochetes, which are pattern). The lesion spreads peripherally with a rate of 1
transmitted by bites from several species of Ixodes tick. cm/day.
●● This disorder is seen commonly in the northeast, mid- ●● DD: Resolving pityriasis rubra pilaris. History of initial

Atlantic Great Lake regions, and Northern and Eastern classic lesion is present, which resolves and resembles
Europe. erythema gyratum repens. Can be differentiated
●● The peak incidence is seen in spring and the summer. histologically.
●● Males and females are equally affected, and there is a

bimodal age of onset with one peak at 5 to 19 years and Fixed drug eruption (FDE)
second at 55 to 70 years. ●● FDE usually presents with a discoid erythematous

●● The lesion starts after the tick bite as a small, red macule patch that resolves with post-inflammatory
or papule at the site. After 7 to 15 days it expands, with hyperpigmentation. Sometimes, during recurrence,
 Annular lesions  519

Figure 23.3  Recurrent lesion of fixed drug eruption may

appear annular with central pigmentation (from prior epi-
sode) and peripheral erythema.

only the margin becomes active and erythematous with

a pigmented center, assuming an annular appearance
(Figure 23.3).

Bullous impetigo
●● Lesions of bullous impetigo start healing from the

center with epithelization, and lesions assume an

annular configuration for a brief period of time. The
still-active margin is clinically characterized by erosion
or pustules (Figure 23.4a,b).

Livedo reticularis
●● It is a benign condition that occurs after a physiologic

vasospastic response to cold exposure or may occur due

to various pathological causes (Box 23.3).
●● The disease commonly affects young children and
Figure 23.4  During the course of disease, bullous impe-
middle-aged women. tigo may appear annular with central erosion or crust and
●● Lesions present as a transient or persistent blotchy, peripheral rim of pus. In late stages, central epithelization
reddish-blue to purple, net-like reticulated cyanotic and peripheral erosion may be observed.

BOX 23.3: Causes of livedo reticularis

●● Hypercoagulable states like antiphospholipid syndrome, DIC, protein C and S deficiency, Homocystinuria.
●● Vasculitis like – cutaneous polyarteritis nodosa, cryoglobulinemic vasculitis, autoimmune CTD vasculitis like- SLE,
rheumatoid arthritis, Sjögren’s syndrome.
●● Calciphylaxis
●● Sneddon syndrome
●● Embolic states
●● Medications, such as amantadine, interferons, heparins.
●● Neoplasms
●● Neurological disorders, such as complex regional pain syndrome, multiple sclerosis, diabetes mellitus.
520  Annular lesions

Figure 23.5  Livedo reticularis as fixed erythema in a net-

like pattern.

pattern that is symmetric, reversible, and uniform and

affects the extremities (Figure 23.5).
●● Different types of livedo reticularis are recognized:
●● Congenital (cutis marmorata telangiectatica Figure 23.6  Circinate balanitis seen as whitish slough and
congenital) presents as a persistent reticular erythema in a serpiginous pattern.
vascular pattern limited mostly to one extremity.
The lesion is present since birth and complete
resolution is seen in few years in some patients.
●● Physiological livedo reticularis (cutis marmorata) Dermatophyte infection
●● The clinical presentation of dermatophyte infection
is seen in neonates, infants, and young women
commonly. It occurs in response to cold exposure. is highly variable and depends on a lot of factors. The
Resolution of the lesion is noted on rewarming. patients usually present with single or multiple circular,
●● Primary or idiopathic livedo reticularis presents annular, circinate, arcuate, concentric, or oval patches
with a widespread fine network affecting the lower or plaques. Central clearing of the lesions with sharply
extremities. The lesion persists on warming. defined, raised erythematous scaly borders gives
●● Livedo reticularis with systemic diseases (Box 23.3) them an annular appearance. The lesion spreads in
is due to vasospasm, autoimmune connective tissue, centrifugal fashion from the core, with central clearing
vessel-wall pathology, or intraluminal pathology. and mild scaling, giving them a ring shape, hence the
●● DD: Livedo racemosa (symmetric, irreversible and term ringworm.
●● It may be localized or generalized and unilateral or
broken pattern), erythema ab igne (develops in
response to heat and evolves into a fixed reticulated bilateral. It is usually asymmetrical when it has bilateral
hyperpigmentation localized to the rea exposed to heat), involvement.
●● Non-inflammatory types present with itchy, annular, or
reticulated erythematous mucinosis, poikiloderma.
polycyclic lesions with scales and papules at the margin
Circinate balanitis (Figure 23.7a,b).
●● Circinate balanitis (or circinate vulvitis in females) is a ●● Inflammatory types are characterized by itchy

mucosal hallmark of reactive arthritis and, rarely, can polycyclic or annular lesions with erythematous
be a presenting feature. papules, pustules, erosions, crusts, and scales at the
●● Affected patients develop moist erythema and/ margin (Figure 23.7c,d).
or pustules in a serpiginous, arcuate, or annular ●● Tinea imbricata (tokelau) – It is commonly seen in

configuration (Figure 23.6). Southeast Asia, the South Pacific, Central America,
●● Similar lesions have been noted in pustular psoriasis and South America and is caused by Trichophyton
too. concentricum. It clinically presents as concentric,
●● When patients with secondary syphilis develop annular scales, with variable erythema.
similar lesions, the condition is called pseudo circinate ●● Steroid-modified tinea/tinea incognito – The

balanitis.6 characteristic clinical features are lost, and hence,

diagnosis requires a high index of suspicion. Common
Annular lichenoid dermatitis of youth (ALDY) clinical features are as follows:
●● The condition is mostly reported in young males and ●● The border becomes less prominent or barely
shows a predilection for the axilla, groin, and flanks. appreciable.
●● ALDY presents as solitary or multiple sharply ●● The lesions become less scaly and more
demarcated annular or arcuate erythematous patches or erythematous.
plaques with central hypopigmentation. ●● The central clearing is less appreciable, with
●● The lesions heal with post-inflammatory eczematous changes in the central part
hyperpigmentation. (Figure 23.7e).
 Annular lesions  521

Figure 23.7  (a) Tinea cruris and corporis as polycyclic lesion

with erythema, papules, pustules, and scaling at the margin and
relatively central clearing. (b) Tinea manuum as polycyclic lesion
with scaling at the margin. (c,d) Inflammatory tinea corporis
with prominent pustule formation at the margins. (Continued)
522  Annular lesions

Figure 23.7 (Continued)  (e) Steroid-modified tinea with central eczematous changes. (f) Steroid-modified tinea presenting
as concentric rings of erythema (tinea pseudoimbricata). (c,d,f – Courtesy: Dr. PC Das, Katihar, India.)

●● The lesions may have more inflammatory papules

and pustules without much central clearing.
●● The lesions may show concentric rings of tinea
lesions (Tinea pseudoimbricata) (Figure 23.7f).7
●● The lesions become more widespread.
●● DD: Classical tinea lesions (Figurate erythema,
pityriasis rosea, secondary syphilis), steroid-modified
tinea (nummular eczema, contact dermatitis).

Granuloma annulare
●● The lesion presents as solitary or multiple, skin-

colored or erythematous, small grouped papules. The

lesions spread with central clearing, forming annular
lesions. Papules at the periphery give the lesions their
characteristic “beaded” appearance. The lesions are
typically non-scaly, and this finding is of diagnostic
value (Figure 23.8a–d).

 
Figure 23.8  (a) Granuloma annulare with indurated margin. (b) Granuloma annulare with prominent indurated margin.
Note beaded appearance of the margin and absence of surface changes. (Continued)
 Annular lesions  523

granuloma annulare, palmar granuloma annulare,

granuloma annulare in HIV patients, granuloma
annulare with malignant disease.
●● DD: Annular sarcoidosis, Tinea corporis, leprosy,
eruptive xanthoma.

Elastosis perforans serpiginosa (EPS)

●● It is a rare skin condition caused by transepidermal

elimination of abnormal elastic fibers and focal

dermal elastosis. The disease can be inherited or
acquired.
●● It commonly affects children or young adults.

●● It can be seen in association with Down syndrome,

Ehlers-Danlos syndrome, osteogenesis imperfecta,

Marfan syndrome, pseudo xanthoma elasticum,
Rothmund-Thomson syndrome, aerogeria, long-term
use of penicillin, chronic renal failure, cutis laxa,
diabetes mellitus, Wilson’s disease, cystinuria, and
scleroderma.
●● The lesion of EPS starts as 2- to 5-mm keratotic papules

that are arranged in serpiginous or annular patterns.

Lesions may enlarge to several centimeters.
●● They involve the face, neck, arms, and flexures.

●● Spontaneous resolution of the lesion is often observed,

or the lesion may persist for many years.

●● DD: Tinea corporis, granuloma annulare, annular

sarcoidosis, porokeratosis of Mibelli (annular or

serpiginous plaque with thready keratotic border with
central groove. Center may show hyperpigmentation,
atrophy, or hypertrophy).

Pityriasis rosea
●● It is a self-limited inflammatory papulosquamous

eruption usually affecting adolescents and young adults

between 10 and 35 years.
●● The disease is most prevalent in spring and autumn.

●● At first a solitary lesion (herald patch or mother patch)

appears, mostly over the trunk and enlarges to 2–4

cm over several days. This lesion is salmon-pink or
brown, oval patches or plaques with advancing raised
Figure 23.8 (Continued)  (c) Multiple lesions of granuloma margins. The center of the lesion shows small fine
annulare on the trunk. (d) Multiple papules of granuloma scales, and the margin has a large trailing collarette of
annulare. Lesions have started to assume annular configu- scales with free edges pointing inward. The scales tend
ration. (b – Courtesy: Dr. PC Das, Katihar, India.) to fold across the line of stretch – “hanging curtain”
sign (Figure 23.9).
●● After some days, similar but smaller lesions (daughter

●● Lesions are often in a symmetrical distribution lesions) appear, involving the trunk, neck, and proximal
and affect the distal upper extremities, but legs, extremities. Rarely eyelids, palms, soles, scalp, or penis
feet, trunk are also involved. Facial involvement is may be involved. The lesions appear with their long axis
rare. following lines of cleavage on the trunk, arranged in
●● Spontaneous resolution of the lesions is seen. a “fir tree” or “Christmas tree” pattern (Figure 23.10).
●● Many forms of granuloma annulare are present, and the Pruritus is mild to severe.
patient shows only one type during its course of illness ●● The eruption persists for six to eight weeks followed

except in the subcutaneous form. by spontaneous resolution. Occasionally lesions may

●● The variants of granuloma annulare are localized persist longer. Relapse and recurrences are infrequent.
granuloma annulare, generalized granuloma ●● Some of the atypical variants are as follows:

annulare, patch-type or macular granuloma annulare, ●● Inverse pityriasis rosea involves the axilla and
subcutaneous granuloma annulare, perforating inguinal area.
524  Annular lesions

Figure 23.9  Multiple annular lesions of pityriasis rosea on

the trunk. (Courtesy: Dr. Niharika Ranjan Lal, ESIPGIMSR,
Kolkata, India.)

●● Oral variants are asymptomatic, erythematous

macules with raised borders and clearing centers.
Sometimes aphthous ulcer-like lesions are
present.
Figure 23.10  Annular lesions of Pityriasis rosea on the
●● Papular variant has a predilection for the face and
back.
scalp and is seen commonly in black children.
●● Purpuric variant has petechiae and ecchymosis
along Langer lines of the neck, trunk, and proximal
extremities and can be a sign of acute myeloid ●● Lesions of SCLE occur on sun-exposed areas and may
leukemia. present in two forms (Figure 23.11).
●● Urticarial, pustular, vesicular, and erythema 1. The annular form presents with erythematous,
multiforme-like variants are also present. annular scaly plaques with central clearing. They
●● Herald patches may be absent or multiple. tend to coalesce and form a polycyclic array.
●● DD: Seborrheic dermatitis (yellow greasy scales are 2. The papulosquamous form presents as eczematous
present over the lesions), secondary syphilis (split or psoriasiform lesions.
papules and condyloma lata, a history of chancre), tinea ●● Induration is minimal, and the lesion heals without
corporis, tinea versicolor (perifollicular hypopigmented scarring. Dyspigmentation or hypopigmentation can
lesions), guttate psoriasis (raindrop like scaly papules, develop.
Koebnerization). ●● Patients may have associated arthralgia or arthritis and
leukopenia.
Subacute cutaneous lupus erythematosus (SCLE) ●● Fifty percent of patients meet the ARA criteria for SLE.
●● It is a subtype of cutaneous lupus erythematosus and ●● DD: Pityriasis rosea, secondary syphilis, tinea corporis,
commonly affects young to middle-aged women. erythema annulare centrifugum, classic granuloma
●● SCLE may present without any systemic involvement. annulare.
 Annular lesions  525

Figure 23.12  Annular and plaque type papulosquamous

lesions in secondary syphilis.

Secondary syphilis
●● Secondary syphilis presents typically as generalized,

macular, papular or papulosquamous eruptions that are

copper-colored, non-pruritic, discrete, and distributed
over the flanks, shoulders, extremities and trunk,
including palms and soles.
●● Atypical lesions, such as annular plaques, pustular,

nodular, ulcerative, corymbiform, and SLE-like lesions,

can also appear.
●● Annular lesions with central hyperpigmentation
Figure 23.11  Annular scaly plaque of subacute cutaneous
lupus erythematosus. commonly affect children and dark-skinned people and
are observed on the cheeks or around the angle of the
mouth. They can also be seen over the penis, feet, and
Neonatal lupus erythematosus legs. Lesions can range from 1–2 mm to 15 –20 mm
●● Neonatal lupus erythematosus is an infantile form (Figure 23.12).
of SCLE caused by transplacentally acquired Ro/SSA ●● Condyloma lata, syphilitic sore throat, moth-eaten
antibodies. alopecia, painless aphthae, necklace of Venus (diffuse
●● It appears to be more common in blacks, Latinos, and hyperpigmentation of neck with superimposed
Asians. hypopigmented macules), corona veneris (papular
●● The lesion presents as an annular, round or elliptical syphilitic lesions along the anterior margin of the
erythematous macule and scaly plaques, commonly scalp or on the back of the neck), and split papules
involving the face over the periorbital region and scalp. around oral commissures are other presenting
The extremities can also get involved. It is present since features.
birth or develops within the first few weeks. The mucosa ●● DD: Pityriasis rosea, guttate psoriasis.
is spared.
●● These lesions resolve in six to eight months without Urticaria
scarring, but some cases show dyspigmentation and ●● Urticaria is characterized by wheals and angioedema.

residual telangiectasia. Wheals during the course of disease may resolve

●● The major associated systemic finding is congenital centrally while peripheral activity is maintained,
heart block, with or without cardiomyopathy. The heart resulting in annular appearance (Figure 23.13a).
block is usually present from birth (or may appear ●● Urticaria multiforme (acute annular urticaria) is

later) and is permanent. Other systemic concerns considered a morphological variant of acute urticaria
are thrombocytopenia and hepatobiliary disease, and is a benign, self-limited disease.
which may present as liver failure during gestation or ●● It commonly affects pediatric age groups from
hyperbilirubinemia in neonates. infants to children.
●● DD: Tinea faciei, seborrheic dermatitis, pityriasis ●● Viral infections, antibiotics, and immunization are
versicolor. known triggering factors.
526  Annular lesions

Figure 23.13  (a) Annular lesion of urticaria with central blanching. (b) Urticaria multiforme with dusky center.

●● The lesion presents as itchy, blanchable annular

or polycyclic erythematous plaques with an
ecchymotic dusky center, followed by central
clearing. It commonly affects the face, trunk, and
extremities. The individual lesion does not last for
more than 24 hours (Figure 23.13b).
●● Acral edema, dermatographism, and mild fever are
seen.
●● The disease lasts 2 to 12 days.
●● DD: Erythema multiforme (typical target lesions,
persists for more than 24 hours, mucous membrane
affected), urticarial vasculitis (commonly affects
adults, lesions persist for more than 24 hours,
burning sensation).

Annular elastolytic giant cell granuloma (AEGCG)

(actinic granuloma, elastolytic giant cell granuloma)
●● AEGCG is a rare dermatosis possibly related to

ultraviolet rays and infrared radiation exposure.

●● The disease commonly affects middle-aged females.

●● Initial lesions are single or grouped skin-colored to

pink papules that coalesce to form annular plaques.

Well-developed lesions present as annular plaques 1–10
cm in diameter with a raised erythematous border
and slightly atrophic, hypopigmented center. Surface
changes including scales are usually absent. The lesions
are present over sun-exposed areas (Figure 23.14a).
●● Lesions are usually asymptomatic, but pruritus has been

seen in many cases. Figure 23.14  (a) Annular and arcuate lesions of annular
●● Spontaneous remission of the plaques occurs within
elastolytic giant cell granuloma on the upper back.
months to years, and then mottled dyspigmentation or (b) Brown colored annular plaque with central atrophy in
normal appearing skin is left behind. necrobiosis lipoidica. (Continued)
 Annular lesions  527

Annular tufted angioma

●● It is a rare form of benign vascular neoplasm and

commonly affects children and young adults without

any gender preference.
●● The disease presentation can be congenital or acquired.

●● The lesion presents as mottled red to pink asymptomatic

patches or plaques with superimposed angiomatous

papules. The lesion grows slowly by lateral expansion,
acquiring annular and arciform shapes over a period of
months to years (Figure 23.15).
●● Lesions commonly affect the upper trunk, neck, and

proximal part of the extremities.

●● Over years lesions stabilize, shrink, or heal, leaving a

fibrotic scar. Complete spontaneous regression may be

observed in some cases.
●● Congenital disease may develop the Kasabach-Merritt

phenomenon.
●● DD: Infantile hemangioma (grows rapidly during first

year of life followed by spontaneous slow involution),

Kaposiform hemangioendothelioma (lesion presents
with deeper involvement of tissues as bulky deep
seated lesion infiltrating multiple tissue planes), Kaposi
sarcoma (lesions lack tufting and are multicentric; they
are pink to violaceous in appearance).

Figure 23.14 (Continued)  (c) Necrobiosis lipoidica pre-

senting with brownish annular plaques. (Courtesy:
Dr. Dependra Kumar Timshina, Remedy Clinic, Siliguri, India.)

●● DD: Tinea corporis, granuloma annulare, necrobiosis

lipoidica (yellowish plaques with telangiectatic center,
ulceration), erythema annulare centrifugum (annular
erythematous plaque with trailing scales).

Necrobiosis lipoidica
●● The lesion usually starts as small, firm red-brown

papules with slight scales that enlarge gradually to form

an annular lesion.
●● A well-developed lesion is clinically characterized by

yellow-brown glistening or telangiectatic plaque with

central atrophy and an elevated violaceous well-defined
rim (Figure 23.14b,c).
●● The lesion commonly occurs on shins, but the upper

extremities, trunk, face, scalp, palms, and soles can also

get affected.
●● Decreased sensation to pin prick, fine touch,

hypohidrosis, and partial alopecia can be seen in some

cases.
●● Ulceration and Koebnerization are common.

●● Development of squamous cell carcinoma is a late

complication.
●● DD: Granuloma annulare, lipodermatosclerosis

(presents as hardening of the skin on lower limb), Figure 23.15  Annular tufted angioma presenting as
necrobiotic xanthogranuloma (yellowish plaques and pigmented, indurated plaques in arciform and annular
nodules mostly around periorbital area). pattern.
528  Annular lesions

Hansen’s disease8 (Figure 23.16a,b). Sometimes, TT leprosy may assume

●● Two forms of leprosy are present, depending upon the annular configuration during the course of the disease.
degree of immunity – tuberculoid type and lepromatous ●● Tuberculoid type (TT) – Sometimes, the plaque may
type. Ridley and Jopling have classified leprosy on the heal from the center, resulting in an atrophic center
basis of clinical, bacteriological, immunological and with a sharply defined and elevated border, giving an
histopathological features into five types – tuberculoid annular appearance (saucer right way up appearance).
(TT), borderline tuberculoid (BT), mid-borderline (BB), ●● Borderline tuberculoid (BT) – Annular lesion of BT
borderline lepromatous (BL), and lepromatous leprosy leprosy is characterized by a raised and well-defined
(LL). margin raised in one part but with an ill-defined
●● Annular lesions are typically seen in borderline margin merging gradually with normal skin in another
leprosy (BT, BB, BL) and careful analysis of annular part. Satellite lesions can also be seen. The center is
morphology serves as an important clinical clue dyshidrotic (Figure 23.16c,d).

TT BT BB BL
(a)

TT BT BB BL
(b)

 
Figure 23.16  (a) Schematic diagram: Surface view of annular lesions in leprosy. (b) Schematic diagram: Cross-section view
of annular lesions in leprosy. (c) Annular plaque of BT leprosy with ill-defined borders at the lower pole. (d) BT leprosy with
type 1 lepra reaction presenting as annular scaly plaque with ill-defined borders at places. (Continued)
 Annular lesions  529

Figure 23.16 (Continued)  (e) Annular lesion of BB leprosy with

sharp inner border and sloping outer border. (f) Note sharp
vertical inner borders in BB leprosy. (g) Annular plaque of BL
leprosy with sloping inner and outer borders. There are many
plaques of BL leprosy too. (h) Annular lesion of BL leprosy.
Note sloping inner and outer borders. (a,b – Courtesy: Prof
RC Gharami, Medical College and Hospital, Kolkata, India.)

●● Mid-borderline leprosy (BB) – Annular lesions of the BB Jessner lymphocytic infiltration of

leprosy characteristically assume a punched-out (Swiss- the skin
cheese) appearance: a sharp clear-cut inner margin with ●● It is a benign T-cell infiltrative disease that presents as
sloping outer edges (Figure 23.16e,f). asymptomatic, recurrent arciform or annular plaques
●● Borderline lepromatous (BL) – Annular lesions of BL over the face.
leprosy are characterized by sloping outer as well as ●● The lesion presents as solitary or multiple,
inner margins. However, the center of the lesion is more asymptomatic, erythematous papules and annular
infiltrated (and elevated) than the periphery, giving an plaques or nodules over the head, neck, and upper back
inverted saucer appearance (Figure 23.16g,h). (Figure 23.17).
●● DD: annular plaques of leprosy (granuloma annulare, ●● Individual lesions may disappear in several weeks to
annular sarcoidosis, necrobiotic xanthogranuloma, months, with complete resolution and no sequelae.
psoriasis). Recurrence is common.
530  Annular lesions

Figure 23.17  Annular erythematous plaque of Jessner’s

lymphocytic infiltration. (Courtesy: Dr. Hiral Shah, Baroda
Medical College and Hospital, Vadodara, India.)

●● DD: Granuloma annulare, plaque-type polymorphous

light eruption, lupus erythematosus tumidus, cutaneous
lymphoid hyperplasia.

Annular sarcoidosis Figure 23.18  Arcuate erythematous plaque of sarcoidosis

●● Sarcoidosis is a chronic multisystem inflammatory
on the face. (Courtesy: Dr. Rajib Gogoi, Guwahati, India.)
granulomatous disease that affects both skin as well as
internal organs. commonly affects the face, neck, and extremities. The
●● The disease has two peaks of onset, first between 25 nose, earlobes, and cheeks are common sites.
and 35 years and second between 45 and 65 years. It ●● In developing countries like India, lupus vulgaris is
commonly affects women more than men. commonly acquired after trauma, and hence trauma-
●● The disease is more prevalent in African-Americans prone areas are frequently affected.
than whites. ●● One of the most characteristic features of LV is peripheral
●● Annular lesions in sarcoidosis are uncommon. expansion with central healing (with scarring).
●● The lesion starts as papules, which may coalesce to form red- Because of this feature, LV frequently presents with
brown annular or serpiginous plaques with patchy scales. annular (when spread is uniform in all directions) and
●● The lesions appear indurated, with central clearing, and serpiginous or arciform (when expansion is not uniform)
they heal with hypopigmentation, atrophy, or scarring lesions. LV lesions developing into annular or arciform
(Figure 23.18). lesions is a valuable diagnostic clue (Figure 23.19a–c).
●● It commonly affects head and neck and is associated ●● LV commonly presents as single annular plaque with a
with chronic sarcoidosis. Sun-exposed areas are the raised hyperkeratotic border with central scarring. The
favored sites. lesion starts as red-brown grouped papule and nodule
●● Alopecia can result in the center of the lesion. that shows blanching on diascopy with apple jelly color.
●● DD: Secondary syphilis, discoid lupus erythematosus, The lesion expands by development of new papules at the
annular lichen planus, Hansen’s disease. periphery, which coalesce with the main plaque. As the
lesion progresses in one direction, previously involved
Lupus vulgaris (LV) parts tend to heal with scarring (Figure 23.19d,e).
●● LV is a chronic, progressive, common form of cutaneous ●● The disease has a slow rate of progression. The lesion
tuberculosis in adults. remains limited to a particular area for several years.
●● It commonly affects people with poor socioeconomic ●● Other clinical variants of LV are ulcerative type,
status and those with moderate to high immunity. vegetative type, papulonodular type, etc.
●● The disease affects all age groups and has female ●● DD: Leprosy, sarcoidosis, discoid lupus erythematosus.
preference compared to male in the ratio of 2–3:1.
●● It is acquired through hematogenous or lymphatic Annular lichen planus
spread or may develop after direct contiguous spread ●● It is an uncommon variant of lichen planus.
from other primary sites of infection, usually from ●● These lesions start as purple to polygonal papules
bone, joint, and lymph-node infection. The lesion that spread peripherally with central clearing. The
 Annular lesions  531

Figure 23.19  (a) Arciform crusted plaque in lupus vulgaris.

Note atrophic scarring. (b) Lupus vulgaris with erythema-
tous indurated border. (c) Lupus vulgaris on the hand with
hyperkeratotic arciform plaque. (d) Giant lesion of lupus
vulgaris affecting the contiguous areas of the face, neck,
and upper trunk. (e) Same patient with lupus vulgaris on
face. Note ectropion due to scarring. (c – Courtesy: Dr.
Shreya Singh, India; d,e – Courtesy: Dr. Rajesh Kumar
Mandal, North Bengal Medical College and Hospital, India.)
532  Annular lesions

raised border is violaceous in color, and the center is Basal cell carcinoma (BCC)
hyperpigmented. ●● Basal cell carcinoma is the most common benign non-
●● The most commonly affected sites are axilla, penis, melanocytic skin cancer, presenting mostly on sun-
extremities, groin, back, buttocks, flanks, neck, and exposed areas.
eyelids (Figure 23.20a–d). ●● The incidence of BCC is higher in white populations,
●● DD: Tinea corporis, porokeratosis (elevated thread but it can affect all races. Fitzpatrick skin types 1 and
border with central groove). type 2 are the ones with greater incidence.

Figure 23.20  (a) Lichen planus with annular violaceous

plaques with pigmented atrophic center. (b) Annular lichen
planus on the dorsum of the hand. (c) Annular lichen planus
on the penis. Note reticulate whitish areas surrounding the
lesion. (d) Annular lesions of lichen planus are common on
mucosa. (d – Courtesy: Dr. Rajeev Ranjan, Ara, India.)
 Annular lesions  533

●● Men have a higher tendency to develop the disease border. The lesion typically shows some
than women. areas of spontaneous regression with atrophy
●● Incidence is higher in older individuals aged 55 to and hypopigmentation. The tumor mostly
70 years. grows horizontally but may infiltrate deep
●● Annular lesions are appreciable in nodular, superficial with induration, infiltration, and ulceration
spreading and morpheaform types of BCC. (Figure 23.21d,e).
●● Nodular type – It is the most common subtype ●● Morpheaform type – The lesion presents as a pink
and starts as a shiny, pearly papule or nodule with to white or yellow waxy, sclerotic plaque that rarely
a smooth surface with telangiectasia. Over time, ulcerates. The lesion can be slightly elevated or
the lesion enlarges to form an elevated rolled, waxy depressed, fibrotic or firm with ill-defined borders.
border. In colored skin, the border is commonly The typical pearly border is absent for the most
pigmented. The center may get ulcerated and parts of the lesion. The lesion is more aggressive,
crusted. The lesion favor the face, especially the with extensive local destruction.
cheeks, nasolabial folds, forehead, eyelids, and ●● DD: Bowen’s disease (well-defined erythematous,
nose (Figure 23.21a–c). slightly scaly and crusted, non-infiltrated patch),
●● Superficial type – The lesion presents as annular lichen planus, seborrheic keratosis (tan to
well-circumscribed annular plaque with light-brown to black, sharply demarcated papule or
scales and pigmented, waxy papules on the plaque, “stuck on” appearance).

Figure 23.21  (a) Annular lesion of basal cell carcinoma with

pigmented, waxy border. (b) Large nodulo-ulcerative basal
cell carcinoma at the lateral canthus. Note waxy border,
prominent telangiectasia over the lesion and the crusted
ulceration. (c) Annular plaque with central ulcer covered with
crust. (Continued)
534  Annular lesions

centrifugally with an elevated border that corresponds

to cornoid lamella on histology.
●● The disease may be inherited in autosomal dominant
patterns except the punctate type.
●● Sun exposure, immunosuppression, and radiation
therapy act as aggravating factors.
●● The lesion starts as an asymptomatic keratotic papule
that expands over weeks or months to form an
annular plaque with keratotic border. The border has a
central groove (resembling the Great wall of China), a
diagnostic feature of porokeratosis.
●● Commonly known clinical types include
(Figure 23.22a) the following:
●● Porokeratosis of Mibelli – It appears during infancy
or childhood and affects boys more than girls. It
commonly affects the distal extremities but may be
localized or may be widespread. The lesions may
be solitary or multiple, giant (as large as 20 cm) or
itchy (especially flexural lesions). Clinically, the
Mibelli type shows typical lesions of porokeratosis
(described earlier) with the center of lesion showing
hyperpigmentation, hypopigmentation, atrophy, or
anhidrosis (Figure 23.22b,c).

Figure 23.21 (Continued)  (d) Superficial spreading type of

basal cell carcinoma. Note central scarring and pigmented
papules at the margin. (e) Large lesion of Superficial
spreading type of basal cell carcinoma. Part of the lesions
have evolved into nodular basal cell carcinoma. (c,e –
Courtesy: Dr Rajeev Ranjan, Ara, India.)

Porokeratosis
Figure 23.22  (a) Porokeratosis: annular lesion with
●● It comprises a rare group of diseases that show
atrophic center and keratotic margin with groove (black
abnormal epidermal keratinization and present as arrow). (b) Porokeratosis on the sole showing prominent
keratotic papules or annular plaque that expands groove. (Continued)
 Annular lesions  535

Figure 23.22 (Continued)  (c) Porokeratosis with annular erythematous, scaly plaques. (d) Lesions in disseminated superfi-
cial actinic porokeratosis are smaller and keratotic ridge is less prominent. (e) Disseminated superficial porokeratosis. Like
DSAP, the keratotic ridge is usually subtle. (f) Linear porokeratosis following Blaschko’s lines on the trunk. (Continued)
536  Annular lesions

patches and plaques along the lines of Blaschko

and has the highest tendency of malignant
transformation. In colored skin, lesions often appear
pigmented (Figure 23.22f,g).
●● Punctate porokeratosis – It appears during
adolescence and childhood as small, keratotic,
sometimes tender papules of 1–2 mm diameter
with raised peripheral rims over the palm and
soles. Clinically it resembles punctate keratoderma,
Darier disease, Cowden syndrome and arsenical
keratosis.
●● Porokeratosis palmaris et plantaris disseminata –
The lesions are similar to punctate porokeratosis,
but they affect the trunk, extremities, and mucous
membranes in addition to palms and soles. The
condition shows male preponderance and usually
arises during adolescence and childhood.
●● Porokeratosis ptychotropica – It presents as red to
brown pruritic papules and plaques involving the
intergluteal clefts and buttocks. Central coalescence
of lesions with peripherally scattered papules is also
seen.
●● DD:
●● Porokeratosis of Mibelli – annular lichen planus,
granuloma annulare
●● Linear porokeratosis – linear lichen planus, linear
epidermal nevus and incontinentia pigmenti.

Tuberculosis verrucosa cutis

●● It is a common form of cutaneous tuberculosis caused

by accidental inoculation of Mycobacterium tuberculosis

in previously sensitized or infected persons with
moderate to high cell-mediated immunity. Trauma
prone sites are commonly affected.
●● The lesion starts as asymptomatic small indurated

wart-like papules having an inflammatory rim that

slowly progresses in firm reddish-brown verrucous or
Figure 23.22 (Continued)  (g) Linear porokeratosis with hyperkeratotic plaques over months to years. Superficial
hyperkeratotic center on the dorsum of foot.
scaling and fissuring with intermittent purulent
(g – Courtesy: Dr. PC Das, Katihar, India.)
discharge is seen.
●● Sometimes, the lesion may undergo central clearing

with scarring and atrophy, giving an annular

●● Disseminated superficial actinic porokeratosis configuration.
(DSAP) – It is common in countries with high ●● DD: Majocchi granuloma, lichen simplex chronicus

sun exposure and is usually present in the third (well-circumscribed plaque resulting from repeated
to fourth decade of life. Patients develop several rubbing or scratching), warts, psoriasis, hypertrophic
small (about 1 cm) annular lesions distributed in a lichen planus.
bilaterally symmetric manner on sun exposed sites.
The border is not as prominent as that of the Mibelli Keratoacanthoma centrifugum marginatum
type, and the central part of the lesion is usually ●● It is a rare variant of keratoacanthoma.

pigmented (Figure 23.22d). ●● The disease commonly affects white adults in their fifth

●● Disseminated Superficial PK (DSP) – The lesions decade of life.

are similar to DSAP, but the lesions appear on sun- ●● It starts as solitary or multiple lesions that expand

covered parts too and the condition has an early by peripheral extension reaching up to 5–20 cm,
onset (Figure 23.22e). with raised, rolled borders and central healing (with
●● Linear porokeratosis – It arises during infancy or atrophic scarring). The margin may show erythematous
childhood as keratotic papules and erythematous papulonodular lesions with keratotic plugs; the
 Annular lesions  537

Figure 23.24  Chronic bullous disease of childhood with

tense blisters arranged in an annular configuration.

●● Patients present with bullae on the erythematous base

or on normal-looking skin, commonly over the lower
trunk, thighs, buttocks, groin, perioral, and scalp region.
New blisters appear at the periphery of crusted old bullae
and thus, the bullae appear arranged in annular or
rosette pattern (“string of pearls” or “cluster of jewels’).
Oral mucosa involvement is seen (Figure 23.24).
●● The classical clinical appearance (bullae in annular pattern)
may be less distinctive in the adult form. LABD usually
mimics dermatitis herpetiformis or bullous pemphigoid.
●● DD: Dermatitis herpetiformis (itchy, grouped
papulovesicles and excoriations over extensors, scalp
and trunk), bullous pemphigoid, bullous impetigo,
Stevens-Johnson syndrome.

Figure 23.23  Keratoacanthoma centrifugum marginatum Cutaneous larva migrans (creeping eruption)
with central atrophic scarring and erythematous nodules ●● It is a serpiginous or curvilinear eruption caused by
on the margin. The surface of the nodules are crusted and the accidental penetration and migration of animal
hyperkeratotic. (Courtesy: Dr. Shahid Hassan, Purnea, India.) hookworms (mostly by Ancylostoma braziliense or
Ancylostoma caninum) larvae through the epidermis.
latter mimic lesions of nodular keratoacanthoma ●● The disease is endemic the Caribbean, Central America,

(Figure 23.23). South America, Southeast Asia, and Africa.

●● Lesions usually appear on sun-exposed areas. ●● It occurs commonly in warmer climates, in the people

●● The tumor is benign and locally destructive. The lesion who walk barefoot over contaminated ground, and in
does not show a tendency of spontaneous resolution and manual workers, carpenters, plumbers, etc.
may progress to huge dimensions. ●● The disease onset is characterized by slight local itching

●● DD: Lupus vulgaris, squamous cell carcinoma, and small vesicles, followed by formation of serpiginous
botryomycosis. tracks. Intermittent stinging pain is present.
●● Each larva forms a separate tract and migrates at the
Linear IgA bullous dermatosis (LABD) rate of 1–2 cm/day. The linear lesions are interrupted
●● LABD (and its childhood counterpart, chronic bullous by papules formed from resting larvae. As the eruption
disease of childhood, [CBDC]) is an immune-mediated, advances, the earlier involved parts begin to fade. If left
subepidermal, vesiculobullous disease. untreated the larva dies in two to four weeks, leading to
●● It affects children two to three years of age, the average spontaneous resolution (Figure 23.25a).
being four and a half years, and adults above 60 years of ●● Larvae may remain quiescent for several days or months

age, with slight female preponderance. before they start migrating.

538  Annular lesions

●● DD: Inflammatory tinea corporis, larva currens

(“running” larva) (serpiginous erythematous papules
coalescing into urticarial linear plaques caused by
Strongyloides stercoralis).

Neutrophilic figurate erythema (NFE)9

●● NFE presents as an arcuate or annular lesion on

face, trunk, or extremities, without any predilection.

The lesion spreads centrifugally, and the active
margin is clinically characterized by erythema,
edema, vesicles and bullae, and purpura with some
scaling (Figure 23.25b,c). The condition may heal
spontaneously but may recur in summers.

Necrolytic migratory erythema (NME)

●● NME is a cutaneous hallmark of glucagonoma,
characterized by a triad of weight loss, diabetes
mellitus, and NME. Pseudoglucagonoma syndrome
has been reported in liver diseases and intestinal
malabsorption.
●● The cutaneous lesions start as intensely pruritic or

painful erythematous scaly patches with centrifugal

growth. The lesions are predominantly seen in the
periorificial area and in areas with increased pressure
or friction (intertriginous areas, buttocks, lower
abdomen, and distal extremities). The central part of
the lesion develops into flaccid blisters, and the latter
get ulcerated and crusted and eventually heal with
hyperpigmentation. As the central part of the lesions
heal, erythema spreads centrifugally and fresh blisters
at the advancing margins. These new blisters too heal in
a similar manner.
●● The disease runs a chronic relapsing course.

●● Associated mucosal changes include angular cheilitis,

glossitis, and stomatitis. Nails may show onychoschizia.

●● Systemic features found in NME are diarrhea,

neuropsychiatric changes, anemia, and

hypercoagulability.

Sneddon-Wilkinson disease (subcorneal pustular

dermatosis)
●● It is an autoimmune neutrophilic dermatosis with a

chronic relapsing course commonly affecting women

over 40 years of age. Children and adolescents are also
affected.
●● It presents as annular or polycyclic lesions commonly
Figure 23.25  (a) Linear and serpiginous vesicles in cuta-
neous larva migrans. (b,c) Annular erythematous scaly involving flexures of the extremities, intertriginous
patches in neutrophilic figurate erythema in an elderly areas, and trunk.
male. ●● The initial lesion presents as superficial sterile pustules

that progress to flaccid blisters with clear fluid in the

upper part and pus in the lower part. These pustules
●● It commonly affects lower extremities, but buttocks, coalesce and form annular lesions. The blisters rupture
hands, thighs, and perianal areas can also get involved. with superficial scaling, crusting, and secondary
●● Purulent manifestations can occur due to secondary hyperpigmentation. New lesions keep coming, and the
infections; erosions and excoriations are seen frequently. disease runs a chronic relapsing course.
●● Migratory pulmonary infiltrates and Löffler syndrome ●● DD: IgA pemphigus, pustular psoriasis, bullous
are rarely seen. impetigo.
 Annular lesions  539

Figure 23.26  (a) Annular pustular psoriasis with pustules surrounding central erythematous scaly area. (b) Arcuate lesions
of pustular psoriasis. (c) Annular pustular psoriasis. Note superficial nature of pustules. (b,c – Courtesy: Dr Hiral Shah,
Baroda Medical College, Vadodara, India.)

Annular pustular psoriasis ●● Lesions may clear almost completely in summer.

●● It is a rare variant of pustular psoriasis that has a ●● Lichenification of the popliteal and cubital fossa
chronic relapsing course with a good prognosis. with red, scaly face and eyelids are also seen due to
●● It affects mostly children, but adults are also coexistence of atopic dermatitis.
affected.
●● The patient presents with well-circumscribed, annular

lesions consisting of erythema, scaling, and pustules

at the periphery. The lesions enlarge in centrifugal
fashion, with central healing within several days.
Lesions heal within days but have a relapsing course
(Figure 23.26a–c).
●● DD: Tinea corporis.

Ichthyosis linearis circumflexa (ILC)

●● It is an inherited autosomal recessive disorder in

which migratory annular or polycyclic patches

occur.
●● The lesion appears in infancy or early childhood.

●● The lesion appears as severe generalized exfoliative

erythroderma at birth or shortly after. Later the lesion

becomes annular, polycyclic, or serpiginous with a
double-edged scaly border over the trunk and proximal
extremities. Lesions attain their maximum diameter Figure 23.27  Annular and serpiginous double-edged
in one week and resolve without atrophy, scarring, or scales in ichthyosis linearis circumflexa. (Courtesy: Dr. Anil
pigmentation (Figure 23.27). Patki, Pune, India.)
540  Annular lesions

Figure 23.28  (a) Bilaterally symmetrical annular scaly plaque with well-defined margins in erythrokeratodermia variabilis.
(b) Axillary lesion in erythrokeratodermia variabilis.

●● The patient also presents with bamboo hairs called ●● Hyperkeratosis with a spiny, hystrix-like appearance is
trichorrhexis invaginata. Hair may fracture below the present mostly on lower limbs.
surface of the scalp and the patient may appear bald. ●● The lesion shows improvement over time, and the
●● Ichthyosiform dermatitis, hair abnormality, and atopic condition stabilizes after puberty, following gradual
diathesis together are called Netherton syndrome. progression throughout infancy and childhood.
●● DD: Erythrodermic atopic dermatitis, Tinea corporis, ●● DD: Progressive symmetric erythrokeratoderma,
familial peeling skin syndrome. ichthyosis linearis circumflexa in Netherton syndrome,
psoriasis, epidermolytic ichthyosis, Greither syndrome,
Erythrokeratodermia variabilis MEDNIK syndrome.
●● It is an inherited autosomal dominant disorder that

presents as transient erythematous patches and REFERENCES

hyperkeratotic plaque.
●● Patients usually present at birth or within the first year 1. Jackson R. Circles: Concerning circles in nature, circles pro-
of life. duced by man, and circles in dermatology. Int J Dermatol
1994;33(11):818–825.
●● Lesions present as well-defined round to oval,
2. Sharma A, Lambert PJ, Maghari A, Lambert WC. Arcuate, annular,
geographic, circinate, annular, or target-like lesions and polycyclic inflammatory and infectious lesions. Clin Dermatol
that may coalesce into figurate patches that vary in size, 2011;29(2):140–150.
number, and position. Erythema is associated with a 3. Narayanasetty NK, Pai VV, Athanikar SB. Annular lesions in derma-
burning sensation. Individual lesions persist for only tology. Indian J Dermatol 2013;58:157.
4. Trayes KP, Savage K, Studdiford JS. Annular lesions: Diagnosis and
minutes to hours or may sometimes last for days. treatment. Am Fam Physician 2018;98(5):283–291.
●● Erythema is sometimes surrounded by a blanched halo.
5. Unwala R. Approach to the patient with annular skin lesions.
●● Lesions are aggravated by emotional stress, In: Stratman E, Ofori AO, editors. UpToDate. Waltham,
environmental heat or cold, mechanical friction, and Massachusetts: UpToDate, 2018. Available from https://www.
sun exposure. uptodate.com/contents/approach-to-the-patient-with-annular-
skin-lesions. Accessed on November 1, 2019.
●● Well-demarcated, hyperkeratotic or scaly, erythematous
6. Daroach M, Vinay K, Kumaran MS. Pseudo circinate balanitis: Great
plaque evolves simultaneously with or following the masquerader lives. Postgrad Med J 2019;95(1123):283.
development of migratory red scaly patches. The lesion 7. Singal A, Jakhar D, Kaur I, Pandhi D, Das S. Tinea pseudoimbricata
commonly affects the extensors of limbs, buttocks, and as a unique manifestation of steroid abuse: A clinico-mycological
trunk in a symmetrical pattern, similar to progressive and dermoscopic study from a tertiary care hospital. Indian
Dermatol Online J 2019;10:422–425.
symmetric erythrokeratoderma (Figure 23.28a,b). 8. Bhushan P, Thatte SS. Saucer lesions in leprosy: Anatomy of the
●● Thickening of palms and soles, with sparing of face,
controversy. Indian J Dermatol 2016;61:100–102.
scalp, and flexures, is present. 9. Wu YH, Hsiao PF. Neutrophilic figurate erythema. Am J
●● The surface of the lesion can be verrucous, velvety, Dermatopathol 2017;39(5):344–350.
and ridged. It may have collarette-like peeling or
psoriasiform scales. Hypertrichosis may be seen.
 24
Alopecia

PIYUSH KUMAR, PRIYA RAJBANSH

INTRODUCTION round or oval, spontaneously appearing, alopecic

patches. No changes in scalp skin are noted. Active
Hair, just like nails, is an ectodermal structure and is a visi- disease is characterized by a positive hair pull test
ble component of one’s self-image and personality. Since ages from the margin and the presence of exclamation hairs
past hair has been linked to masculinity and femininity, sex- (hairs tapered near the proximal end). Non-pigmented
uality, and attractiveness. No wonder alopecia impairs psy- hairs are spared, and their presence in alopecic
chological well-being and social functioning, and becomes patches is an important clinical clue (Figure 24.1a,b).
an issue of serious concern, despite causing no serious mor- Spontaneous regrowth of hairs in the affected area is
bidity in most cases. Hair may be lost in various genoderma- common and is seen over a period of a few months
toses and acquired dermatoses, systemic illness, traumatic (Figure 24.1c).
events and as a primary disease of hairs. Some of these ●● Various clinical patterns of AA are recognized:
processes can result in scarring and irreversible alopecia. ●● Patchy/localized AA – Solitary or multiple patches
Timely diagnosis and efficient treatment can prevent scar- of AA are noted. Sometimes, multiple patches may
ring alopecia in most cases. Though there are various tools coalesce to form a reticular pattern (Figure 24.1d,e).
for diagnosis, many conditions can be diagnosed clinically ●● Alopecia totalis – There is total or near total loss of
with reasonable certainty. The clinical diagnosis rests on scalp hairs (Figure 24.1f).
determination of non-scarring and scarring nature of alo- ●● Alopecia universalis – There is total or near total
pecia, age of onset, pattern and extent of involvement, hair loss of hair on all hair-bearing sites of the body
care and grooming practices, presence of various dermatoses (Figure 24.1g).
and telltale signs (skin lesions), and knowledge of systemic ●● Ophiasis type – This type is characterized by
illness, if any (Table 24.1). Additional clinical clues include band-like loss of hair along the temporal and lower
easy pluckability of hairs or presence of fragile, brittle hairs, occipital areas (Figure 24.1h).
scalp skin changes, etc. Some of the common and rare condi- ●● Sisaipho (ophiasis spelt backwards) type – In this
tions causing alopecia are discussed in this chapter.1–8 variant, there is extensive loss of hair that spares the
sides and the back of the head.
Alopecia areata ●● Diffuse AA – There is loss of hair from all over
●● Alopecia areata (AA) is a non-scarring, polygenic, the scalp in equal density without any patches
and autoimmune disorder that is characterized by its (Figure 24.1i).
relapsing and remitting course. The antibodies in AA ●● Nail involvement is seen in severe cases of AA and is
are heterogenous and target multiple structures of associated with poor prognosis. Nail findings include
anagen hairs. It can affect all hair-bearing areas but nail pitting, trachonychia, brittle nail, onycholysis,
most commonly affects scalp hairs. koilonychias, and, rarely, onychomadesis (Figure 24.1j).
●● AA does not show any racial variation and can occur ●● AA is found to have association with other autoimmune
at any age and in either sex, but mostly it is reported conditions such as vitiligo, diabetes mellitus,
to develop before the age of 40. Early onset AA is autoimmune thyroid disease, and atopy (atopic
considered when the mean age of presentation is dermatitis, allergic rhinitis, asthma) (Figure 24.1k).
between 5 and 10 years and is associated with poor ●● DD: Depends on clinical pattern of AA. DD of localized
prognosis. AA includes tinea capitis (h/o infection, lesion on
●● Patients may complain of pruritus or a burning scalp), and trichotillomenia. Diffuse AA mimics telogen
sensation. Patients present with single or multiple, effluvium.

DOI: 10.1201/9781351054225-75 541

542 Alopecia

Table 24.1  Clinical approach to alopecia

Nature of alopecia Features Diseases

Patterned Patchy/ • Non-scarring – alopecia areata (reticulate type), Tinea capitis, syphilitic alopecia
moth-eaten • Scarring – Pseudopelade of Brocq
Frontotemporal • Non-scarring – androgenetic alopecia (male), tractional alopecia, lupus hair
• Scarring – frontal fibrosing alopecia, morphea
Temporal • Non-scarring – temporal triangular alopecia
• Scarring – morphea
Vertex • Non-scarring – female androgenetic alopecia, lipedematous alopecia
• Scarring – central centrifugal cicatricial alopecia
Occipital • Non-scarring – transient neonatal alopecia, pressure alopecia
• Scarring – acne keloidalis
Temporal- • Non-scarring – alopecia areata (ophiasis pattern), tractional alopecia, hair
occipital region shaft disorders with increased hair fragility
Localized Non-scarring • With surface changes – Tinea capitis, nevus sebaceous
• Without surface changes – alopecia areata, trichotillomania (excoriations may
be seen), tumor alopecia, bubble hairs
Scarring Usually secondary cicatricial alopecia – burn, trauma, folliculitis, morphea, aplasia
cutis, etc.
Diffuse/ Non-scarring • Hypotrichosis without brittle hairs – telogen effluvium, anagen effluvium,
generalized loose anagen syndrome, short anagen syndrome
• Hypotrichosis with brittle hairs – hair shaft disorders
• Complete alopecia – diffuse alopecia areata, congenital universal atrichia,
atrichia with papular lesions, hereditary vitamin D-resistant rickets with alopecia
Scarring • With papules – lichen planopilaris, keratosis follicularis spinulosa decalvans,
acne keloidalis, alopecia mucinosa
• With plaque – discoid lupus erythematosus, lichen planus, alopecia mucinosa
• With nodules – Dissecting cellulitis, furunculosis
• With pustules – folliculitis decalvans, erosive pustular dermatosis of scalp,
furunculosis
• Without any skin lesion – central centrifugal cicatricial alopecia,
pseudopelade of Brocq, end stage of lichen planopilaris and tufted folliculitis

Tinea capitis ●● Tinea capitis results from invasion of hair structure by

●● Tinea capitis is a common dermatophytic infection of fungus; three modes of invasion are known – endothrix
scalp hair follicles and surrounding skin that mainly (arthroconidia seen within the hair shaft and cuticle is
occurs due to Trichophyton and Microsporum genera. intact), ectothrix (arthroconidia are seen outside the hair

Figure 24.1  (a) Single lesion of alopecia areata. Note

unremarkable scalp epidermis. (b) Alopecia areata affect-
ing medial part of eyebrow. Note gray hairs within alope-
cic patch. (Continued)
 Alopecia 543

Figure 24.1 (Continued)  (c) Regrowing hairs within the lesions of alopecia areata may be gray. (d) Alopecia areata in the
beard area. (e) Reticular alopecia areata. (f) Alopecia totalis progressing to alopecia universalis. Note that right eyebrow is
still appreciable. (g) Alopecia universalis with nail changes. (h) Ophiatic alopecia areata. (Continued)
544 Alopecia

Figure 24.1 (Continued)  (i) Diffuse alopecia areata.

(j) Regular, shallow nail pitting in a case of alopecia tota-
lis. (k) A child with alopecia areata of the occipital region
with segmental vitiligo and lichen planus. (i – Courtesy:
Dr. Tanumay Raychaudhury, Skin and Cancer Foundation,
The University of Sydney, Australia.)

●● The clinical picture consists of varying degrees of

scaling, hair loss, and inflammation and can be broadly
divided into two groups – non-inflammatory and
inflammatory types.
●● Non-inflammatory types (seborrheic form) – This
type of infection is usually caused by anthropophilic
organisms such as Microsporum audouinii or
M. ferrugineum. It clinically presents with single or
multiple, well-circumscribed or diffuse patches of
scaling and hair loss.
●● Gray patch – The characteristic presentation is
patchy, well-circumscribed, circular hair loss with
fine scaling. The hairs in affected areas appear
dull gray due to arthrospores coating the hairs
(Figure 24.2a,b).
●● Diffuse scale type – It presents with diffuse,
seborrheic dermatitis like scaling with decreased
hair density (Figure 24.2c).
shaft and cuticle is lost), and favus (hyphae and air bubbles ●● Black dot type – It is an endothrix infection, caused
are observed within the hair shaft). As a result of fungal by various species of Trichophyton (T. tonsurans,
invasion, the hair shaft becomes brittle and may break near T.violaceum). The characteristic finding is multiple
the scalp (“black dot”). Also, affected hairs may become patchy hair loss with fine scaling and grouped black
dystrophic and distorted (comma or corkscrew hairs). dots (Figure 24.2d,e). The black dots appear due to
●● The condition is particularly prevalent in the tropics, in breakage of hairs near the scalp. Black dot type is
children of African descent, and in children aged 3 to most non-inflammatory in nature, but some patients
14 years, without any sex predilection. Rarely, adults too may may develop follicular pustules and nodules.
be affected, and in adults, female preponderance is noted. ●● Inflammatory type – Such infections are usually
●● There may be a history of dermatophytic infection in caused by zoophilic or geophilic pathogens, such as
family members. M. canis, M. gypseum, and T. verrucosum. Patients
 Alopecia 545

may complain of itching, pain, and tenderness. The ●● Kerion – It is a severe clinical presentation of
severity of infection varies from follicular pustules to inf lammatory variant and occurs mainly due
kerion. Patients often have associated posterior cervical to T. verrucosum or T. mentagrophytes. Well-
lymphadenopathy. developed kerion presents as single or multiple,
●● Pustular form – It is a milder infection and presents painful, crusted, boggy, matted tender plaque
with patchy alopecia and scattered follicular papules or mass along with purulent discharge. Hairs
and pustules (Figure 24.2f). are matted in this region and easily pluckable.

Figure 24.2  (a) Gray patches of tinea capitis. (b) Gray patch of tinea capitis. (c) Diffuse scaling in tinea capitis (d) Black dot
type of tinea capitis. (Continued)
546 Alopecia

Figure 24.2 (Continued)  (e) Black dot tinea capitis with alopecia. (f) Inflammatory tinea capitis seen as alopecic patch with
erythematous papules, pustules and a few nodules over an erythematous skin. (g) Kerion as erythematous, boggy plaque
with alopecia. Surface is remarkable for pustules and crusts. (h) Kerion in a young boy. (a,b – Courtesy: Deverashetti
Srinivas, Nizamabad, India.)

The condition is potentially scarring, and if antihelix, and retroauricular regions are frequently
not treated in time, it may heal with scarring affected (ear sign).
alopecia (Figure 24.2g,h). ●● DD: depends on the clinical presentation of tinea capitis
●● Favus – It is also the inf lammatory variant of (Table 24.2).
tinea capitis, which is caused by T. schoenleinii
infection. The classical presentation is a thick,
yellow, cup-shaped crust (called scutula),
Table 24.2  Differential diagnosis of tinea capitis
composed of hyphae and skin debris. Adjacent
scutula may coalesce to form a large mass of Type of tinea
pale powdery crusts. Untreated cases lead to capitis Clinical differential diagnosis
cicatricial alopecia.
Gray patch Scalp psoriasis, trichotillomania,
●● Inflammatory tinea capitis, especially kerion, may
Diffuse scaly type Seborrheic dermatitis, scalp psoriasis
be associated with dermatophytid reactions –
Mild inflammatory Bacterial folliculitis, pustular psoriasis
eczematous lesions consisting of papules, vesicles, and
type
pustules and scaling. The dermatophytid reaction may
be localized or generalized, and when localized, helix, Kerion Folliculitis decalvans, bacterial abscess
 Alopecia 547

Syphilitic alopecia9 ●● Clinically, four morphological types are recognized:

●● Syphilitic alopecia (SA) is non-scarring, ●● Moth-eaten or patchy – This is the most common
inflammatory alopecia caused by immune response variant of SA and typically presents with patchy loss
against Treponema pallidum that have invaded the of hair of irregular shape and size over the parieto-
hair bulge epithelium and peribulbar capillaries. occipital region. These patches are not completely
It may be seen with mucocutaneous lesions of devoid of hairs, and margins of lesions are not well
secondary syphilis (symptomatic) or, rarely, without defined. The scalp skin does not show any sign of
any mucocutaneous features of secondary syphilis inflammation. This variety of SA can also occur on
(essential SA). other hair-bearing areas of body such as the beard,
●● It can occur in any age group but is most frequently axilla, pubis, legs, and trunk (Figure 24.3a,b).
found in sexually active people. ●● Diffuse SA – It is characterized by diffuse loss
●● There may be a history of unprotected sex with multiple of hair without any obvious patches and mimics
partners, and painless genital ulcer. telogen effluvium (Figure 24.3c).

Figure 24.3  (a) “Moth-eaten” alopecia in secondary

syphilis. (b) Moth-eaten alopecia in secondary syphilis.
(c) Diffuse hair loss in a female with secondary syphilis.
(d) Loss of lateral eyebrows in secondary syphilis. Note
eroded papule on the ear and hyperpigmented patches
on the face. (a – Courtesy: Prof. Santoshdev P Rathod,
Smt. NHL Municipal Medical College, SCL General
Hospital, Ahmedabad, India; b – Courtesy: Dr. Shekhar
Neema, Armed Forces Medical College, Pune, India.)
548 Alopecia

●● Mixed type – Here the features of both patchy and

diffuse alopecia are found.
●● Alopecia of the eyebrows (“omnibus sign”) – This
involves loss of the lateral third of eyebrows
(Figure 24.3d).
●● In primary syphilis, alopecia is extremely rare and is found
in only cases having primary chancre over the scalp.
●● Non-scarring alopecia is seen in secondary syphilis,
whereas scarring alopecia is a feature of tertiary syphilis.
●● The different clinical variants of SA are associated
with various cutaneous and systemic findings, such
as malaise, low-grade fever, lymphadenopathies,
and anorexia, and cutaneous findings such as
generalized non-pruritic papulo-squamous rashes
predominantly over trunk. Other finding includes
palmoplantar pigmented lesion, mucosal ulceration,
and condyloma lata.
●● DD: Tinea capitis (history of scalp infection), telogen
effluvium, trichotillomenia (hair texture changed,
fractured hairs), alopecia areata.

Androgenetic alopecia (male pattern baldness) and

female androgenetic alopecia/female pattern hair
loss (FAGA/FPHL)
●● Androgenetic alopecia (AGA) occurs due to genetically

determined sensitivity of hair follicles of scalp to the

androgen dihydrotestosterone (DHT). Under the
influence of DHT, there is progressive thinning of hairs,
characteristically over the frontal and vertex region.
●● In AGA, the anagen phase of hairs is shortened and

there is gradual, progressive conversion of terminal

hairs (large, thick, pigmented) into vellus hairs (short,
fine, non-pigmented) in the characteristic pattern
(involvement of vertex and frontal region with sparing of
occipital and parietal regions, called Hippocratic wreath).
●● AGA is most common in Caucasian people, followed

by Asians. It normally affects middle-aged and elderly

people but can occur any time after puberty and,
rarely, in prepubertal children (six to ten years of
age). Its incidence increases with increasing age. Men
outnumber women.
●● There is gradual bitemporal recession of anterior

hairline (professor’s angle), with loss of hairs on vertex

in affected men. Positive family history is present
(Figure 24.4a–c).
●● The Hamilton-Norwood classification is used to

evaluate the severity of male pattern hair loss.

●● In females, FAGA results in diffuse hair loss over the

crown (Figure 24.4d–h). There is a gradual thinning

of the central hair with widening of the part width,
rather than an area of marked baldness. The part
width is markedly increased in the frontal region, with
Figure 24.4  (a) Bilateral temporal recession of anterior
progressive decline in width posteriorly (Christmas tree
hairline and reduced density of hairs on the vertex in
pattern). The frontal hairline is often preserved. The androgenetic alopecia. (b) Advanced androgenetic alope-
clinical diagnosis of FAGA, especially in early cases, cia in a young male. (c) Androgenetic alopecia with diffuse
may be difficult, and trichoscopy is a very valuable tool hair loss in frontal and vertex region. Hairs from parietal
for making a diagnosis. regions are relatively preserved. (Continued)
 Alopecia 549

Figure 24.4 (Continued)  (d,e) Female androgenetic alopecia in a middle-aged lady. (f,g) A middle-aged lady with
advanced female androgenetic alopecia. Top view (f) and lateral view (g). (h) Advanced female androgenetic alopecia with
extensive hair loss.
550 Alopecia

Table 24.3  Traumatic hair styling by different population ●● TA starts with perifollicular erythema, which
progresses to follicular papules and pustules formation.
Population who commonly
Other findings are hair casts, reduction in hair density
Traumatic hair styles practice it
and replacement with vellus hairs, and the presence of
Braids, cornrows, hair Afro-Caribbean men, broken hairs in the affected areas. If left untreated, the
weave women, and children disease may progress to cause scarring alopecia. TA to
Dreadlocks African men and women, start with is non-scarring alopecia and may progress to
Indian sadhus, Western scarring alopecia if patients do not adopt non-traumatic
fashion and counterculture hair care practices.
Ponytail Asian and Hispanic women, ●● The pattern of hair loss in TA is variable and correlates
athlete women with distribution of the traction. The following patterns
Turban Sikh men are commonly observed:
Hijab Muslim women ●● Marginal TA – The hair density is reduced over
Bun Indian women, ballerinas the frontotemporo parietal area. A strip of thin
Nurse’s cap Nurses in developing and hair (“the fringe sign”) is retained at the margin of
Asian countries alopecic patch (Figure 24.5a–e).
Excessive long hairs Women

●● The severity of FAGA can be assessed by the Ludwig

classification and Sinclair scale.
●● DD: Telogen effluvium (equal density of hair over all areas
of scalp), loose anagen syndrome (specific age group),
diffuse alopecia areata (exclamation sign on dermoscopy).

Traction alopecia10
●● Traction alopecia (TA) is an acquired disorder of gradual,

progressive hair loss resulting from traumatic hair styles

(Table 24.3) that cause repetitive pressure or tension on
the hair roots. It has been observed in many races.
●● It can occur in any age group, and the incidence increases

with age, but young adult females predominate.

●● There may be a history of use of chemical relaxers,

extensions, and curlers. Also, affected patients may have

a history of chronic use of hair wefts, hair pins, or clips.
●● Also, patients may give a history of headache that is

relieved when hairs are loosened.

Figure 24.5  (a) Traction alopecia with fringe sign. (b) Traction alopecia in a Sikh male. (c) A Sikh male wearing turban regu-
larly has developed marginal traction alopecia of parietal region. (Continued)
 Alopecia 551

●● The non-marginal variant of TA has also been

reported after hair wefts, hair pins, or clips use.
Some notable examples of non-marginal TA include
the following:
a. Horseshoe pattern of T – It is seen in persons
using wefted hair extensions.
b. Chignon alopecia – It occurs in women with
long hair making a tight ponytail or tight bun.
c. Band like alopecia – A band-like hair loss in
the frontotemporal line, commonly seen in
Sikhs because they tie their hair in a bun over
the vertex.
●● The presence of swelling and tenderness at the site
of tight braiding is an ominous sign and indicates
impending scalp necrosis.
●● DD: Androgenetic alopecia, telogen effluvium,
trichotillomenia.

Lupus hair
●● Lupus hair is a distinct non-scarring alopecia seen

in patients with chronically active systemic lupus

erythematosus (SLE). Some authors consider it a variant
of telogen effluvium, while others believe it a result of
retarded hair growth.
●● Lupus hair presents as short, fragile, coarse, and

dry hairs along the anterior hairline. Sometimes,

background erythema may be appreciated. Other
peripheral scalp regions too can be affected but are less
obvious (Figure 24.6a–c).
●● The presence of lupus hair in SLE suggests disease

activity and exacerbations.

Frontal fibrosing alopecia11

●● Frontal fibrosing alopecia (FFA) is a variant of LPP

and is clinically characterized by progressive band-

like hair loss affecting the frontotemporal zone of
scalp.
●● The prevalence is not known, but the disease appears

to be common among people from Europe, the United

Figure 24.5 (Continued)  (d) Traction alopecia in a young

girl who keeps hairs in a tight bun. (e) Marginal trac-
tional alopecia is not always restricted to frontotemporal
region. (b – Courtesy: Dr. Dhiraj Kumar, Patna, India;
e – Courtesy: Dr Shekhar Neema, Armed Forces Medical Figure 24.6  (a) Hair loss with frontal, short, broken hairs
College, Pune, India.) (lupus hairs) in systemic lupus erythematosus. (Continued)
552 Alopecia

Figure 24.7  (a) Frontal fibrosing alopecia in a 56-year-old

male. (b) Frontal fibrosing alopecia in a female with lichen
planus pigmentosus. (a – Courtesy: Dr. Hiral Shah, Baroda
Medical College, Vadodara, India.)

Figure 24.6 (Continued)  (b) Lupus hairs. (c) Extensive

●● These recessions of hair can extend up to the
hair loss during an exacerbation of systemic lupus preauricular and retro-auricular regions of scalp.
erythematosus. The frontal recession varies from 1 cm to 6 cm
(observing contraction of the frontalis muscle
States, and Japan. Typically, postmenopausal women allows differentiation of forehead from scalp and
are affected. Affection of young female or males is allows quantification of hairline regression) and can
uncommon. extend up to occipital region. The affected skin is
●● FFA is characterized by asymptomatic, progressive pale, shiny and devoid of follicles (including vellus
recession of the frontotemporal (anterior) hairline due hairs), and hairs at the edge may show erythema and
to scarring alopecia, along with the loss of eyebrows. perifollicular papules in active stage of disease. The
This recession of the hairline is typically bilateral and hair pull test is positive on the affected area during
symmetrical (Figure 24.7a,b). the active stage of disease.
 Alopecia 553

●● Presence of “lonely hair” is another important clinical ●● The diagnostic criteria has been proposed by Inui et al.
clue. In the alopecic area, isolated terminal hair TTA is diagnosed if the following features are present:
may persist and may show perifollicular erythema. ●● A triangular or lanceolate alopecic patch located
Sometimes, terminal hairs at the original hairline may over the frontotemporal region or other regions of
be spared (pseudo fringe sign) and are associated with the scalp
good prognosis. ●● Trichoscopy showing normal hair follicles with
●● Alopecia of eyebrows sometimes precedes the frontal vellus type hair
alopecia and typically involves the lateral third of
eyebrows.
●● Patients may have associated lichen planus pigmentosus.
●● DD: Androgenetic alopecia, alopecia areata.

Temporal triangular alopecia (congenital triangular

alopecia, Brauer nevus)
●● Triangular temporal alopecia (TTA) is a non-scarring

and patterned hair loss characterized by replacement of

normal terminal hairs by vellus hairs. The condition is
non-progressive and remains unchanged throughout life.
●● TTA can be present since birth or be acquired

thereafter. The onset is usually in childhood, but, rarely,

adults too may present with recent onset of disease.
●● As the name suggests, the classical site of affection

is the frontotemporal area, but it can occur over

temporoparietal or occipital area too.
●● Patients present with unilateral or, uncommonly,

bilateral, triangular, oval, or lanceolate (with the tip of

lancet points superiorly and posteriorly) patches of hair
loss, with or without small islands of dark, terminal
hairs within the patch. The affected area appears
hairless, but fine vellus hairs may be appreciated by the
naked eye or on trichoscopy. The underlying skin shows
no visible changes (Figure 24.8a–c).

Figure 24.8  (a) Lanceolate-shaped alopecic patch in temporal triangular alopecia. (b) Temporal triangular alopecia in a child.
(c) Temporal triangular alopecia. (a,b – Courtesy: Dr. Anil Patki, Pune, India; c – Courtesy: Dr. Ganesh Avhad, Mumbai, India.)
554 Alopecia

●● Absence of fractured hairs, black dots or yellow dots

with preserved follicular orifices on trichoscopic
examination
●● Lack of significant hair growth six months after
confirming the presence of vellus hairs
●● DD: Alopecia areata (exclamation hair on dermoscopy),
pressure alopecia, tinea capitis (history of infection),
trichotillomenia (texture of hair changed).

Lipedematous Alopecia
●● This disorder is characterized by a thick, boggy scalp

due to expansion of the subcutaneous fat layer. The

condition is most commonly seen in women with
colored skin, and the vertex is usually affected. The
condition may be associated with vertex alopecia.

Central centrifugal cicatricial alopecia (CCCA) (hot

comb alopecia, follicular degeneration syndrome)
●● CCCA is an idiopathic, primary, scarring alopecia

characterized by progressive loss of hair starting at

crown or vertex region and progressing in a centrifugal
manner.
●● It can affect any population but is predominantly seen

in colored skin, with a female preponderance The

disease typically starts in the second or third decade of
life.
●● A history of hair straightening, traumatic hair styles,

hair lengthening, etc., may be present.

●● The condition begins with mildly pruritic or painful

hair loss over the crown or vertex of the scalp, which

gradually progresses in a centrifugal manner and
extends up to the side of the scalp. However, the severity
of the disease remains the maximum at the vertex.
The condition is characterized by slowly progressive
scarring alopecia, and the symmetrical forward
progress of hair loss resembles female pattern hair loss
(Figure 24.9a,b).
●● The scalp skin appears shiny, shows nil/minimal

inflammation or scaling, and may show a few short,

brittle hairs. Also, as in much of scarring alopecia,
polytrichia may be seen (a group of hairs coming out of
one follicle).
●● A minority of patients suffering from rapidly

progressive disease or bacterial super infection can have

pustules and crusts.
●● DD: Female pattern hair loss, male pattern hair loss,

lichen planopilaris, telogen effluvium. Figure 24.9  (a) Central cicatricial scarring alopecia –
vertex of scalp is affected and alopecic patch is progress-
Neonatal occipital alopecia (NOA) (transient ing peripherally. (b) Extensive scalp involvement in central
neonatal hair loss) cicatricial scarring alopecia. (b – Courtesy: Dr. Shashank
●● It is non-scarring alopecia caused by synchronized Bansod, Nagpur, India.)
physiologic telogen effluvium after a prolonged anagen
phase (under the influence of maternal hormones) since ●● NOA characteristically involves the occipital area. The
the prenatal period. Earlier, the condition was attributed frontotemporal region is now increasingly recognized to be
to friction from sleeping in a supine position. affected simultaneously or independently (Figure 24.10a,b).
●● It can affect any race and typically occurs after two to ●● Affected infants present with a linear band or oval area
three months after birth. of alopecia over the occipital area. The lower end of this
 Alopecia 555

Figure 24.10  (a) Band-like neonatal occipital alope-

cia. (b) Neonatal occipital alopecia. (c) Focal alopecia
and ulcer in a child using cochlear implant. (d) External
unit of cochlear implant causes continuous pressure.
(c,d – Courtesy: Dr. Hiral Shah, Baroda Medical College,
Vadodara, India.)

alopecic patch has a sharp margin, but the upper margin ●● It usually presents as a solitary, roughly oval patch at
merges with the hairs of vertex and is less distinct. the site of greatest pressure, usually the upper occiput
●● The condition is self-limiting, and spontaneous (Figure 24.10c,d).
resolution is noted. ●● DD: Alopecia areata, trichotillomania.
●● DD: Alopecia areata, temporal triangular alopecia,
telogen effluvium. Acne keloidalis
●● The condition is mostly seen in males and in people
Pressure alopecia with colored skin.
●● Pressure alopecia results from ischemic damage to the ●● It is clinically characterized by follicular pustules

scalp in patients undergoing prolonged surgery or in and smooth, firm papules on the occipital scalp and
bedridden patients. The alopecia may be non-scarring posterior neck. With time, lesions heal with scarring
or scarring, depending on the severity of ischemia. alopecia (Figure 24.11a,b).
556 Alopecia

●● Occasionally, papules may coalesce to form hairless,

keloid-like plaques.

Nevus sebaceous (see Chapter E20: Scalp)

●● Nevus sebaceous presents with hairless, yellowish

plaque on the scalp in infants or children. Later lesions

assume a verrucous appearance and may develop
various tumors.

Trichotillomania
●● Trichotillomania is a psychocutaneous disorder

(an impulse control disorder) characterized by an

irresistible urge to pull out one’s hair, leading to
alopecia. Patients often deny the history of hair pulling.
●● There may be associated trichophagia and

trichobezoars.
●● Affected patients may have other associated psychiatric

disorders such as anxiety disorder, attention-deficit

disorder, obsessive-compulsive disorder, mood disorder,
tic disorder, and body-focused repetitive behaviors (e.g.,
skin picking, nail biting).
●● It can occur in any age group but is more common

among the children. The age of onset usually lies

between 5 and 12 years of age, without any gender
predilection. However, trichotillomania can also have
adult onset, which is more common in females.
●● Patients may complain of some kind of discomfort in

the affected area, such as itching, pain, etc. There may

be a history of disease aggravation during the stressful
periods. Also, there may be a history of distress,
impairment of social or academic functioning, and
impact to family relationships.
●● The scalp is the most affected site, but other areas,

including eyebrows and eyelashes, can be involved.

●● The presentation is variable – it ranges from localized

patches to complete scalp involvement, and from

decreased density to complete alopecia. Patients
commonly present with single or multiple, geometric-
shaped patches with incomplete, non-scarring alopecia.
The hairs in the affected area are characteristically of
different lengths in an irregular pattern. The hairless
patch is usually seen over easily accessible sites. In
severe cases, complete scalp involvement and even
involvement of eyebrows and eyelashes may be noted
(Figure 24.12a–c).
●● Clinically, different zone of hairs can be identified:

●● Long normal texture hair in the periphery of scalp

●● Patchy hair loss with or without spots of bleeding
●● Short, thin, regrowing hairs within the patch
●● Sometimes the vertex of the scalp may be involved

and marginal hairs are spared; such a presentation

is called tonsure trichotillomania or Friar Tuck
(a fictional character in legends about Robin Hood)
Figure 24.11  (a) Keloidal lesions and scarring alo- sign (Figure 24.12d).
●● The hair pull test is negative.
pecia in acne keloidalis. (b) Acne keloidalis nuchae
with scarring alopecia. (a – Courtesy: Deverashetti ●● Clinical diagnosis in suspected cases can be confirmed

Srinivas, Nizamabad, India; b – Courtesy: Dr PC Das, by creating a “hair growth window” – a small area
Katihar, India.) in the affected area is shaved on a weekly basis to
 Alopecia 557

Figure 24.12  (a) Extensive hair loss in trichotillomania. (b) In same patient, hairs in mid-occipital area are spared. (c) Different
lengths of hairs in trichotillomania. (d) Friar Tuck type of trichotillomania. (e) Trichoteiromania causing patchy alopecia in an
elderly female. (f) Trichoteiromania in an elderly female. Note prurigo nodularis like lesions on the scalp. (Continued)
558 Alopecia

Table 24.4  Causes of secondary scarring alopecia

Causes Example
Physical and Burns (Figure 24.13a,b), acid or
chemical injury alkali burns, ionizing radiation
Infections Bacterial (Figure 24.14a), viral and
fungal infections
Granulomatous Sarcoidosis
diseases
Autoimmune and Vesicobullous disorders, morphea
inflammatory (Figure 24.14b,c), systemic
diseases sclerosis, graft-versus-host
disease, psoriasis,
Benign and Primary tumors, metastases,
malignant lymphoproliferative diseases,
tumors organoid nevi
Genodermatoses Aplasia cutis congenita, ectodermal
dysplasias, epidermolysis bullosa
Others Amyloidosis

Tumor alopecia
●● Many benign or malignant tumors on the scalp may

replace normal appendageal structures during their

growth and may cause localized alopecia (Figure 24.12g).
Figure 24.12 (Continued)  (g) Benign cystic lesion causing ●● Alopecia neoplastica refers to hair loss seen in

localized alopecia. (e – Courtesy: Prof. Bhushan Madke, metastatic scalp tumors.

Jawaharlal Nehru Medical College, Datta Meghe Institute
of Medical Sciences, India.) Secondary scarring alopecia
●● Secondary scarring alopecia results from non-specific

damage to the tissue, including hair follicles. The

demonstrate the dense regrowth of hair within the
conditions responsible for secondary scarring alopecia
affected area. DSM-5 criteria are used to diagnose this
are diverse and are summarized in Table 24.4.
condition (Box 24.1).
●● It needs to be differentiated from other compulsive
hair disorders such as trichotemnomania (cutting or
shaving hairs with an instrument), trichoteiromania
(rubbing of hairs, leading to fracturing of hair shafts)
(Figure 24.12e,f), trichorrexomania (cutting of hairs
by nails), and trichodagnomania (compulsive biting of
hairs of accessible areas).
●● DD: Tinea capitis, alopecia areata, traction alopecia.

BOX 24.1: Diagnostic criteria (DSM-5) for

trichotillomania

1. Recurrent habit of pulling one’s own hair, eventu-

ally leading to alopecia
2. Repeated attempt to reduce or stop the hair pull-
ing behavior
3. The hair pulling behavior leads to clinically signifi-
cant distress, impairment of social, occupational,
or other important area of functioning
4. Repetitive hair pulling or alopecia is not attributed
to other medical or dermatological condition
5. Hair pulling cannot be better explained by the
symptom of another mental disorder Figure 24.13  (a) Scarring alopecia due to burn. There are non-
healing ulcers and crusted ulcers on the scalp. (Continued)
 Alopecia 559

Aplasia cutis congenita (ACC)

●● ACC is a rare cutaneous malformation that is

characterized by the congenital absence of skin. The

scalp is the most commonly affected site.
●● ACC presents as solitary (or rarely, multiple), well-

demarcated, circular, linear, oval, or stellate atrophic

patches with hair loss on the scalp, usually lateral to
midline. The size of the lesion varies from 0.5–10 cm.
Often there is distorted hair growth around the scalp
lesion (hair collar sign).
●● ACC can be membranous or non-membranous,

with membranous being the most common type

(Figure 24.15).
●● If the lesion occurs in first week of gestation, the

lesion heals completely before birth and presents

as atrophic, membranous, and fibrotic patch of
alopecia.
●● ACC can also present as ulcer of variable depth and
Figure 24.13 (Continued)  (b) Scarring alopecia follow-
heals spontaneously within the first year of life
ing burn.
●● DD: Traumatic ulcer.

Telogen effluvium (TE)

●● Telogen effluvium (TE) occurs with excessive shedding

of normal telogen hair in response to physiologic and

pathologic stress (Table 24.5). Drugs such as retinoids,
anticoagulants, antithyroid, cimetidine, enalapril and
discontinuation of oral contraceptive pills too are
associated with TE.
●● Chronic telogen effluvium (CTE) is a diffuse hair loss

of the scalp that persists for more than six months and

Figure 24.14  (a) Focal scarring alopecia following furuncle. (b) A burned-out case of morphea with facial lesions and scar-
ring alopecia. (c) Patchy scarring alopecia in the same case (Figure 24.14b).
560 Alopecia

is commonly seen in middle-aged females. The disease

may run a chronic relapsing and remitting course over
several years.
●● TE normally affects women between 30 and 60 years of
age but can occur in any age and sex.
●● TE usually develops approximately two to three months
after the triggering event.
●● The patient complains of excessive shedding of
hairs (Box 24.2) while combing or washing. On
examination, there is excessive thinning of scalp
hairs that involves the whole scalp. Bitemporal
recession is seen. Characteristically, any patch
or area with complete alopecia is not noted
(Figure 24.16a,b).
●● The shed hairs have a visible depigmented club shaped
bulb.
●● Spontaneous recovery is common and is seen
usually within six months if the precipitating factor
has been taken care of. Short regrowing hairs
are found on frontal and other areas of scalp in
resolving TE.
Figure 24.15  Aplasia cutis with focal alopecia, atrophy, ●● Both hypothyroidism and hyperthyroidism can
and crusted ulcer. (Courtesy: Dr. Hiral Shah, Baroda cause TE. Hairs become sparse, dry, and brittle. In
Medical College, Vadodara, India.) hyperthyroidism, hairs become extremely thin and
sparse.
Table 24.5  Different types of telogen effluvium
●● Nutritional deficiency of iron and zinc may be
associated with TE; however, studies have shown
Type of conflicting results.
telogen ●● DD: Androgenetic alopecia (hair loss follows a pattern:
effluvium Feature Examples more thinning over frontal and vertex area), diffuse
Immediate Premature apoptosis of Physiologic or alopecia areata, anagen effluvium.
anagen hair bulb leads anagen pathologic
release hairs into catagen and stress, high fever
telogen phase.
Delayed Hairs are in prolonged Postpartum TE BOX 24.2: Objective assessment of
anagen anagen phase during (telogen
hair loss
release gestation and gravidarum)
decreased hormones
There are certain parameters to evaluate the hair loss
level at delivery cause
in TE:
them to enter catagen
and telogen phase. ●● Hair pull test – gently grasp about 40 hairs
Immediate Telogen phase is Minoxidil use between the thumb and index finger and pull;
telogen shortened. a count of four to six club hairs is abnormal for
release unwashed hair whereas two to three hairs is con-
Delayed Hairs remain in Seasonal hair loss, sidered abnormal for washed hair.
telogen prolonged telogen hair loss ●● Trichogram (forcible hair pluck) – shows the mix-
release phase and are shed associated with ture of telogen and anagen hairs, and tells about
when finally teloptosis travel from the anagen to telogen ratio. If the quantity of
(termination of low-daylight to telogen hairs exceeds 20% of total hair, then it is
telogen phase with high-daylight considered abnormal shedding.
hair shedding) occurs. areas ●● One-minute combing test – the patient is asked
Short Anagen phase is Hereditary to comb their hair for one minute before washing
anagen shortened. hypotrichosis, on three consecutive days, over a cloth or white
phase ectodermal floor. A count of greater than 10 hairs found on the
dysplasia, short cloth or floor is abnormal, where as in TE the count
anagen syndrome increases to 40 to 50 hairs.
 Alopecia 561

syphilis, pemphigus vulgaris, radiation, malnutrition

due to inflammatory insult to the hair matrix cells.
●● The condition does not show any racial or gender
predilection.
●● The patient presents with shedding of hairs within two
week of chemotherapy. The shed hairs are anagen hairs –
full pigmented hairs with inner and outer root sheath. Here
the hair loss doesn’t follow any pattern and involves the
whole scalp. Scalp skin examination is characteristically
unremarkable. Other sites, such as eyebrows, armpits and
the genital area, may be affected (Figure 24.17a–c).

Figure 24.16  (a) Telogen effluvium with reduced hair den-

sity. (b) Chronic telogen effluvium in a middle-aged lady.

Anagen effluvium (AE) (chemotherapy-induced

alopecia)
●● It is non-scarring alopecia that commonly occurs after

administration of chemotherapeutic drugs. These drugs

cause impairment of mitotic or metabolic activity of
hair matrix cells, leading to loss of anagen hairs. In
addition, there is constriction of hair shafts (Pohl-
Pinkus constriction).
●● AE can follow administration of chemotherapeutic

agents (e.g., doxorubicin, nitrosoureas,

cyclophosphamide) and other drugs (isoniazid, Figure 24.17  (a) Anagen effluvium due to azathioprine in a
levodopa, colchicine, and cyclosporine), or may also lady with vitiligo vulgaris. (b) Clump of shed hairs brought
occur in conditions like alopecia areata, secondary by same patient. (Continued)
562 Alopecia

Figure 24.18  Loose anagen hair syndrome with reduced

Figure 24.17 (Continued)  (c) Another case of anagen hair density and length.
effluvium due to azathioprine.

●● The condition is reversible, and hair regrowth is noted Short anagen syndrome (SAS)
upon discontinuation of the offending agent. ●● It is a congenital hair condition characterized by
●● DD: Telogen effluvium (no history of chemotherapy), persistently short hair since birth, which occurs due to a
androgenetic alopecia (follows a characteristic pattern), shortened anagen phase.
loose anagen syndrome (particular age group involved), ●● Females are more commonly affected, but it can affect
trichotillomenia. males also.
●● Typical presentation is in two- to five-year-old white
Loose anagen hair syndrome (LAHS) girls. The condition is apparently rare in Africans and
●● LAHS is a rare, sporadic, or autosomal dominant Asians.
condition causing premature keratinization of the ●● The parents complain that the child has very short hairs
outer root sheath, inner root sheath, and cuticle of that do not grow long even though the hairs have never
the hair shaft. The end result is a malformed hair that been cut.
can be extracted painlessly with little effort. The hair ●● The hairs in these children are normal in texture and
assumes a distorted shape (triangular, quadrangular, or density.
flattened) with a longitudinal groove, noted on electron ●● The hair pull test is negative in SAS, which helps to
microscopy. differentiate it from other hair shedding conditions such
●● The condition is common in whites and the as telogen effluvium and loose anagen syndrome, where
most common presenting age group is children, the hair pull test is positive.
though adults too may present with LAHS. Female ●● DD: Telogen effluvium, loose anagen syndrome.
preponderance is noted. Parents complain of thin,
sparse hair, less growth of hair that requires less Hair shaft disorders (Table 24.6)
frequent haircuts, or dry lusterless unmanageable Diffuse alopecia areata (alopecia areata incognita)
hairs of the child. There may be a history of easily and ●● Diffuse AA is a rare clinical variant of AA,

painlessly pluckable hairs. characterized by abrupt onset of extensive hair loss. The
●● Clinical examination reveals dry, lusterless, thin condition is usually seen in young adults and shows a
hairs and diffuse (or patchy) non-scarring alopecia female preponderance. Here, there is generalized loss
without any inflammation or scalp skin changes. of hair involving the entire scalp over a period of a few
Gentle traction results in painless extraction of hairs, weeks. In this condition the non-pigmented hairs are
which, on light microscopy, are shown to be anagen initially spared, so the patient may complain of rapid
hairs with distorted hair bulbs (bent at an acute angle graying of hairs. The hair pull test is positive.
to hair shaft) and baggy, ruffled cuticle (“rumpled ●● Along with the thinning of scalp hair, nail findings

sock” appearance). Hairs may be of varying lengths can also be appreciated in this condition. Nail findings
(Figure 24.18). include regular nail pitting, brittle nails, trachonychia
●● DD: Diffuse AA, trichotillomenia, telogen effluvium. (sandpaper like roughness), and onycholysis.
 Alopecia 563

Table 24.6  Hair shaft disorders causing alopecia

Disease Features Clinical presentation Comments

Monilethrix • Autosomal dominant • Scalp involvement may be • Persists lifelong, may
(beaded hairs) condition localized (occiput) or regress during puberty.
(Figure 24.19a,b) • Periodic thinning of the hair widespread. Occasionally, • Beading may not be
shaft resulting in beaded eyelashes, eyebrows, pubic, evident to naked eye and
appearance of the hair axillary, and limb hair may may require dermoscopy
• Presents in infancy be involved. or light microscopy for
• Hairs are dry, lusterless, and better visualization.
brittle.
• Keratosis pilaris (on scalp,
nape of the neck, and
extensor surfaces
extremities) and koilonychias
are often associated.
Pili torti • Hair shaft flattened at • Sparse and brittle hairs are • Twisting of the hair shaft is
irregular intervals and twisted strikingly sparkling in best appreciated on light
180° along its axis reflected light. microscopy.
• Onset in second or third year • Occiput and temporal areas • Pili torti may be an isolated
of life are most severely affected. condition or a part of
• Late onset after puberty is different syndromes
known. (Menkes syndrome,
Rapp-Hodgkin syndrome,
trichothiodystrophy etc.).
• Retinoid therapy too can
cause acquired pili torti.
Trichorrhexis • Occurs due to abnormal • Hairs are sparse, short, • Classically seen in
invaginata keratinisation leading to lusterless, and brittle. Netherton syndrome
(Bamboo hairs) structurally weak hair shafts at • Changes are most (ichthyosiform
certain points along its pronounced on friction- erythroderma, ichthyosis
length. Harder (normal) prone areas – occiput and linearis circumflexa,
adjacent segments of the hair temporal areas. atopic diathesis, and
shaft invaginate into bamboo hairs).
structurally weak segments,
creating a ball and socket
appearance.
• Onset after one year
Trichorrhexis • May be congenital or • Hairs are short, lusterless • Congenital form may be
nodosa (TN) acquired after various and brittle. seen in argininosuccinic
traumatic hair care practices • Acquired TN is of three aciduria, Menkes disease,
(hair straightening, overuse of types: and trichothiodystrophy.
hair dryer, frequent swimming • Proximal – Common in • Acquired TN is reversible
in chlorinated water, dark-skinned individuals after avoidance of hair
excessive traction) who use chemical hair trauma.
• Characterized by small nodes relaxers.
at irregular intervals • Distal – Common in
throughout the length of the Asians and whites;
hair shaft. associated with traumatic
• Acquired form common in hair care practices.
females and in middle-aged • Localized – seen in pruritic
or older persons. scalp dermatoses.
Bubble hairs • Results from thermal damage • There is a localized area of
(from hair dryers, heating sparse, dry and brittle hairs.
tongs or hot curls) to hairs in
genetically predisposed
persons.
564 Alopecia

Figure 24.19  (a) Short broken hairs, dry lusterless hairs, and reduced hair density in monilethrix. (b) Monilethrix in a
young girl. Her father too had monilethrix. (a,b – Courtesy: Dr. Hiral Shah, Baroda Medical College, Vadodara, India.)

●● DD: Telogen effluvium, anagen effluvium (history of ●● There may be a history of consanguineous marriage of
chemotherapy), androgenetic alopecia (follows a typical parents.
pattern). ●● The affected child may have a normal or sometimes,
scant hair growth at birth. Hairs are gradually
Atrichia with papular lesions (APL) lost by the age of five to six months and are never
●● APL is a rare autosomal recessive disorder caused by regained.
mutations in the hairless (HR) gene, leading to failure ●● Clinical examination reveals hair loss and multiple
of catagen hairs to re-enter the anagen phase. The skin-colored, keratotic papules (Box 24.3). These
condition manifests as irreversible alopecia universalis papules keep increasing in number as the child grows
with papular lesions. and may involve the entire body (Figure 24.20a,b).

Figure 24.20  (a) Atrichia with papules. (b) Loss of eyebrows in the same patient.
 Alopecia 565

BOX 24.3: Diagnosis of atrichia with papular lesions

MAJOR CRITERIA (four out of five required)

1. Permanent or complete absence of scalp hair by the first few months of life
2. Few to widespread smooth, whitish, or milia-like papules on the face, scalp, arms, elbows, thighs, or knees from
infancy or childhood
3. Replacement of mature hair follicle structure by follicular cyst, filled with cornified material in scalp histology
4. Mutation in human hairless gene through genetic testing
5. Clinical or molecular exclusion of vitamin D-dependent rickets

MINOR CRITERIA

1. Family history consanguinity

2. Absence of secondary axillaries, pubic or body hair growth and/or sparse eyebrows and eyelashes
3. Normal growth and development including normal bone, teeth, nail, and sweating
4. Whitish hypopigmented streaks on scalp
5. Lack of response to any treatment modality

●● The patients have normal growth and development. Smaller areas may coalesce to form larger irregular
●● DD: Alopecia areata (universalis type), KFSD, vitamin areas. Hairs at the margin of these lesions show
D-dependent rickets. perifollicular erythema and perifollicular scale.
The hair pull test may be positive during active
Hereditary vitamin D-resistant rickets with disease. The disease may have an insidious or
alopecia explosive course and results in scarring alopecia
●● It is an autosomal recessive condition that occurs
(Figure 24.21a–g).
●● The lesions of cutaneous lichen planus may present
due to mutation of the vitamin D receptor (VDR)
before, during, or after the scalp involvement.
gene, which leads to unresponsiveness of the
●● Graham-Little-Piccardi-Lasseur syndrome is considered
mutant receptor to active form of vitamin D3 (1,
a variant/sub-type of LPP and is characterized by LPP
25-dihydroxyvitamin D3).
●● The condition is characterized by alopecia,
of scalp, non-cicatricial alopecia of the axillae and the
perineum, and follicular hyperkeratosis of the trunk
hypocalcemia, hypoparathyroidism, increased 1,
and extremities. The onset of these lesions may occur in
25-dihydroxyvitamin D3, and early onset rickets.
any order.
Alopecia can be present since birth or may develop in
●● Frontal fibrosing alopecia is another variant of LPP.
later life.
●● Alopecia is one of the earliest symptoms and is
●● DD: Discoid lupus erythematosus, Alopecia areata,
frontal fibrosing alopecia.
associated with poorer prognosis. There is complete
loss of scalp hair, eyebrows, eyelashes, and other
body hairs, and it is resistant to all treatment Keratosis follicular spinulosa decalvans (See Chapter 6:
modalities. Facial papules)
●● DD: Alopecia universalis, atrichia with papular lesions. ●● KFSD is a rare X-linked follicular syndrome usually

presenting in infancy. The disease is clinically

Lichen planopilaris characterized by widespread follicular keratotic papules;
scarring alopecia of the scalp, eyebrows, and eyelashes;
●● Lichen planopilaris (LPP) is an inf lammatory,
and photophobia.
primary cicatricial alopecia and is considered a ●● DD: Atrichea with papular lesion, lichen planopilaris.
follicular variant of lichen planus. Many patients
may show co-existing lichen planus of skin, mucosa,
or nails. Discoid lupus erythematosus
●● The disease is common in females, and the most ●● DLE can occur in any age group but is most commonly

commonly presenting age group is 40 to 60 years. seen between 20 and 40 years of age. It is two to three
●● Patients may complain of itching, burning, pain, and times more common in females than males. It affects all
tenderness. races.
●● The alopecic patches may be single or multiple, ●● The classical lesion of DLE presents as erythematous

focal or diffuse, and occur anywhere on the scalp. to violaceous scaly atrophic plaque with follicular
566 Alopecia

Figure 24.21  (a) Lichen planopilaris seen as scarring

alopecia and pigmentation of the scalp. (b) Lichen
planopilaris in a young female. Note perifollicular pig-
mentation. (c) Lichen planopilaris with lesions of annular
lichen planus on the scalp. (d) A case with extensive
scarring alopecia due to lichen planopilaris. Note
follicular keratotic papules. (e) Same case with frontal
hair loss. (Continued)
 Alopecia 567

plugging. When DLE lesions are present on the scalp

or other hair-bearing areas, scarring alopecia develops
within the lesion (Figure 24.22a,b).
●● DD: Lichen planopilaris, secondary scarring alopecia
(due to trauma and burn, etc.).

Alopecia mucinosa (follicular mucinosis)

●● The hallmark of alopecia mucinosa is accumulation of

mucin within the hair follicle and sebaceous glands.

It can be a primary disease or may occur secondary
to both benign (lupus erythematosus, angiolymphoid

Figure 24.21 (Continued)  (f,g) A male having andro-

genetic alopecia had a pigmented patch of scar- Figure 24.22  (a) Plaque of discoid lupus erythematosus
ring alopecia due to lichen planopilaris on the left with lesional alopecia. (b) Extensive scarring alopecia due
parietal region. to discoid lupus erythematosus.
568 Alopecia

hyperplasia) and malignant (cutaneous T-cell ●● The condition initially presents as painful or pruritic,
lymphoma, Hodgkin lymphoma, etc.) conditions. erythematous follicular papules, pustules, and nodules
●● It is a rare condition with no racial predilection. Both over the frontal and vertex area and progress towards
the sexes are affected, with male predominance. the occipital area. The pustules come in crops and heal
●● The primary localized type mainly affects young with erythematous scarring with alopecia.
adults under 40. The primary generalized type ●● The lesions usually progress in one direction
predominantly affects people over 40. The secondary by peripheral extension. Well-developed lesions
alopecia mucinosa, either with benign or malignant are characterized by multiple erythematous
association, generally affects people in the fifth to papules and pustules over the margin of alopecic
seventh decade of life. patch, with atrophic, shiny scarring at the center
●● The patient presents with hair loss in hair-bearing areas. (Figure 24.23a,b).
Alopecia in the early part of the disease is reversible and
is accompanied by pruritic, erythematous, follicular
papules and scaly plaque over scalp. Occasionally
mucinous material can be expressed from the active
lesion. As the disease progresses, follicular units are
destroyed, and dilated follicular orifices with keratin
plug can be appreciated.
●● The primary AM lesions usually resolve spontaneously
within two years with scarring alopecia.
●● DD: Tinea capitis, Lichen planopilaris, folliculitis decalvans.

Dissecting cellulitis (DC) (perifolliculitis capitis

abscedens et suffodiens/Hoffman disease)
●● It is an uncommon suppurative condition of the scalp

resulting from the occlusion of the pilosebaceous unit.

It may be a part of follicular occlusion triad or tetrad
●● Follicular occlusion triad – acne conglobata,
hidradenitis suppurativa, dissecting cellulitis
●● Follicular occlusion tetrad – follicular occlusion
triad and pilonidal cysts
●● DC is more common in colored skin and usually presents

in males in their second to fourth decade of life.

●● The lesions are typically seen on vertex and occiput.

The lesions start as painful follicular pustules

and nodules that rapidly progress to abscess and
intercommunicating sinus formation. Nodules may
coalesce to form tubular or linear ridges with loss
of hair. Seropurulent discharge with a foul smell is
frequently present. The disease runs a chronic, relapsing
course and heals with scarring alopecia and keloids.
●● One of the prominent features is sinus formation;

pressure on one site elicits pus discharge from a distant

site on the scalp.
●● Sometimes, arthritis, keratitis, and pyoderma

gangrenosum are seen in association.

●● DD: Kerion, folliculitis decalvans, tufted folliculitis.

Folliculitis decalvans (FD)

●● Folliculitis decalvans is a neutrophilic inflammatory

disorder characterized by follicular pustules and

nodules that eventually lead to scarring alopecia.
Staphylococcus aureus has been frequently identified in Figure 24.23  (a) Erythematous nodules with pustules and
the lesions, but its role in development of FD is not clear. lesional alopecia in folliculitis decalvans. (b) Lesions in
●● It can occur at any age and in either sex but is most
folliculitis decalvans showing progression towards central
commonly found among young adults and middle-aged scalp in this girl. Pustules and nodules appear at active
people, between 30 and 60 years of age. margin, leaving scarring behind.
 Alopecia 569

●● The most common site is scalp, but it can involve other

hair-bearing sites of body.
●● Tufted folliculitis is a variant of folliculitis decalvans
in which multiple hairs emerge from a single dilated
follicular opening in a “doll’s hair” pattern. These
tufts of hairs are present within the areas of scarring
alopecia with complete loss of hairs. The areas of
scarring and tufted folliculitis are usually well
circumscribed. Varying degrees of edema, erythema,
and tenderness are noted.
●● DD: Dissecting cellulitis, bacterial folliculitis.

Erosive pustular dermatosis of scalp (EPDS) (erosive

pustulosis of scalp)12
●● EPDS is an idiopathic, inflammatory disorder

of chronically sun-damaged skin. It may also

occur after trauma, surgical procedures, burn
injuries, etc.
●● The disease appears to be common in light-skinned

persons and is mostly seen in elderly people, with a

female predilection.
●● The classical site of affection is the scalp, especially bald

areas, but similar disease has been observed on legs

too. Hence, some authors prefer to use “erosive pustular
dermatosis.” Figure 24.24  Random and irregular patches of scarring
●● The lesions start as itchy or painful, non-follicular
alopecia in pseudopelade of Brocq. (Courtesy: Dr. Aman
Goyal, Haryana, India.)
pustules that progress to lakes of pus and get covered
with yellow-brown crusts. The surrounding skin is
atrophic. The disease runs a chronic, relapsing and REFERENCES
remitting course and ultimately heals with scarring
1. Shapiro J, Hordinsky M. Evaluation and diagnosis of hair loss.
alopecia. In: Callen J, Ofori AO, editors. [UpToDate] 2019. https://www.
●● The disease may be associated with co-existing actinic uptodate.com/contents/evaluation-and-diagnosis-of-hair-loss.
keratosis or squamous cell carcinoma and the latter Accessed on 25.04.2020.
should be specifically sought for. 2. Springer K, Brown M, Stulberg DL. Common hair loss disorders.
●● DD: Folliculitis decalvans, dissecting cellulitis, localized
Am Fam Physician 2003;68(1):93–102.
3. Trüeb RM. Systematic approach to hair loss in women. J Dtsch
cicatricial pemphigoid. Dermatol Ges 2010;8(4):284–97.
4. Xu L, Liu KX, Senna MM. A practical approach to the diagnosis and
Pseudopelade of Brocq management of hair loss in children and adolescents. Front Med
(Lausanne) 2017;4:112.
●● It is rare idiopathic, chronic, slowly progressive, non-
5. Kanti V, Röwert-Huber J, Vogt A, Blume-Peytavi U. Cicatricial
inflammatory patchy scarring alopecia of scalp that alopecia. J Dtsch Dermatol Ges 2018;16(4):435–461.
mimics alopecia areata. (The French term for AA is 6. Bolduc C, Sperling LC, Shapiro J. Primary cicatricial alopecia: Other lym-
Pelade.) phocytic primary cicatricial alopecias and neutrophilic and mixed pri-
mary cicatricial alopecias. J Am Acad Dermatol 2016;75(6):1101–1117.
●● It appears to be more common in light-skinned
7. Singh G, Miteva M. Prognosis and management of congenital hair shaft
populations and in females. The usual age of disorders with fragility - Part I. Pediatr Dermatol 2016;33(5):473–480.
presentation is between 30 and 50 years. 8. Singh G, Miteva M. Prognosis and management of congenital hair shaft
●● Patient presents with randomly distributed, disorders without fragility - Part II. Pediatr Dermatol 2016;33(5):481–487.
irregularly shaped, smooth-surfaced, atrophic, flesh- 9. Piraccini BM, Broccoli A, Starace M, Gaspari V, D’Antuono A, Dika E, Patrizi
A. Hair and scalp manifestations in secondary syphilis: Epidemiology,
colored or white alopecic patches over the vertex and clinical features and trichoscopy. Dermatology 2015;231(2):171–176.
occiput of the scalp. The pattern of involvement is 10. Billero V, Miteva M. Traction alopecia: The root of the problem.
called “footprint in snow.” In progressive cases hairs Clin Cosmet Investig Dermatol 2018;11:149–159.
can be plucked from the affected area with a gentle 11. Melo DF, de Mattos Barreto T, de Souza Albernaz E, Haddad NC,
pull (Figure 24.24). Tortelly VD. Ten clinical clues for the diagnosis of frontal fibrosing
alopecia. Indian J Dermatol Venereol Leprol 2019;85:559–564
●● The disease usually progresses insidiously but may burn
12. Wilk M, Zelger BG, Hauser U, Höpfl R, Zelger B. Erosive pustular
out spontaneously at any stage. dermatosis of the scalp: Reappraisal of an underrecognized entity.
●● DD: CCCA, alopecia areata. J Dtsch Dermatol Ges 2018;16(1):15–19.
 E47
Hypertrichosis and hirsutism

B SHINY SHULAMITE

ABSTRACT
Entities presenting with surplus hair over the body may be categorized into hypertrichosis or hirsutism, depending upon
distribution characteristics and sex of the affected person. Hypertrichosis may be seen in either sex and in any age group, and
the excess hair may be anywhere or everywhere over the body. Hirsutism occurs in females only, and the excess hair is noted
typically in androgen-dependent areas. Causes of hypertrichosis encompass physiological causes, endocrine causes, vari-
ous genetic conditions, different syndromes as well as malignancies. Hirsutism presents usually at puberty and is androgen
hormone-influenced, which may be idiopathic or due to benign or malignant ovarian, adrenal, or pituitary causes.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

570 DOI: 10.1201/9781351054225-76

 25
Vascular lesions

SUNIL KUMAR GUPTA

INTRODUCTION ●● DD: Port-wine stain and congenital medial fronto-facial

capillary malformation.
Cutaneous vascular lesions comprise a wide spectrum of enti-
ties, ranging from birthmarks to benign tumors to malignant Port-wine stains (PWS)
tumors. They may be localized or generalized, innocuous look- ●● This is A capillary malformation that appears at birth

ing or disfiguring, and may cause significant morbidity and and remains unchanged throughout life.
sometimes death. Considering the variations in clinical pre- ●● It is usually unilateral and segmental; it may be

sentation, it is important for physicians to suspect (Box 25.1) localized or extensive, involving the whole limb.
and diagnose various cutaneous vascular lesions. The clinical ●● They are low-flow vascular malformations that may

approach to the diagnosis of various vascular lesions is outlined occur on any part of the body but commonly affect
in Table 25.1 and the salient points are discussed below.1–9 the face in the distribution of the trigeminal nerve
(V1, V2, V3).
Salmon patch ●● Lesions are initially pale pink and later turn bluish or
●● It is a capillary malformation (Table 25.2) occurring in violaceous. The margin is well defined (Figure 25.2a,b).
about 30–40% of newborns.
●● Clinically, it presents with irregular bright-red macules/

patches with or without fine telangiectasia. The margin

is poorly defined.
●● The lesions are often triangular or rhomboid in shape.

●● The most common site is the face, particularly the

forehead, glabella (angel’s kiss), and upper eyelid

and the nape of neck (“stork bites”) (Figure 25.1).
Sometimes the sacral area is also involved and is
associated with occult spinal dysraphism.
●● Facial lesions tend to fade by the end of infancy and are

seldom detected after the age of six; however, the neck

and body lesions tend to be persistent.

BOX 25.1: Common clinical presentations

of cutaneous vascular lesions

●● Red or blue patch, papule, or plaque

●● Lesion bleeds spontaneously or on minor trauma
●● Compressible swelling
●● Reticulate erythema or blotchy skin color
●● Painful red or blue lesions
●● Telangiectasia
●● Soft tissue overgrowth, hypertrichosis
●● Pulsatile lesion, bruit
Figure 25.1  Bright red salmon patch in an infant.

DOI: 10.1201/9781351054225-77 571

572  Vascular lesions

Table 25.1  Clinical approach to vascular lesions

Morphology Diseases
Macules/patches • Fades – salmon patch
• Persists/grows – port-wine stain, infantile hemangioma (early), angioma
serpiginosum
Papules • Localized – granuloma pyogenicum (lobular capillary hemangioma)
• Linear – angioma serpiginosum
• Disseminated
• Older population, asymptomatic person – cherry angioma
• Symptomatic person (fever, lymphadenopathy, etc.) – bacillary
angiomatosis, verruga peruana
Papules, plaques, or Infantile hemangioma, congenital hemangioma, tufted angioma,
nodules hemangiopericytoma, reactive angioendotheliomatosis

• Hyperkeratotic/crusted surface – angiokeratoma, verrucous hemangioma,

lymphangioma circumscriptum
• Commonly seen in head and neck region – angiolymphoid hyperplasia with
eosinophilia, Kimura disease
• History/features of chronic venous insufficiency or arteriovenous shunt
– acroangiodermatitis
• Immunosuppressed patients – Kaposi sarcoma
• Targetoid appearance – targetoid hemosiderotic hemangioma (hobnail
hemangioma)
• Ulcerated plaque – ulcerated infantile hemangioma, angiosarcoma, Dabska
tumor
Red or blue nodules • Usually solitary, painful – glomus tumor
• Usually multiple, asymptomatic – glomangioma
Fluid-filled lesions • Solitary – venous lake
• Clear fluid – lymphangioma circumscriptum
• Blood – angiokeratoma, verrucous hemangioma
Compressible swelling Venous lake, venous malformation, arteriovenous malformation
Reticulate erythema/ • Transient, improve on warming – cutis marmorata, livedo reticularis
blotchy skin • Persistent – cutis marmorata telangiectatica congenita
• Localized – nevus anemicus
Telangiectasia See Chapter E18
Purpura See Chapter 14

Table 25.2  Vascular malformations

Simple Combined
• Capillary malformations • Capillary lymphatic
port-wine stain, malformations (CLM) –
telangiectasia angiokeratomas
• Venous malformations • Capillary venous
• Glomuvenous malformations (CVM) –
malformations cutis marmorata
• Lymphatic telangiectatica
malformations – congenital
macrocystic, • Arteriovenous
microcystic malformation (AVM)
• Capillary AVM (CAVM)
Note: Vascular malformations result from an error in vascular
development in the embryonic period and have been Figure 25.2  (a) Bright red patch of port-wine stain. Note
classified on the basis of the nature of the vessels affected. well-defined margin of the lesion. (Continued)
 Vascular lesions  573

Figure 25.2 (Continued)  (b) Port-wine stain on the trunk and

right upper extremity. (c) Port-wine stain becoming thicker
and developing nodular lesions. (d) Port-wine stain along
the cutaneous distribution of trigeminal nerve in a case of
Sturge-Weber syndrome. (e) A case of phakomatosis cesio-
flammea with capillary malformation and dermal melanocy-
tosis. (Continued)
574  Vascular lesions

Figure 25.2 (Continued)  (f) A case of phakomatosis cesioflammea with capillary malformation of face and bilateral dermal
melanocytosis of bulbar conjunctiva. (g) Klippel-Trenaunay syndrome with capillary malformation and increased soft-tissue
swelling. (a,c – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy: Dr. Deverashetti Srinivas, Nizamabad, India; e,f –
Courtesy: Dr. Balaganpathy R, Trichy, India.)

●● Long-standing lesions sometimes develop vascular blebs Granuloma pyogenicum (lobular capillary
or pyogenic granuloma-like lesions. Underlying soft tissues hemangioma, LCH)
and bones may show hypertrophic changes (Figure 25.2c). ●● Granuloma pyogenicum is a misnomer; they are
●● For associated syndromes see Table 25.3 and Box 25.2. neither infectious nor granulomatous. Lobular
●● DD: Infantile hemangioma, tufted angioma, early capillary hemangioma (LCH) is the preferred
arteriovenous malformation. diagnostic term.

Table 25.3  Syndromes associated with port-wine stain (capillary malformation)

Syndrome Capillary malformation Other features

Sturge-Weber syndrome Capillary malformation affecting Intracerebral calcification and early onset
the V1 and V2 cutaneous seizures, mental retardation, migraine,
distribution of trigeminal nerve headache, cognitive impairment, hemiplegia,
(Figure 25.2d) vision abnormalities and neonatal glaucoma
Beckwith-Wiedemann syndrome (BWS) Centrofacial capillary Exomphalos, macroglossia and gigantism,
(exomphalos-macroglossia-gigantism malformations hypoglycemia
syndrome)
Phakomatosis pigmentovascularis Capillary malformation Box 25.2
Klippel-Trenaunay syndrome Mixed capillary venous Soft-tissue and bony hypertrophy of the
(Figure 25.2g) malformation of the limb with affected limb, and varicose veins
lymphatic malformation
Parkes Weber syndrome Capillary malformation with Soft-tissue and bony hypertrophy of the
arteriovenous malformation affected limb
 Vascular lesions  575

BOX 25.2: Phakomatosis pigmentovascularis

●● The term phakomatosis refers to a developmental

malformation simultaneously affecting the eyes,
skin, and central nervous system.
●● Rare group of disorders characterized by the
combination of a capillary malformation with pig-
mented nevi and are traditionally classified into the
following types:
●● Type 1: Port-wine stain/capillary malformation

(CM) + Epidermal nevus

●● Type 2: CM + dermal melanocytosis + nevus

anemicus
●● Type 3: CM + nevus spilus + nevus anemicus

●● Type 4: CM + Nevus spilus + nevus anemicus +

dermal melanocytosis
●● Type 5: Cutis marmorata telangiectatica con-

genita + dermal melanocytosis

●● Each of the above types are further divided into:
A. Only cutaneous involvement
B. Presence of extracutaneous involvement
●● Recent classification by Happle includes
●● Phakomatosis cesioflammea: CM + dermal

melanocytosis (Figure 25.2e,f)

●● Phakomatosis spilorosea: CM + nevus spilus

●● Phakomatosis cesiomarmorata: CMTC + der-

mal melanocytosis
●● Unclassifiable forms of phakomatosis

●● It presents as a solitary, glistening, raspberry-like red or

blue-black papule or nodule, often at the site of injury
(Figure 25.3a–c).
●● It is prone to bleeding and ulceration after slight
manipulation.

Figure 25.3  (a) Lobular capillary hemangioma presenting as

erythematous papule on the scalp. (b) Lobular capillary hem-
angioma on the palm. Note the epithelial collarette at the
base. (c) Lesion of lobular capillary hemangioma can assume
a huge size. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
576  Vascular lesions

●● The common sites are the fingers, feet, lips, head and ●● DD: Unilateral nevoid telangiectasia,
upper trunk, and the mucosal surfaces of the mouth angiokeratoma circumscriptum neviforme,
and perianal area. purpuric dermatosis.
●● It is common in both sexes and in middle age, but
oral lesions are more common in females during
pregnancy.
●● DD: Infantile hemangioma, vascular malformation, and
Kaposi sarcoma.

Angioma serpiginosum
●● It is a rare cutaneous vascular nevus of

superficial capillaries, characterized by minute

puncta (ectatic capillaries) in clusters or in a
serpiginous pattern.
●● Clinically present as minute copper-red to vividly

red satellites that spread into circles and gradually

coalesce, producing the irregular serpiginous pattern
(Figure 25.4a,b).
●● Common sites are the lower limbs and buttocks; it is

initially unilateral, but it can be more widespread.

●● The lesions may not blanch completely under

pressure.
●● Dermoscopy can be helpful in diagnosis by

demonstrating “red lagoons” (Figure 25.4c).

Figure 25.4  (a) Erythematous macules and patches in angioma serpiginosum. (b) Erythematous macules and papules in
angioma serpiginosum. (c) Dermoscopy of angioma serpiginosum showing red lagoons. (b,c – Courtesy: Dr. Piyush Kumar,
Katihar, India.)
 Vascular lesions  577

Cherry angioma (Campbell de Morgan spots) ●● DD: Pyogenic granulomas, angiokeratoma, molluscum
●● These are the most common cutaneous vascular contagiosum, Kaposi sarcoma, deep fungal infections,
proliferations. verruga peruana.
●● They present as tiny cherry-red blanchable macules or ●● Oroya fever and verruga peruana represent two stages of
papules in middle or older age group (Figure 25.5a). infection with Bartonella bacilliformis and are endemic
●● The most common site is the trunk. in Peru.
●● They may be single, discrete or multiple, and ●● First stage (Oroya fever) – Characterized by
widespread. sudden onset of pyrexia, accompanied by a rapidly
●● They are composed of dilated venules and usually do progressive, hemolytic anemia, hepatosplenomegaly,
not undergo spontaneous involution. and generalized lymphadenopathy, and a petechial
●● DD: angiokeratoma, spider angioma (Figure 25.5b), or ecchymotic rash may develop.
pyogenic granuloma, amelanotic melanoma. ●● Second stage (verruga peruana) – It may develop
without previous Oroya fever or may follow it by weeks
Bacillary angiomatosis or months. The eruption is composed of erythematous
●● It is a vascular, proliferative form papules, which appear in crops and often become
of Bartonella infection, caused by B. henselae and nodular or pedunculated. They are most numerous on
B. quintana, that occurs primarily in AIDS and the face, neck, and limbs but may also involve mucous
immunocompromised patients. membranes. Characteristically they may be present in
●● It is characterized by the development of solitary different stages of evolution in the same patient.
or multiple red, purple, or flesh-colored friable
angiomatous papules, nodules or large, pedunculated, Infantile hemangioma (IH)
●● This is the most common benign vascular neoplasm
polypoid exophytic masses.
●● Sometimes constitutional symptoms such as fever, of infancy, occurring in 1–3% of newborns. It usually
chills, malaise, night sweats, anorexia, and weight loss appears during the first month of life and is characterized
are also associated with it. by rapid proliferation followed by slow involution.

Figure 25.5  (a) Bright-red papule of cherry angioma on the chest. (b) Spider angioma with a central red papule with radi-
ating vessels. (a,b – Courtesy: Dr. PC Das, Katihar, India.)
578  Vascular lesions

●● The origin is from invading angioblasts differentiating

towards placental phenotype or from embolized
placental cells.
●● Caused by unknown somatic mutation leading to
increased endothelial expression of angiogenic growth
factors such as VEGF, IGF, and bFGF.
●● More than 50% of hemangiomas correspond to sites of
embryological fusion lines and facial developmental
metameres.
●● Hemangiomas may appear as telangiectatic macules
or blanched spots that may not be recognized at
birth. Nodules develop by two to four weeks of age,
marking the 6- to 12-month proliferative phase
characteristic of hemangiomas (Figure 25.6a,b).
A stationary phase predominates until 15 months
of age. The involuting phase then begins, with the
development of pale gray regions within the nodules
and diminished firmness.
●● Full regression occurs in 50% of patients by age five,
in 70% by age seven and in 90% by age nine. Deep

Figure 25.6  (a) Infantile hemangioma on the tip of nose. (b) A well-developed lesion of infantile hemangioma on the arm.
(c) Healing of infantile hemangioma results in localized atrophy with wrinkled skin surface. (Continued)
 Vascular lesions  579

Figure 25.6 (Continued)  (d) Rapidly proliferating lesion

of infantile hemangioma may develop ulcerations.
(e) Neonatal hemangiomatosis with multiple infantile hem-
angiomas on the abdomen. (f) Same child (Figure 25.6e)
with facial lesions abdomen. (a–d – Courtesy: Dr. Piyush
Kumar, Katihar, India; e,f – Courtesy: Dr. Soumyajit
Roychoudhury, Berhampore, India.)

or mixed-type hemangiomas often show incomplete ●● Neonatal hemangiomatosis is characterized by

involution, with residual atrophic, wrinkled, multiple (typically five or more) hemangiomas, affecting
telangiectatic, redundant skin (Figure 25.6c). skin or mucosa (Figure 25.6e,f). Internal organs (liver,
●● Complications include ulcerations (Figure 25.6d), gastrointestinal tract, and brain) involvement may be
bleeding, heart failure, disfigurement, functional seen (Box 25.3).
impairment (e.g., visual impairment), and airway ●● Associated syndromes include PHACE/PHACES
obstruction. syndrome (posterior fossa brain malformations
580  Vascular lesions

Congenital hemangioma
BOX 25.3: Neonatal hemangiomatosis ●● Congenital hemangioma is different from IH, in being

fully grown at birth, without having an accelerated/

●● Baby is born with multiple small hemangiomas that disproportionate postnatal growth and no gender
may number up to 100. preponderance.
●● Typically resembles infantile hemangioma, but size ●● It is usually solitary, present on the head or limbs near
varies from 2 mm to 2 cm. a joint.
●● Increases in size for the first few months, then starts ●● It typically presents as a reddish, bluish, or purple
involuting, and resolves by two to three years of age. plaque with telangiectasia and peripheral pallor, doughy
●● More common in girls than boys. in consistency and warm to touch.
●● Multifocal/diffuse neonatal hemangiomatosis is a ●● It has two forms – rapidly involuting congenital
rare variant characterized by hemangiomas (RICH) and non-involuting congenital
●● Onset in the neonatal period
hemangiomas (NICH). RICH regresses rapidly, often by
●● Lack of malignant transformation
the first year of life with anetodermic skin atrophy. NICH
●● Involvement of three or more organs
has intrauterine growth, showing a plateau throughout
●● The most common complications are congestive life with no regression postnatally (Figure 25.7).
cardiac failure, hemorrhage, and multi-organ failure. ●● Size may vary from a few to several centimeters.
●● The presence of heart failure and the involvement ●● DD: Infantile hemangioma.
of five or more organs are associated with a higher
mortality rate. Tufted angioma
●● The mortality of patients who received no treat- ●● This is a rare tumor that occurs most commonly in

ment is quite high. prepubertal children.

●● It is characterized by tender, red or purple indurated

nodule or plaques with a size up to 20 cm. Lesions are

large segmental hemangiomas, arterial anomalies,
cardiac anomalies and coarctation of the aorta, eye
abnormalities and endocrine abnormalities, sternal
cleft, supraumbilical raphe, or both), PELVIS syndrome
(perineal hemangioma, external genitalia malformations,
lipomyelomeningocele, vesicorenal abnormalities,
imperforate anus, skin tag), SACRAL syndrome (spinal
dysraphism, anogenital anomalies, cutaneous anomalies,
renal and urologic anomalies, angioma of lumbosacral
localization), and LUMBAR syndrome (lower body
hemangioma, urogenital anomalies, ulceration,
myelopathy, bony deformities, anorectal malformations,
arterial anomalies, Renal anomalies).
●● DD: Vascular malformations (Table 25.4), congenital
hemangioma, pyogenic granuloma, tufted hemangioma.

Table 25.4  Vascular tumors and malformations

Vascular
Features Infantile hemangioma malformations
Sex predilection Females more affected No gender
predilection
Onset Congenital or acquired Congenital
Growth Grows in size Increase in size
in proportion
to growth
Resolution Spontaneous resolution Do not
may be seen involute
Consumption Uncommon, seen Common
coagulopathy in Kaposi
hemangioendothelioma
and tufted angioma
Figure 25.7  Non-involuting congenital hemangioma.
Response to Yes No
(Courtesy: Dr. Hiral Shah, Baroda Medical College,
steroids Vadodara, India.)
 Vascular lesions  581

most commonly seen on the upper thorax, neck, and Hemangiopericytoma

shoulders (Figure 25.8a). ●● It rarely presents with cutaneous lesions.
●● They may have localized hypertrichosis or hyperhidrosis ●● There are two types: infantile (congenital) and adult
on their surface. type.
●● Sometimes, lesions may assume annular configuration ●● It typically presents as a lobulated mass deep in the
(Figure 25.8b) and may be multi-focal in origin. subcutis, having red coloration, present anywhere on
●● DD: Infantile hemangioma, Kaposiform the body.
hemangioendothelioma, venous malformation, ●● It may resolve spontaneously, or excision may be needed.
glomuvenous malformation, subcutaneous fat necrosis
of newborn. Reactive angioendotheliomatosis
●● This is a rare and benign condition characterized by

reactive intravascular proliferation of cells expressing

endothelial cell markers.
●● It presents with multiple erythematous and/or

hemorrhagic macules, papules, and plaques on the

trunk and limbs.
●● Constitutional symptoms, such as fever with chills,

malaise, weight loss, night sweats, arthralgia, and

depression, are also present.
●● It is associated with systemic diseases, including

bacterial endocarditis, peripheral vascular

atherosclerotic disease, cryoglobulinemia, liver and
renal disease, antiphospholipid syndrome, amyloidosis,
and sarcoidosis.
●● DD: Acute cutaneous lupus erythematosus, sarcoidosis,

Dabska tumor, bacillary angiomatosis, erythema

nodosum, vasculitis.

Kaposiform hemangioendothelioma
●● This is a rare localized aggressive vascular proliferation

of childhood that may either be retroperitoneal or

cutaneous (superficial or deep).
●● It usually manifests later than infantile hemangioma.

●● It presents as a solitary vascular lesion that proliferates

rapidly.
●● Kasabach-Merritt phenomenon (KMP) is most

commonly seen in this tumor (Box 25.4). Tufted angioma

and hemangiopericytoma may also develop KMP.

BOX 25.4: Kasabach-Merritt phenomenon

(KMP)

●● KMP is a potentially life-threatening consumption

coagulopathy seen in certain rapidly proliferating
vascular tumors.
●● It is characterized by thrombocytopenia, microan-
giopathic hemolytic anemia, hypofibrinogenemia,
and elevated fibrin split products.
●● It is seen in Kaposiform hemangioendotheliomas,
tufted angiomas, and sometimes other vascular
tumors.
●● With the onset of KMP, the lesion increases in size
Figure 25.8  (a) Erythematous nodule of tufted angioma. and becomes violaceous, ecchymotic, and indurated.
(b) Annular tufted angioma on the trunk. The lesion ●● Thrombocytopenia manifests as petechiae, painful
expands at the periphery while the center heals with bruising, and internal as well as external bleeding.
scarring. (b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
582  Vascular lesions

Angiokeratoma ●● Lesions do not remit with age.

●● This involves cutaneous hyperkeratotic vascular lesions ●● With time, lesions become more warty but bleed
that may be localized or diffuse. after trauma.
●● Localized forms are ●● It may be associated with soft tissue overgrowth –
●● Angiokeratoma circumscriptum Klippel-Trenaunay Syndrome (KTS).
●● Angiokeratoma of Mibelli ●● DD: Verrucous hemangioma (deeper vascular
●● Solitary papular angiokeratoma elements with capillary endothelial proliferation),
●● Angiokeratoma of Fordyce microcystic lymphatic malformation (not
●● The diffuse form is angiokeratoma corporis diffusum. hyperkeratotic), acral pseudolymphomatous
●● Angiokeratoma circumscriptum angiokeratoma of children (more rare entity
●● This is a rare condition, present from birth. affecting the feet).
●● It appears as multiple aggregated bluish-black vascular ●● Angiokeratoma of Mibelli
papules forming a hyperkeratotic plaque, mostly on ●● This appears late in childhood, between 10 and
lower limbs, in a segmental pattern (Figure 25.9a,b). 15 years of age and is more common in girls than boys.

Figure 25.9  (a) Verrucous plaque on an erythematous

background in angiokeratoma circumscriptum. (b) Red-
blue plaques with hyperkeratotic surface and ulceration in
angiokeratoma circumscriptum. (c) Solitary angiokeratoma
presenting with erythematous lobulated nodule with ver-
rucous surface. (Continued)
 Vascular lesions  583

●● Lesions are characterized by bright-red macules that

slowly increase in size to become hyperkeratotic
bluish papules and plaques.
●● Usually there is a history of recurrent chilblains and
acrocyanosis.
●● Common sites are the dorsolateral aspects of fingers
and toes.
●● It is asymptomatic but may ulcerate.
●● DD: Pyogenic granuloma, hemangioma, Kaposi
sarcoma, melanocytic nevus verruca vulgaris.
●● Solitary papular angiokeratoma
●● This is an acquired disorder arising after trauma. It is
common in patients between 10 and 40 years of age.
●● A solitary lesion is indistinguishable from both
angiokeratoma circumscriptum and angiokeratoma
of Mibelli.
●● The lesion is characterized by a 2- to 10-mm dark-
red to blue-black warty papule, usually solitary but
sometimes multiple (Figure 25.9c).
●● It presents as a sudden enlargement and bleeding in
a long-standing lesion on the legs.
●● DD: Viral wart, melanocytic nevi, and malignant
melanoma.
●● Angiokeratoma of the scrotum
●● Also called angiokeratoma of Fordyce, this is the
most common angiokeratoma, generally seen in
older age groups.
●● It is a degenerative disorder with local venous
hypertension.
●● It clinically present as 1- to 4-mm bright-red vascular
papules on the scrotum (Figure 25.9d,e). The lesions
become bluish-black with aging. Discrete lesions are
also present on the penis and groin area.
●● Itching, soreness, and bleeding are associated
symptoms.
●● Angiokeratoma corporis diffusum
●● It presents as clustered symmetrical erythematous
papules on the “bathing trunk” area of the body
(Figure 25.9f).
●● It is considered a cutaneous marker of lysosomal
storage disorders, most notably Fabry disease.
Other conditions associated with widespread

Figure 25.9 (Continued)  (d) Angiokeratoma of Fordyce as erythematous papules. (e) Angiokeratoma of Fordyce.
(f) Widespread erythematous papules in angiokeratoma corporis diffusum. (a – Courtesy: Dr. Anup Kumar Tiwary,
Consultant Dermatologist, Yashoda Hospital and Research Center, Ghaziabad, India; b,d,f – Courtesy: Dr. Piyush Kumar,
Katihar, India; c – Courtesy: Dr. Sushil S Savant, Mumbai, India.)
584  Vascular lesions

angiokeratomas are GM1 gangliosidosis, ●● Acral distribution is commonly present on lower limbs
aspartylglucosaminuria, fucosidosis, β-mannosidosis, in a linear pattern. Oral mucosa is rarely involved.
sialidosis, galactosialidosis, and Kanzaki disease. ●● DD: Angiokeratoma.
●● Rarely, it may be idiopathic without any associated
systemic diseases. Microcystic lymphatic malformation (lymphangioma
circumscriptum)
Verrucous hemangioma ●● Lymphatic malformation is the result of an

●● Congenital vascular lesions characterized by warty, embryologically disturbed lymphatic system.

deep-red papules and nodules (Figure 25.10a,b). ●● Lymphangioma circumscriptum is a microcystic

lymphatic malformation.
●● Characterized by clear or blood-stained vesicles in a

plaque or area of diffuse swelling (Figure 25.11a–d).

Figure 25.10  (a) Erythematous nodules with verrucous

surface in verrucous hemangioma. Note soft-tissue over-
growth of the affected limb. (b) Verrucous hemangioma
in a young child. (a – Courtesy: Dr. Hiral Shah, Baroda Figure 25.11  (a) Grouped clear-fluid–containing vesicles
Medical College, Vadodara, India; b – Courtesy: Dr PC of lymphangioma circumscriptum. (b) Lymphangioma
Das and Dr Piyush Kumar, Katihar, India.) circumscriptum with crusted surface. (Continued)
 Vascular lesions  585

Figure 25.12  Smooth-surfaced, erythematous nodules of

angiolymphoid hyperplasia with eosinophilia on the scalp.

the hairline. Rarely the hands, shoulders, breasts, penis,

scrotum, and oral mucosa are involved (Figure 25.12).
●● Extracutaneous sites include the orbit, peripheral
arteries, the colon, the mandible, the lacrimal gland, the
parotid gland, and throat.
●● DD: Granuloma pyogenicum, sarcoidosis,
lymphocytoma cutis, Kimura disease, Kaposi sarcoma,
and granuloma faciale.

Kimura disease
●● It involves deeper tissues such as lymph nodes, salivary

glands, and the subcutis.

●● It has no initial overlying skin lesions and does not

contain epithelioid endothelial cells.

●● Peripheral blood eosinophilia is much more common in
Figure 25.11 (Continued)  (c) Bleeding into lymphangioma
Kimura disease.
circumscriptum is common, and hemorrhagic vesicles are
●● It is strongly associated with nephrotic syndrome.
frequently noted. (d) Lymphangioma circumscriptum with
lesions containing clear fluid and hemorrhagic fluid. (a–c –
Acroangiodermatitis of Mali (pseudo-Kaposi sarcoma)
Courtesy: Dr. Piyush Kumar, Katihar, India; d – Courtesy: Dr.
Shekhar Neema, Armed Forces Medical College, Pune, India.) ●● It is a hyperplasia of existing vasculature, as opposed to

Kaposi sarcoma, in which the vascular proliferation is

●● Common sites are the mucosa and skin of the head and independent of the existing vessels.
neck and sometimes the shoulders and waist area. ●● It is characterized by tiny purpuric macules that

●● Common complications are oozing with crusting and coalesce to form irregular plaques. The color of the
recurrent cellulitis. lesions has varying shades of yellow (ochre) and brown
●● Extensive limb lymphatic malformation can lead to from hemosiderin and other breakdown products
elephantiasis nostras verrucosa. (Figure 25.13). The epidermis may be normal or show
●● DD: Venous malformation, deep hemangiomas, and mild eczematous changes, edema or ulceration.
infantile fibrosarcoma.

Angiolymphoid hyperplasia with eosinophilia

(epithelioid hemangioma/pseudopyogenic
granuloma/histiocytoid hemangioma)
●● This is a locally benign proliferation of blood vessels

with distinctive large endothelial cells accompanied by

a characteristic inflammatory infiltrate of eosinophils.
●● It presents in young adults with a cluster of small,

translucent, dome-shaped, smooth-surfaced nodules Figure 25.13  Brownish plaque of acroangiodermatitis of

on the head and neck, particularly around the ear or Mali in a patient with Klippel-Trenaunay syndrome.
586  Vascular lesions

●● The lesions are commonly present on the lower legs and 3. Iatrogenic
the dorsum of feet. 4. HIV related
●● It is associated with chronic venous insufficiency, ●● Cutaneous lesions in Kaposi sarcoma are
vascular malformations (e.g., Klippel-Trenaunay characterized by non-pruritic, brown, pink, red,
syndrome, Stewart-Bluefarb syndrome, Prader-Labhart- or violaceous macules, papules, nodules, or plaque
Willi syndrome), protein-C deficiency, and symmetrical (Figure 25.14a–c).
arteriovenous fistulae. ●● Common sites are the lower extremities, head and
●● It is also seen in amputees (especially in those with neck region, and mucous membrane (palate, gingiva,
poorly fitting suction-type devices), in patients with conjunctiva).
paralyzed legs, in patients undergoing hemodialysis ●● It’s size varies from several millimeters to several
(from arteriovenous shunts distally), and in association centimeters in diameter.
with hepatitis C. ●● Lesions may be discrete or confluent and typically
appear in a linear, symmetrical distribution, following
Kaposi sarcoma (granuloma multiplex hemorrhagicum/ Langer lines.
idiopathic multiple pigmented sarcoma) ●● Tumor-associated lymphedema is typically manifested
●● This is a multifocal spindle cell tumor due to endothelial by lower extremity or facial involvement, due to
cell proliferation, predominantly involving the skin and obstruction of lymphatic channels.
other organs. ●● Visceral disease may occasionally precede cutaneous
●● There are four clinical types: manifestations.
1. Classic ●● DD: Bacillary angiomatosis, granuloma pyogenicum,
2. Endemic hemangioma, capillaritis, dermatofibroma.

Figure 25.14  (a) Violaceous nodules and plaques on the leg in Kaposi sarcoma. (b) Violaceous nodules with scaly surface
in Kaposi sarcoma. (c) Multiple violaceous plaques and nodules of Kaposi sarcoma on the back in an young adult with HIV
infection. (a,b – Courtesy: Prof. Reza Yaghoobi, Dermatology, Ahvaz Jundishupor University of Medical Sciences, Iran;
c – Courtesy: Dr. Shekhar Neema, Associate Professor, Dermatology, Armed Forces Medical College, Pune, India.)
 Vascular lesions  587

Targetoid hemosiderotic hemangioma (hobnail Angiosarcoma (malignant hemangioendothelioma/

hemangioma) hemangiosarcoma)
●● This is a benign and reactive vascular neoplasm ●● This is a rare malignant neoplasm characterized by

characterized by dermal proliferation of small channels rapidly proliferating, extensively infiltrating anaplastic
lined by endothelial cells with little cytoplasm and cells derived from blood vessels and lining irregular
prominent dark nucleus (hobnail cells). blood-filled spaces.
●● Clinically it presents as a rapidly developing, ●● Cutaneous angiosarcoma occurs in three settings:

asymptomatic, solitary, red, brown or violaceous papule 1. Idiopathic angiosarcoma of the face, scalp, and
and is surrounded by a pale brown halo (targetoid neck
appearance) (Figure 25.15a,b). 2. Angiosarcoma associated with chronic lymphoe-
●● It is precipitated by trauma and seen on any body part. dema (Stewart-Treves syndrome)
3. Post-irradiation angiosarcoma
●● Clinically, it presents with an area of bruising initially

(patient assumes it to be traumatic) followed by rapid

development of dusky-blue or red nodules over it, and
fresh, discrete nodules nearby.
●● It is a multifocal malignant tumor in which

dissemination occurs early.

●● Angiosarcoma of face and scalp is more common in

males and is invariably fatal.

Dabska tumor (papillary intralymphatic

angioendothelioma)
●● It is a rare, low-grade angiosarcoma.

●● It is commonly seen in infants and children.

●● It presents as a slow-growing, asymptomatic,

violaceous, pink or bluish-black plaque or nodule with a

predilection for the limbs.

Glomus tumor
●● Glomus tumors are encapsulated proliferations of

glomus cells (modified smooth-muscle cells that line

endothelial walls of glomus bodies).
●● The lesion is usually seen on distal extremities, with

most cases involving subungual sites.

●● Clinically, it presents as painful, blue-red papules or

nodules. The pain is characteristically precipitated by

cold temperature and pressure.

Glomuvenous malformations (glomangioma)

●● Glomuvenous malformations (GVMs) are venous

malformations (VM) that are characterized by rows

of glomus cells surrounding the distorted vascular
channels of VM.
●● GVMs present as blue-to-purple, partially compressible

papules or nodules on extremities.

●● The lesions may coalesce to form single or multiple

plaques.
●● Uncommonly, multiple lesions may be distributed

randomly over the body.

Venous lake
●● This is a form of senile angioma.
Figure 25.15  (a) Hobnail hemangioma with targetoid
●● It presents as slow-growing, asymptomatic dark-blue
appearance. (b) Typical targetoid lesion of hobnail heman-
gioma. (a,b – Courtesy: Dr. Hiral Shah, Baroda Medical compressible papules, caused by dilation of venules
College, Vadodara, India.) (Figure 25.16).
588  Vascular lesions

Table 25.5  Syndromes associated with venous malformations

Syndrome Cutaneous lesions Other features

Maffucci’s • Multiple soft, bluish, Enchondroma
syndrome tender nodules on (multiple
hands and fingers ovoid, hard
since birth or in and
childhood. radiolucent
• Involved hand/body nodules) on
part is grossly the hands
deformed. and fingers.
Blue rubber • Blue/purple soft Gastrointestinal
bleb compressible “rubber tract
nevus blebs” nodules on the involvement.
syndrome upper limbs and trunk.
(Bean’s • Mucosa may be
Figure 25.16  Bluish compressible swelling of venous lake on syndrome) affected.
the lower lip. (Courtesy: Dr. Piyush Kumar, Katihar, India.)
●● The most common symptoms are pain and burning
●● It is common on the face, lips, and ears of elderly patients. sensations in involved muscles and bones due to
●● DD: Basal cell carcinoma, blue nevus, melanocytic thrombosis and hemarthrosis.
nevus, lentigo, melanoma. ●● Venous malformations tend to worsen later in
childhood and adolescence.
Venous Malformation ●● The most common site is the head and neck, leading to
●● It is a slow-flow vascular malformation involving the facial asymmetry.
veins. ●● Life-threatening complications include intravascular
●● Clinically, it appears as bluish compressible nodules on coagulopathy, deep venous thrombosis, and pulmonary
the body due to the presence of ectatic veins between embolism.
skin and bones (Figure 25.17a,b). ●● For associated syndromes see Table 25.5.

Figure 25.17  (a) Mixed capillary-venous malformation on

the hip area. Venous malformation component is respon-
sible for diffuse bluish discoloration and swelling of the
skin. (b) Venous malformation affecting the tongue.
(b – Courtesy: Dr Piyush Kumar, Katihar, India.)
 Vascular lesions  589

●● Wyburn–Mason syndrome is also known as Bonnet-

Dechaume-Blanc syndrome. It is characterized by
ipsilateral cerebral, retinal, and cutaneous arteriovenous
malformation. The patient presents with headache,
seizures, and visual disturbances.
●● Cobb syndrome is a dermatomal pattern of
arteriovenous malformation involving the skin and
the segment of spinal cord in the same dermatome. It
clinically presents as a segmental bright-red patch with
spastic paralysis.

Nevus anemicus
●● This is a rare congenital localized vascular constriction.

●● It presents at birth or in early childhood and persists

unchanged throughout life.

●● It is characterized by a pale or hypopigmented

macule or patch with an ill-defined irregular border

(Figure 25.18).
●● It appears as a round or oval asymptomatic lesion,

commonly on the trunk.

●● If the lesion is examined by diascopy, the skin appears

indistinguishable from the surrounding normal

skin. Rubbing the skin causes reactive hyperemia
in the adjacent skin but no change within the lesion
itself. Both these features differentiate it from nevus
depigmentosus.
●● DD: Nevus depigmentosus, early vitiligo, pityriasis

versicolor, ash-leaf macules, post inflammatory

hypopigmentation.
Figure 25.18  Nevus anemicus as hypopigmented patch
with ill-defined margin.
REFERENCES
Arteriovenous malformation
1. Jahnke MN. Vascular lesions. Pediatr Ann 2016 Aug
●● This is the most common high-flow vascular
1;45(8):e299–305.
malformation. 2. Elluru RG. Cutaneous vascular lesions. Facial Plast Surg Clin North
●● It is comprised of a communicating artery and vein, Am 2013;21(1):111–126.
known as nidus. 3. Nosher JL, Murillo PG, Liszewski M, Gendel V, Gribbin CE. Vascular
●● It develops during fetal life from primitive anomalies: A pictorial review of nomenclature, diagnosis and treat-
ment. World J Radiol 2014;6(9):677–692.
arteriovenous channels in the retiform plexus. 4. Goss JA, Greene AK. Congenital vascular tumors. Otolaryngol Clin
●● Clinically, it presents as a red patch or nodule from
North Am 2018;51(1):89–97.
birth, which are confused with capillary malformation 5. Levin LE, Lauren CT. Multifocal vascular lesions. Semin Cutan Med
or infantile hemangioma. Surg 2016;35(3):153–160.
●● Due to high vascularity, the affected body part becomes 6. Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa
T, Tsutsumi Y, Aida N, Masaki H, Saida Y. Syndromes associ-
hyperplastic, showing asymmetry of the body. ated with vascular tumors and malformations: A pictorial review.
●● Parkes Weber syndrome is characterized by a congenital
Radiographics 2013;33(1):175–195.
vascular stain with overgrowth of a limb. It is associated 7. Hagen SL, Hook KP. Overgrowth syndromes with vascular malfor-
with a high-flow capillary arteriovenous malformation mations. Semin Cutan Med Surg 2016;35(3):161–169.
8. Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malforma-
with multiple arteriovenous fistulae along the affected
tions: Part I. J Am Acad Dermatol 2007 Mar;56(3):353–370.
limb. The common differential diagnoses are Klippel- 9. Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malfor-
Trenaunay and Proteus syndrome, which lack true mations. Part II: Associated syndromes. J Am Acad Dermatol
arteriovenous malformation. 2007;56(4):541–564.
 E48
Vasculitis

SUNIL KUMAR GUPTA

ABSTRACT
Vasculitis can be a challenging diagnosis for physicians, and recognition of cutaneous lesions helps physicians in assessing the
caliber of vessels involved, which in turn provides some clue to the etiological diagnosis of vasculitis. This chapter discusses a
clinical approach to the diagnosis of vasculitis.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

590 DOI: 10.1201/9781351054225-78

 E49
Neonatal dermatology

MANJUNATH M SHENOY, AMINA ASFIYA MI, GHALIYAH AZIZ KUTTY

ABSTRACT
Neonates often exhibit a unique spectrum of dermatoses, and many dermatoses (e.g., birthmarks, developmental defects, cuta-
neous manifestations of various genetic diseases and transient physiological conditions) are particularly common in neonates.
Additionally, various dermatoses may express themselves differently in neonates. Hence, neonatal dermatoses require a spe-
cial attention as the dermatoses can range from benign birthmarks or physiological conditions to worrisome manifestations
of a serious condition. Also, one should keep in mind that diagnosis of neonatal dermatoses rests on clinical examination and
maternal (and obstetric) history. This chapter discusses the clinical approach to the diagnosis of various neonatal dermatoses.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-79 591

 E50
Geriatric dermatoses

RAJESH KUMAR MANDAL

ABSTRACT
Skin diseases in old age are sometimes challenging regarding diagnosis as they may not present morphologically similar to
their counterparts in young age. In this chapter the author has tried to classify the different common dermatological diseases
according to their morphology, and emphasis is places on their morphological features, close differentials, and important
differentiating points among them. This may help students and clinicians as a quick reference tool in their busy dermatology
out-patient department.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

592 DOI: 10.1201/9781351054225-80

 E51
Dermatoses of pregnancy

SWETALINA PRADHAN, GAURAV DASH

ABSTRACT
The complex endocrinological, immunological, metabolic, and vascular changes associated with pregnancy result in charac-
teristic skin, mucosal, hair, and nail changes. Some of these changes are common and of purely cosmetic concern and, hence,
do not require any treatment. Such changes are called physiological changes. There are certain pregnancy specific disorders
that are associated with maternal and/or fetal morbidity. Recognition of such pregnancy specific dermatoses can help physi-
cians in identifying high-risk cases.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-81 593

 26
Cutaneous adverse drug reactions

ABANTI SAHA, AMRITA SIL, NILAY KANTI DAS

INTRODUCTION ●● UV-A (320–400 nm) are more likely to cause

drug-induced photosensitivity reactions, although
Cutaneous adverse drug reactions (CADRs) may be defined occasionally UV-B (290–320 nm) can also be
as “unwanted reactions that are not characteristic of the responsible for such effects.
desired pharmacodynamic effects and predict hazard for ●● Phototoxic reaction clinically mimics sunburn
future administration of a particular drug.” Some of the and presents as painful erythematous, edematous,
common causative drugs include non-steroidal anti-inflam- and sometimes scaly lesions on sun-exposed areas.
matory drugs (NSAIDs), sulphonamides, fluoroquinolones, In severe cases, vesicles and bullae may develop
imidazoles, antiepileptics and allopurinol. Although the (Figure 26.1a–d).
prognosis is good in most cases, 2% of CADRs are severe ●● DD: Sunburn, photoallergic reaction (Table 26.3).
and can be lethal. Severe cutaneous adverse reactions ●● Drugs causing phototoxic reactions are tetracyclines,
(SCARs) comprise Drug Reaction with Eosinophilia and f luoroquinolones, sulfonamides, ibuprofen,
Systemic Symptoms (DRESS), Stevens-Johnson syndrome/ naproxen, furosemide, amiodarone, diltiazem,
toxic epidermal necrolysis (TEN) and Acute Generalized quinidine, etc.
Exanthematous Pustulosis (AGEP). Pathologically, almost ●● Drugs causing only photoallergic reactions are few
80% are non-immunological (predictable and dose depen- in number, namely celecoxib, diaminodiphenyl
dent) while the rest are immunological/hypersensitivity sulfone (dapsone), and oral contraceptive pills.
reactions. Clinically they can mimic any inflammatory Among chemotherapeutic agents, flutamide (an
disorder – “a great mimicker.” Thus CADRs should be con- antiandrogen) and tegafur may cause photoallergic
sidered as a differential for any skin eruption of acute onset reactions.
and causality association of a particular drug is assessed ●● Ketoprofen, hydrochlorothiazide, phenothiazines,
by the Naranjo Probability Scale and World Health itraconazole, and 5-FU cause both types of
Organization–Uppsala Monitoring Centre (WHO–UMC) photosensitizing reactions.
Probability Scale (not discussed here) among others. This
chapter discusses the clinical approach to various CADRs Fixed drug eruption
based on clinical presentation (Table 26.1).1–8 The salient ●● Common causative drugs include fluoroquinolones

clinical features of these entities are discussed below, and and nitroimidazole fixed-dose combination or alone,
detailed discussion of these CADRs is beyond the scope of non-steroidal anti-inflammatory drugs (NSAIDS),
this chapter. History of chemotherapy necessitates consid- paracetamol, naproxen, sulphonamides, trimethoprim,
eration of a separate set of CADRs unique to chemotherapy antifungals, phenolphthalein, anticonvulsants,
(Table 26.2), though common CADRs too can be observed antibiotics, hydroxyzine, etc.
in such patients.9–11 Another subset of the population that ●● Clinically, it presents as solitary or multiple, well-

requires specific consideration is persons on highly active circumscribed, erythematous, bright-red or dusky-red
antiretroviral therapy, but these are not covered in this itchy macules that evolve into an edematous plaque and
chapter. finally resolve after few days to weeks, leaving residual
grayish pigmentation. Bullous-type lesions may also
Drug-induced phototoxic reaction appear (Figure 26.2a–f).
●● Drug-induced photosensitivity may be of two types: ●● Lesions may occur anywhere on the body. Oral mucosa

phototoxic and photoallergic reactions. and genitalia are commonly affected.

594 DOI: 10.1201/9781351054225-82

 Cutaneous adverse drug reactions  595

Table 26.1  Clinical approach to cutaneous adverse drug reactions.

Morphology Feature Diseases

Erythema Localized • Sun-exposed area – phototoxic reaction
• Random distribution – fixed drug eruption (FDE)
Generalized • Flexures – symmetrical drug-related intertriginous and flexural
exanthema (SDRIFE)
• With papules – morbilliform drug rash, drug rash with eosinophilia and
systemic symptoms (DRESS)
• With scales – drug-induced erythroderma
• Without scales – early Stevens-Johnson syndrome/toxic epidermal
necrolysis
Pigmentary changes Drug-induced hyperpigmentation
Drug-induced hypopigmentation
Papules Acneiform eruptions
Eczematous lesions Photoallergic drug reaction
Plaque Non-scaly Neutrophilic eccrine hidradenitis, pseudolympoma
Scaly Psoriasiform drug reaction
Lichenoid drug reaction
Pityriasis rosea
Drug-induced lupus
Vesicobullous lesions With (variable) systemic • Target/targetoid lesions – erythema multiforme, Stevens-Johnson
features syndrome
• Sheets of epidermal necrosis – toxic epidermal necrolysis
• Palpable purpura – drug-induced vasculitis
With minimal/absent • With photosensitivity – phototoxic drug reaction, pseudoporphyria
systemic features • Without photosensitivity – bullous FDE, drug-induced pemphigus,
drug-induced pemphigoid, linear IgA dermatosis
Pustules Acute generalized exanthematous pustulosis
Nodules Erythema nodosum
Scaling Drug-induced xerosis
Palpable purpura Drug-induced vasculitis
Urticaria lesions Drug-induced urticaria
Without skin lesions Drug-induced angioedema, drug-induced lupus

Table 26.2  Cutaneous adverse reactions due to chemotherapeutic agents

Morphology/
presentation Feature Diseases/chemotherapeutic agents
Pigmentation Diffuse • Fluorouracil, busulfan, methotrexate, procarbazine
Flexural • Bleomycin, cyclophosphamide, busulfan, and doxorubicin
Sun-exposed area • Fluorouracil, daunorubicin
Trauma/pressure-prone • Bleomycin, hydroxyurea, cisplatin
areas
Patterned • Serpentine supravenous – fluorouracil, vincristine, docetaxel, CHOP regimen
• Linear (flagellate) – bleomycin
• Reticulate – paclitaxel, cytarabine, fluorouracil
• Annular/polycyclic – daunorubicin (scalp pigmentation)
Local • Site of application – topical fluorouracil, topical mechlorethamine
• Site of adhesives – docetaxel
Mucosal • Busulfan, cyclophosphamide (gingival, may be permanent), tegafur,
doxorubicin, cisplatin, fluorouracil
(Continued)
596  Cutaneous adverse drug reactions

Table 26.2  Cutaneous adverse reactions due to chemotherapeutic agents (Continued)

Morphology/
presentation Feature Diseases/chemotherapeutic agents
Erythematous Localized • Acral – hand-foot syndrome (acral erythema)
patches • Photoexposed area – phototoxic reaction
Generalized • Diffuse erythema – hydroxyurea, busulfan, and cladribine
• Erythroderma – cisplatin, methotrexate
• Stevens-Johnson syndrome/toxic epidermal necrolysis
Papules Maculopapular eruptions • Bortezomib, lenalidomide, cladribine, fludarabine, gemcitabine,
pemetrexed, and cytarabine
• Drug reaction with eosinophilia and systemic symptoms
Papulopustules • Acneiform eruptions/chemotherapy-induced papulopustular eruptions
– epidermal growth factor receptor (EGFR) inhibitors¸ tyrosine kinase inhibitors
• PRIDE complex
Plaques Non-scaly • Neutrophilic eccrine hidradenitis
Scaly • Drug-induced subacute cutaneous lupus erythematosus
Inflamed plaques • Inflammation of seborrheic keratosis
Vesicobullous Photo-exposed area • Phototoxic reaction
lesions Acral area • Hand-foot syndrome (acral erythema)
Generalized • Erythema multiforme
• Stevens-Johnson syndrome/toxic epidermal necrolysis
Eczematous Sun-exposed area • Photoallergic reactions, photo recall
lesions Site of previous radiation • Radiation recall dermatitis
Scaling/xerosis • Cetuximab
Scleroderma • Bleomycin, gemcitabine, and docetaxel
like changes

●● On repeated exposure to a particular drug, lesions Symmetrical drug-related intertriginous and flexural
reappear on the same site and new lesions appear at exanthema (SDRIFE)
different areas. ●● This is characterized by erythema over the buttocks,
●● Variants are generalized, non-pigmenting, with linear thighs, groin, and flexural regions.
distribution. ●● It is most commonly associated with the use of beta-
●● DD: Active lesions (erythema multiforme, irritant lactam antibiotics
contact dermatitis), healed lesions (lichen planus ●● Diagnostic criteria are as follows:
pigmentosus). ●● Exposure to the systemic drug at first or repeated dose

Figure 26.1  (a) Doxycycline-induced phototoxic reaction presenting as erythema and xerosis. (b) Phototoxic reaction caus-
ing erythema and dryness of extensor forearms. (Continued)
 Cutaneous adverse drug reactions  597

  
Figure 26.1 (Continued)  (c) Imatinib-induced phototoxic
reaction. (d) Griseofulvin-induced phototoxic reaction
presenting as facial edema, erythema, and xerosis. (a,b –
Courtesy: Dr. Piyush Kumar, Katihar, India; c – Courtesy:
Dr. Rajesh Kumar Mandal, North Bengal Medical College
and Hospital, India; d – Courtesy: Prof. Bhushan Madke,
Jawaharlal Nehru Medical College, Datta Meghe Institute
of Medical Sciences, India.)

Table 26.3  Phototoxic and photoallergic reaction. ●● Erythema of the gluteal or perianal area and/or V-shaped
erythema of the inguinal area involvement of at least one
Phototoxic reaction Photoallergic reaction other intertriginous localization (Figure 26.3)
More common Less common ●● Symmetry of affected areas
Offending agent: large Offending agent: relatively ●● Absence of systemic toxicity
in amount small in amount ●● The most common drug association is beta-lactam
Immediate or early Occurs after 24 to antibiotics, particularly amoxicillin. Other well-
onset, even after 72 hours of exposure; documented medications include antihypertensives,
single exposure more than one radiocontrast media, and monoclonal antibodies
exposure is required for intravenous immunoglobulin, barium sulphate,
the lesion to develop mitomycin C, oxycodone, rivastigmine.
Sun-exposed parts Sun-exposed as well as
Morbilliform (exanthematous) drug reaction
unexposed parts
●● This is the most common cutaneous adverse reaction to drugs.
Looks like exaggerated Mimics dermatitis
●● Reaction rates are highest for antibiotics, ranging from
sunburn
1%–8% of cases.
598  Cutaneous adverse drug reactions

Figure 26.2  (a) Well-circumscribed, circular, dusky erythematous patches of fixed drug eruption. (b) Fixed drug eruption
lesion developing bulla on its surface. (c) Bullous fixed drug eruption. (d) Bulla may rupture to leave erosions. (e) Older,
dried-up lesion of fixed drug eruption. (f) Fixed drug reaction ultimately heals with post-inflammatory hyperpigmentation.
(a–f – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Cutaneous adverse drug reactions  599

Drug rash with eosinophilia and systemic

symptoms (DRESS)/drug hypersensitivity
syndrome (DHS)
●● Synonyms for this condition are drug

hypersensitivity syndrome, dapsone syndrome,

febrile mucocutaneous syndrome, graft-vs-host
disease-like illness, Kawasaki-like illness, drug-
induced delayed multiorgan hypersensitivity
syndrome (DIDMOHS).
●● It is one of the severe forms of CADR (SCAR).

●● It is characterized by a clinical triad of fever, skin

rash, and internal organ involvement. Mucous

membrane is rarely involved, and necrolysis of skin
is never present.
●● The cutaneous lesions start as maculopapular lesions

on the face, trunk, and extremities and rapidly progress

to diffuse and confluent erythema (Figure 26.5). The
diagnosis of DRESS is considered when more than 50%
body surface area is affected and there is facial edema,
diffuse scaling, or purpura. In some cases, the disease
may progress to erythroderma and vesicles and bulla, or
pustules may be seen.
●● Mucosal inflammation and pain are seen, but erosions

Figure 26.3  Symmetrical drug-related intertriginous and are not observed.

●● Characteristically it occurs with the first exposure to
flexural exanthema presenting as symmetrical erythema-
tous, scaly patches on the buttocks. (Courtesy: Dr. Chetan the drug but not with the first dose; an interval of 1 to
D Rajput, SBH Govt. Medical College, Dhule, Department 8 weeks with optimal dosage of the drug is required for
of Skin and VD, Krishna Institute of Medical Sciences, the hypersensitivity to develop.
Karad, India.) ●● Eosinophilia (90%) or mononucleosis-like atypical

lymphocytosis (40%) is the hematological hallmark of

●● There is a profuse eruption of bright-red, symmetrically this syndrome. Hence the acronym DRESS (drug rash
distributed, macules, papules, or both types of lesion with eosinophilia and systemic symptoms).
●● It is A rare entity associated with considerable morbidity
(Figure 26.4).
●● It heals within 10 to 14 days after withdrawal of the and mortality. Because of systemic involvement, it may
causative drug but can progress to exfoliative dermatitis mimic several other disorders.
●● Common drugs involved are dapsone and other
if the drug is not withdrawn.
●● Common causative drugs are penicillins, sulphonamides, carbamazepine, barbiturates,
sulphonamides, antiepileptics, and non-nucleoside
reverse transcriptase inhibitors.
●● DD: Viral exanthem.

Figure 26.5  Diaminodiphenyl sulfone (dapsone)–induced

drug hypersensitivity syndrome in a leprosy patient. Note
Figure 26.4  Amoxicillin-induced maculopapular eruption. erythematous plaque of leprosy on the face. (Courtesy:
(Courtesy: Dr. Piyush Kumar, Katihar, India.) Dr. Piyush Kumar, Katihar, India.)
600  Cutaneous adverse drug reactions

Figure 26.6  Drug-induced erythroderma. Patient was

on highly active antiretroviral therapy for the previous
six months and was started on antitubercular drugs one
month back. (Courtesy: Dr. Piyush Kumar, Katihar, India.)

phenytoin, minocycline, azathioprine, allopurinol, and

the antiretroviral drug abacavir.
●● Offending chemotherapeutic drugs are chlorambucil
and lenalidomide.

Drug-induced erythroderma/exfoliative dermatitis

●● This involves widespread inflammation of the skin

with abrupt onset of erythema, edema, and scaling

involving more than 90% of the body surface area.
Lymphadenopathy hepatosplenomegaly, leukocytosis,
eosinophilia, and anemia may be present (Figure 26.6).
●● Common offending drugs include allopurinol,

anticonvulsants, aspirin, barbiturates, captopril,

carbamazepine, cefoxitin, chloroquine, chlorpromazine,
cimetidine, diltiazem, griseofulvin, lithium, nitrofurantoin,
omeprazole, phenytoin, sulfonamides, and thalidomide.
●● Offending chemotherapeutic agents are cisplatin and

methotrexate.

Drug-induced pigmentation
●● Drug-induced pigmentary abnormalities include (1)

hyperpigmentation/melanosis, (2) hypopigmentation/

leukoderma, and (3) dyspigmentation or occurrence of
unusual skin color.
Figure 26.7  (a) Clofazimine-induced hyperpigmentation
●● The most commonly implicated medications are
on the face. Note ear involvement. (b) Clofazimine-induced
antimalarials, chemotherapeutic agents, heavy metals, pigmentation of the palms. (c) Clofazimine-induced pig-
amiodarone, zidovudine, minocycline, clofazimine mentation of the dorsum of hands. (a–c – Courtesy:
(Figure 26.7a–c), psoralens, and psychotropic drugs. Dr. Piyush Kumar, Katihar, India.)
 Cutaneous adverse drug reactions  601

●● Antimalarial-induced hyperpigmentation: Acneiform eruption

●● Affects almost one in four patients receiving ●● This presents as monomorphic acne pattern
antimalarials for at least four months. lesions beyond the seborrheic areas
●● Bluish-gray or purple pigmentation most frequently (Figure 26.8a).
in the pretibial areas; can also involve the entire nail ●● It has an unusual age of onset.
bed, nose, cheeks, forehead, ears, and oral mucosa ●● There is a resistance to conventional acne
(specifically the hard palate). therapy and, of course, a history of recent drug
●● Reversible several months after stopping the inciting introduction.
agent. ●● Causative agents include corticosteroids
●● Minocycline induced pigmentation: (Figure 26.8b,c), neuro psychotherapeutic drugs
●● Type 1: blue-black discoloration localized to scars like phenytoin, lithium, antituberculosis drugs
and post-inflammatory sites. like isoniazid, halogenated compounds, high-
●● Type 2: blue-gray pigmentation of normal skin dose vitamin B-complex, epidermal growth
on the extremities, especially the anterior shins factor receptor inhibitor (Figure 26.8d), and
mimicking antimalarial pigmentation. immunomodulating molecules.
●● Type 3: generalized “muddy” brown hyperpigmentation ●● Disruption of the culprit drug is usually not
seen most prominently in sun-exposed skin required.

Figure 26.8  (a) Acneiform lesions on the trunk. Patient was

on antitubercular therapy. (b) Acneiform lesions on chest
due to systemic corticosteroid. (c) acneiform lesions on
shoulder region due to topical corticosteroid. (d) Erlotinib
induced acneiform lesions in a middle-aged male.
(a,b – Courtesy: Dr. Piyush Kumar, Katihar, India;
c – Courtesy: Dr PC Das, Katihar, India; d – Courtesy:
Dr. Deverashetti Srinivas, Nizamabad, India.)
602  Cutaneous adverse drug reactions

Psoriasiform drug reaction Drug-induced pityriasis rosea-like reaction

●● This is an eruption of papulosquamous skin lesions, ●● Annular, circular to oval shaped scaly plaques are

indistinguishable from lesions of idiopathic psoriasis distributed mainly over the extremities and trunk with
vulgaris. The suspicion of the etiology arises only after variable degrees of itching.
seeing the temporal association. ●● Drugs involved include arsenicals, bismuth, gold,

●● Drugs that can either precipitate or aggravate barbiturates, beta-blockers, clonidine, captopril,
psoriasiform lesion include lithium, beta-blockers, griseofulvin, isotretinoin, metronidazole,
antimalarials, ACE inhibitors, terbinafine, NSAIDS, pyribenzamine, methoxypromazine and
aspirin, interleukins, and interferons. omeprazole.

Lichenoid drug eruption Drug-induced lupus erythematosus

●● Clinical features are similar to and indistinguishable ●● Drug-induced systemic lupus erythematosus (SLE)

from idiopathic lichen planus. However, lesions may not symptoms are identical to those of SLE, but skin
show flexural predilection and may be more scaly. findings are uncommon.
●● Eruptions may be photodistributed without oral ●● Skin manifestations are common with drug-induced

mucosal involvement and presence of eczematous or subacute cutaneous lupus erythematosus (SCLE),
psoriasiform lesions (Figure 26.9a,b). characterized by annular or psoriasiform, non-scarring
●● It is caused by amlodipine, antimalarials, beta-blockers, lesions in a photodistributed pattern, identical with
captopril, diflunisal, diltiazem, enalapril, furosemide, spontaneously occurring variant.
glimepiride, gold, leflunomide, levamisole, L-thyroxine, ●● In drug-induced SLE, hydralazine, procainamide

orlistat, penicillamine, phenothiazine, pravastatin, and minocycline, beta-blockers, chlorpromazine,

proton pump inhibitors, rofecoxib, salsalate, sildenafil, cimetidine, clonidine, estrogens, isoniazid, lithium,
tetracycline, thiazides, imatinib, and ursodeoxycholic acid. lovastatin, methyldopa, oral contraceptives, quinidine,

Figure 26.9  (a) Lichenoid drug reaction involving extensor surface of

upper extremities. (b) Lichenoid drug reaction due to anti-tubercular
drugs. (a – Courtesy: Dr. Piyush Kumar, Katihar, India; b – Courtesy:
Dr. Niharika Ranjan Lal, ESI-PGIMSR, Kolkata, India.)
 Cutaneous adverse drug reactions  603

sulfonamides, tetracyclines, and tumor necrosis factor

(TNF)–alpha inhibitors have been reported.
●● Drug-induced SCLE involves hydrochlorothiazide
(most common), calcium channel blockers, cimetidine,
griseofulvin, leflunomide, terbinafine, and TNF-alpha
inhibitors.
●● Offending chemotherapeutic agents include
taxanes, fluorouracil and capecitabine,
doxorubicin plus cyclophosphamide, and
gemcitabine.

Erythema multiforme
●● Acrally distributed lesions present as papules or

plaques of erythema surrounding an area of central

clearing with a central hemorrhagic crust or vesicles;
the lesions may enlarge and eventually form the
typical target lesions. They might then evolve further,
resulting in more confluent patches or annular lesions
(Figure 26.10a,b). Mucosal involvement is in the form
of oral or genital erosions.
●● Bullous lesions are also seen.

●● Offending chemotherapeutic drugs include busulfan,

chlorambucil, cyclophosphamide, docetaxel,

hydroxyurea, and tamoxifen.
●● DD: Other factors causing erythema multiforme,

including viral infections, bacterial infections,

histoplasmosis, systemic lupus erythematosus,
Wegener’s granulomatosis, X-ray therapy.

Stevens-Johnson syndrome (SJS), toxic epidermal

necrolysis (TEN) and SJS-TEN overlap (Lyell’s
syndrome)
●● This is a life-threatening cutaneous adverse reactions to

drugs (SCAR).
●● SJS is clinically characterized by widespread,

f lat, targetoid lesions with blisters arising on

erythematous or purpuric macules and/or
ulceration of the conjunctiva and the oral and
genital mucosae, with systemic upset, a prodromal

Figure 26.10  (a) Extensive lesions of drug-induced erythema multiforme. (b) Erythema multiforme with central dusky
erythematous skin (indicating damaged epidermis). (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India.)
604  Cutaneous adverse drug reactions

phase of fever, sore throat, and stinging eyes for one (>30% in TEN). They are hence distinct from erythema
to three days (Figure 26.11a,b). Nikolsky’s sign may multiforme.
be positive. ●● Mucous membrane erosions also affect the trachea,
●● TEN starts as dusky-red macules and patches that bronchi, and gastrointestinal tract.
progress rapidly to full-thickness epidermal necrosis ●● Offending drugs include sulfonamides, phenobarbital,
and dermal–epidermal detachment. The lesions phenytoin, carbamazepine, valproic acid,
become confluent and widespread erosions develop oxicam, non-steroidal anti-inflammatory drugs,
(Figure 26.11c,d). Prominent erosive mucositis is chlormezanone, allopurinol, acetaminophen, imidazole,
often present. antifungal agents, corticosteroids for systemic use,
●● SJS and TEN are considered a spectrum of drug- aminopenicillins, cephalosporins, quinolones, and
induced skin diseases having the same pathogenesis tetracyclines, and COX-2 inhibitors.
and are characterized by epidermal detachment ranging ●● Offending chemotherapeutic drugs include bleomycin,
from mild (up to 10% of total body surface area in SJS), capecitabine, doxorubicin, etoposide, lenalidomide,
moderate (10–30% in SJS/TEN overlap), and severe methotrexate, and procarbazine.

Figure 26.11  (a) Stevens-Johnson syndrome with vesicobul-

lous lesions over an erythematous base. (b) Erythematous
and purpuric lesion on the palm in Stevens-Johnson syn-
drome. (c) Toxic epidermal necrolysis with widespread dusky
erythematous lesions on the body and erosive mucositis.
(d) Toxic epidermal necrolysis with confluent dusky erythem-
atous lesions with vesiculation due to epidermal detachment.
(e) Vildagliptin induced bullous pemphigoid in a 76-year-old
male. (a-e – Courtesy: Dr. Piyush Kumar, Katihar, India.)
 Cutaneous adverse drug reactions  605

●● DD: Erythema multiforme (typical target lesions with Drug-induced bullous pemphigoid
elevated atypical target lesions). ●● Patients are commonly younger than patients with

idiopathic pemphigoid.
Drug-induced pseudoporphyria ●● Itching is common.
●● This is characterized by formation of vesiculobullous
●● Involvement of the epiglottis may lead to acute airway
drugs with skin fragility without any blood or urine obstruction.
abnormalities. History of offending drug is an ●● Tense bullae occur on normal skin or on an
additional clue. erythematous base.
●● Clinical features to differentiate it from porphyria
●● Denuded areas heal spontaneously.
cutanea tarda (PCT), its commonest differential, are as ●● There are erythematous patches, urticarial plaques, and
follows: targetoid lesions on face, trunk, limbs, palms, soles, and
●● Present both in PCT and pseudoporphyria: mucous membranes (Figure 26.11e).
photosensitivity, skin fragility, and blistering of the ●● Nikolsky’s sign may be positive, unlike in idiopathic
hands and forearms and sometimes milia. pemphigoid.
●● Not seen in pseudoporphyria: hypertrichosis, ●● Causative drugs include furosemide, gliptins,
dyspigmentation, and skin sclerosis. amoxicillin, ampicillin, phenacetin, penicillin,
●● True PCT may be precipitated by barbiturates,
penicillamine, psoralen-ultraviolet-A light, and
estrogens, griseofulvin, rifampicin, and beta-blockers.
sulfonamides. ●● Cicatricial pemphigoid occurred after the use of
●● Pseudoporphyria can be induced by furosemide,
practolol, topical echothiophate, D-penicillamine,
nabumetone, nalidixic acid, naproxen, oxaprozin, clonidine, topical pilocarpine, topical demecarium,
tetracycline, and voriconazole. indomethacin, topical glaucoma, and sulfadoxine. Oral
terbinafine has been associated with the development of
Drug-induced pemphigus
bullous pemphigoid.
●● Drug-induced pemphigus needs to be differentiated

from drug-triggered pemphigus. (Table 26.4). Linear IgA bullous dermatoses (LAD)
●● The clinical presentation of drug-induced LAD differs
Table 26.4  Drug-induced and drug triggered pemphigus
from other causes of LAD in that mucosal involvement
Drug-induced may be less likely in drug-induced cases.
pemphigus Drug-triggered pemphigus ●● Usually develops within one to two weeks of exposure

Exposure to drugs only, Genetic predisposition. to offending agent.

●● Patients may complain of severe burning and pruritus.
no genetic
●● The most common presentations include urticated
predisposition.
plaques, papulovesicles resembling dermatitis
No such association. Autoimmune disorders such
herpetiformis, targetoid lesions as in EM, and
as lupus, bullous
bullae that may be hemorrhagic resembling bullous
pemphigoid, and
pemphigoid.
myasthenia gravis may
●● Common sites are the trunk and limbs.
already be present.
●● Drugs known to cause LAD include vancomycin (most
Thiol drugs (–SH or Drugs known to trigger
common) diclofenac, somatostatin, lithium, phenytoin,
sulfhydryl group), which pemphigus include
captopril, amiodarone, cefamandole, amoxicillin, and
are present in drugs amoxicillin, ampicillin,
ampicillin-sulbactam.
like penicillamine, cephalosporins, penicillin,
captopril, pyritinol, rifampicin, propranolol, Acute generalized exanthematous pustulosis
piroxicam, and phenytoin, and (AGEP)
thiopronine, are the phenobarbitone. ●● This is a rare disease; more than 90% of cases are caused
most common agent
by drugs.
implicated in drug- ●● Myriad disseminated, non-follicular, sterile pustules
induced pemphigus.
occur on a background of diffuse erythematous skin;
Thiol-drug–induced Pemphigus vulgaris commonly the patient may have a fever (Figure 26.12a–d).
pemphigus foliaceus an occurs following therapy ●● Common offending drugs include beta-lactam
average of 11 months with non-thiol drugs for a antibiotics, aminopenicillins, sulfonamides, diltiazem,
after commencing an average of four months. anticonvulsants, and antifungals.
treatment and regress Spontaneous recovery is ●● There is a localized variant.
spontaneously after lower in non-thiol–induced ●● DD: Pustular psoriasis (differentiating points are acute
discontinuation of the pemphigus (only 15%) after onset, temporal relationship with drug and rapid resolution
drug. stopping the drug. following drug withdrawal within 10 to 15 days).
606  Cutaneous adverse drug reactions

Figure 26.12  (a) Acute generalized exanthematous pustulosis with numerous tiny pustules over an erythematous background.
(b) Close-up of acute generalized exanthematous pustulosis lesions. (c) Discrete and confluent pustules in acute generalized
exanthematous pustulosis. (d) Extensive pustules and scaling on an erythematous skin in acute generalized exanthematous
pustulosis. (a,b – Courtesy: Dr. Piyush Kumar, Katihar, India; c – Courtesy: Dr. Supratim Karmakar and Dr. Shiladitya Chowdhury,
Medical College and Hospital, Kolkata, India; d – Courtesy: Dr. Guruvani Ravu, Sunshine hospital, Hyderabad, India.)

Drug-induced erythema nodosum sulfonamides, analgesics, potassium iodide, and

●● This is characterized by tender, red, subcutaneous tetracycline.
nodules that typically appear on the anterior aspect of
the legs. Drug-induced xerosis
●● Lesions do not suppurate or ulcerate. ●● This is commonly seen with statins, isoniazid,

●● Drugs involved include oral contraceptives retinoids, butyrophenones, cimetidine, allopurinol, and
(most common), halogens, penicillin, clofazimine (Figure 26.13).
 Cutaneous adverse drug reactions  607

Figure 26.14  Drug-induced vasculitis with palpable pur-

pura and necrotic lesions. (Courtesy: Dr. Piyush Kumar,
Katihar, India.)

Drug-induced urticaria/angioedema
●● This is another common type of CADR.

●● It appears within 36 hours of drug administration but

on rechallenge may develop within minutes.

●● Two-thirds of patients have associated extensive

morbilliform rash.
●● Common drugs include ACE inhibitors, NSAIDs,

ketoconazole, fluconazole, penicillin, cephalosporins,

aspirin, codeine, hydantoin, hydralazine, and
cetirizine.
●● DD: Urticaria and angioedema due to other causes,

urticarial vasculitis.
Figure 26.13  Isotretinoin-induced xerosis of the face.
(Courtesy: Dr. Piyush Kumar, Katihar, India.) Flagellate dermatoses
●● This is an uncommon figurate dermatoses characterized

by a parallel linear or curvilinear arrangement

Drug-induced vasculitis (DIV) simulating the marks of whiplashes.
●● It is a relatively common drug eruption in clinical ●● It is originally seen with bleomycin, but can be seen
practice. with peplomycin and docetaxel too.
●● Clinical features include blanching erythematous ●● Other causes are shiitake mushroom ingestion, poison
macules, quickly followed by palpable purpura. ivy, dermatogtraphism, religious punishment, torture,
Fever, myalgias, arthritis, and abdominal pain may child abuse, dermatitis artefacta, etc.
accompany (Figure 26.14).
●● It typically develops 7 to 21 days after the onset of drug Acral erythema/palmar-plantar erythrodysesthesia/
therapy. hand-foot syndrome (HFS)
●● Hemorrhagic blisters, urticaria, ulcers, nodules, Raynaud’s ●● It is a dose-limiting adverse effect and is related

phenomenon, and digital necrosis are less frequent. to both the peak blood level and the cumulative
●● Propylthiouracil induces a distinct type of vasculitis dose of chemotherapy drug. The lesions may recur
in which the face and earlobes are involved. with increasing severity with successive cycles of
Antineutrophil cytoplasmic antibodies (ANCA) chemotherapy.
and antinuclear antibodies may be produced in this ●● The lesions start as painful, well-defined erythema

particular type of DIV. on palms and soles, and are associated with tingling,
●● Common drugs causing vasculitis include adalimumab, numbness, and stiffness of digits. In severe cases,
allopurinol, aspirin/NSAIDs, aminopenicillins, blisters and ulceration may develop. Onycholysis may
cimetidine, gold, hydralazine, leflunomide, levofloxacin, accompany HFS. The lesions usually resolve over two to
minocycline, montelukast, phenytoin, proton pump four weeks (Figure 26.15).
inhibitors, quinolones, ramipril, sulfonamide, ●● In darker skin, the skin becomes dry, darker, and

tetracycline, thiazides, and thioridazine. thicker. It interferes with daily activities.

608  Cutaneous adverse drug reactions

Figure 26.15  Imatinib-induced hand-foot syndrome with

erythema and isolated pustular lesions. (Courtesy: Dr.
Piyush Kumar, Katihar, India.)

●● Causative drugs include cytarabine, doxorubicin

hydrochloride, fluorouracil, and docetaxel. Nowadays
targeted therapy drugs such as sorafenib, sunitinib, and
other multikinase inhibitors are implicated more frequently.
●● DD: Graft-vs-host disease.

Neutrophilic eccrine hidradenitis (drug-induced

eccrine hidradenitis)
●● Clinical features include dusky-red or violaceous

papules, plaques, and nodules, commonly occurring on Figure 26.16  Erythematous papules and nodules of
Neutrophilic eccrine hidradenitis in an elderly male.
the face, back, trunk, and extremities. The lesions may
(Courtesy: Dr. Hiral Shah, Baroda Medical College,
be asymptomatic or may be associated with pain and Vadodara, India.)
tenderness (Figure 26.16).
●● It generally follows the administration of chemotherapy

by 2 to 21 days, particularly during treatment of acute PRIDE complex

and chronic myeloid leukemias. It may appear in ●● This is a spectrum of cutaneous adverse reactions

chemotherapy-naïve patients too. known as the PRIDE complex (papulopustules and/

●● It is most commonly associated with the use of or paronychia, regulatory abnormalities of hair
cytarabine (Ara-C) and/or anthracyclines. This growth, itching, dryness due to EGFR inhibitors). It is
cutaneous reaction is not a contraindication to further considered to correlate positively with the effectiveness
administration of the suspected agent. of the EGFR treatment.
●● It may be seen with non-chemotherapy drugs (including ●● Lesions appear around 8 days to 15 weeks after the onset

minocycline, granulocyte colony-stimulating factors, of treatment.

cyclophosphamide, carbamazepine, antiretroviral ●● Itchy, follicular papulopustular eruptions over the

medications) too. seborrheic area (sparing palms and soles) appear

●● DD: Sweet syndrome, graft-vs-host disease, erythema earliest, but xerosis is most common adverse effect
nodosum, erythema multiforme. (Figure 26.17).
●● Other manifestations include maculopapular
Inflammation of actinic keratoses rash, mucositis, trichomegaly, telangiectasia, and
●● Inflammation of actinic keratoses occurs due to hyperpigmentation.
systemic chemotherapy. ●● Epidermal growth factor receptor (EGFR) inhibitor
●● Originally it was observed with fluorouracil. therapy comprises erlotinib and gefitinib and
●● Doxorubicin, dactinomycin, dacarbazine, vincristine, monoclonal antibody (cetuximab).
deoxycoformycin, fludarabine, and cisplatin, may also ●● Impaired growth and migration of keratinocytes,

be responsible. and inflammatory chemokine expression lead to

●● This particular reaction may even be considered to inflammatory cell recruitment and subsequent
be beneficial. Once the acute reaction has subsided, cutaneous injury, which accounts for the majority of
chemotherapy may be reinstituted. symptoms.
 Cutaneous adverse drug reactions  609

Radiation recall dermatitis

●● Radiation recall dermatitis refers to an acute inflammatory

reaction of the skin at the site of previous irradiation.

●● Patients clinically present with itchy and/or painful,

maculopapular eruptions with erythema, edema, vesicle

formation, and exfoliation. Severe cases may develop
necrosis of the skin.
●● It usually starts within days to weeks after exposure to

the precipitating drug.

●● Common culprit drugs are actinomycin-D,

5-fluorouracil, hydroxyurea, vinblastine, methotrexate,

adriamycin, etoposide, tamoxifen, bleomycin,
melphalan, paclitaxel, docetaxel, gemcitabine, and
pegylated liposomal doxorubicin.
●● Among non-chemotherapeutic drugs, antituberculous

drugs and simvastatin can cause radiation recall dermatitis.

REFERENCES
1. Valeyrie-Allanore L, Obeid G, Revuz J. Drug Reactions. In: Bolognia
JL, Schaffer JV, Cerroni L, editors. Dermatology. 4th edition. North
York: Elsevier Limited; 2018. P. 348–375.
2. Hötzenecker W, Prins C, French LE. Erythema Multiforme, Stevens–
Johnson Syndrome, and Toxic Epidermal Necrolysis. In: Bolognia
JL, Schaffer JV, Cerroni L, editors. Dermatology. 4th edition. North
York: Elsevier Limited; 2018. P. 332–347.
3. Ardern-Jones MR, Lee HY. Benign Cutaneous Adverse Reactions to
Drugs. In: Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer
D, editors. Rook’s Textbook of Dermatology. 9th edition. West
Sussex: John Wiley & Sons; 2016. P. 118.1–118.17.
Figure 26.17  Erlotinib induced itchy scaly papular
4. Walsh S, Lee HY, Creamer D. Severe Cutaneous Adverse Reactions
lesions on the face. (Courtesy: Dr. Deverashetti Srinivas, to Drugs. In: Griffiths CEM, Barker J, Bleiker T, Chalmers R,
Nizamabad, India.) Creamer D, editors. Rook’s Textbook of Dermatology. 9th edition.
West Sussex: John Wiley & Sons; 2016. P. 119.1–119.23.
●● There are abnormalities in keratinocyte differentiation 5. Heelan K, Sibbald C, Shear NH. Cutaneous reactions to drugs. In:
Kang S, Amagai M, Bruckner AL, Enk AH, Margolis DJ, McMichael
(i.e., premature expression of keratin 1 and signal
AJ, Orringer JS, editors. Fitzpatrick’s Dermatology. 9th edition.
transducer and activator of transcription 3 [STAT3]) New York, NY: McGraw-Hill Education; 2019. P. 749–764.
causing defective stratum corneum responsible for 6. Mockenhaupt M, Roujeau JC. Epidermal Necrolysis (Stevens-
xerosis and pruritus. Johnson Syndrome and Toxic Epidermal Necrolysis). In: Kang S,
●● Management of these dermatological adverse effects Amagai M, Bruckner AL, Enk AH, Margolis DJ, McMichael AJ,
Orringer JS, editors. Fitzpatrick’s Dermatology. 9th edition. New
rarely requires discontinuation of targeted therapy and
York, NY: McGraw-Hill Education; 2019. P. 733–748.
can be managed symptomatically. 7. Sengupta SR, Das NK. Cutaneous adverse drug reaction to sys-
temic drugs: Recent updates. In: Ghosh S, editor. Recent advances
Photo recall phenomenon in Dermatology: Volume 3. 1st edition. New Delhi: Jaypee Brothers
●● It is an acute inflammatory reaction in a previously Medical Publishers Ltd; 2004: P. 88–114.
sunburned area after the administration of systemic 8. Masatkar V, Nagure A, Gupta LK. Unusual and interesting adverse
cutaneous drug reactions. Indian J Dermatol 2018;63:107–116.
chemotherapeutic agents without further sun exposure. 9. Wyatt AJ, Leonard GD, Sachs DL. Cutaneous reactions to
●● It is commonly seen after the intake of methotrexate
chemotherapy and their management. Am J Clin Dermatol
and paclitaxel (taxanes). 2006;7(1):45–63.
●● Antibiotics like cefazolin, gentamicin sulphate and 10. Kaul S, Kaffenberger BH, Choi JN, Kwatra SG. Cutaneous adverse
tobramycin, piperacillin and ciprofloxacin too may reactions of anticancer agents. Dermatol Clin 2019;37(4):555–568.
11. Payne AS, Savarese DMF. Cutaneous side effects of conven-
cause a photo recall phenomenon. tional chemotherapy agents. In: Drews RE, Corona R, edi-
●● The latent period may vary from a couple of days to
tors. [UpToDate]. https://www.uptodate.com/contents/
weeks. cutaneous-side-effects-of-conventional-chemotherapy-agents/print
 E52
Dermatitis artefacta, dermatitis neglecta, and
terra firma-forme dermatosis

SAUMYA PANDA, RASHID SHAHID

ABSTRACT
Dermatitis artefacta refers to cutaneous lesions produced by a patient’s or caretaker’s act and mimics many common derma-
toses. Dermatitis neglecta results from inadequate cleansing and usually presents with localized flakes and/or crust. Terra
firma-forme dermatosis mimics dermatitis neglecta closely.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

610 DOI: 10.1201/9781351054225-83

 E53
Pruritus Sine Materia

ANURADHA PRIYADARSHINI

ABSTRACT
Pruritus sine materia refers to chronic pruritus without any associated primary skin lesions, and patients may exhibit second-
ary lesions (e.g., excoriations only on clinical examination). Pruritus in such patients is of systemic, neurological, or psychi-
atric origin and requires thorough evaluation to arrive at a diagnosis. This chapter discusses the clinical approach to pruritus
sine materia.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-84 611

 E54
Hyperhidrosis

ANKAN GUPTA, NAVYA HANDA

ABSTRACT
Hyperhidrosis is a clinical condition characterized by excessive sweating, compared to the general population. It can be local-
ized to a particular anatomical site or generalized. It can be physiological or associated with metabolic disorders, endocrine
disorders, and more sinister neurologic disorders and malignancy, apart from drugs and addiction. Some cases, called pri-
mary hyperhidrosis, have no discernible cause. A thorough investigation is required about this condition, as it constitutes an
important reason for dermatologic consultation, for psychological and even occupational problems.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

612 DOI: 10.1201/9781351054225-85

 E55
Anhidrosis

SABHA MUSHTAQ

ABSTRACT
Hypohidrosis and anhidrosis refer to diminished and absence of sweating respectively, in response to appropriate stimuli.
Depending on the presentation, it can either be localized or generalized. There are several causes, viz. exogenous (localized
damage to skin and sweat glands or systemic medications) and endogenous (dermatological and neurologic disorders). This
chapter focuses on the clinical entities of diminished sweating, their important causes, and a brief framework to approach
such a patient.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-86 613

 27
Photosensitivity in children

RESHAM VASANI

INTRODUCTION ●● The lesions are self-limiting within one year of life

after the clearance of the anti Ro antibody acquired
Photosensitivity is an abnormal or adverse reaction of the transplacentally.
skin on exposure to ordinary light that includes ultraviolet ●● Telangiectasia may be noted and is the sole cutaneous
(UV) light or visible radiation. It usually follows sunlight but manifestation reported in some patients. The atrophic
can also be caused by artificial light sources. Photosensitivity telangiectatic changes are most evident near the temples
in children (Box 27.1) may be seen in a diverse group of and scalp.
hereditary and genodermatoses, idiopathic photodermatoses, ●● These infants should be monitored for congenital heart
and metabolic dermatoses, or may be induced by exogenous block as well as hepatic and hematologic manifestations.
agents. The clinical approach to photosensitivity disorders in ●● DD: Seborrheic dermatitis, atopic dermatitis,
children has been summarized in Table 27.1.1-3 Langerhans cell histiocytosis, Bloom syndrome,
Salient features of commonly occurring photodermato- Rothmund-Thomson syndrome.
ses in children are given below.1–4

Neonatal Lupus erythematosus (NLE)

●● NLE is characterized by development of cutaneous, cardiac,

and systemic abnormalities in newborn infants born of

mothers having autoantibodies against Ro/SSA and La/SSB.
●● It may present at birth or within weeks of birth.

●● The lesions can be exacerbated by UV exposure, but

sun exposure is not required for the development of the

lesions and they can be seen at birth prior to any sun
exposure.
●● Classically, NLE occurs in the periorbital area and

the scalp as erythematous annular or polycyclic scaly

plaques (Figure 27.1). The lesions are typically non-
scarring and resemble subacute lupus erythematosus.
Periorbital erythema (“raccoon eye” or “owl eye”) is
a characteristic feature. Bullous lesions may develop,
especially on the soles.

BOX 27.1: When to suspect

photosensitivity in a child

1. Subjective symptoms: sunburn reaction/swelling/

intense itching after exposure to sunlight
2. Objective symptoms: skin eruption/skin fragility/
scarring, predominantly over the sun-exposed Figure 27.1  Case of neonatal lupus erythematosus pre-
areas – face/V of the neck/dorsa of hands and arms senting with erythematous telangiectatic atrophic plaques
on the face.

614 DOI: 10.1201/9781351054225-87

 Photosensitivity in children  615

Table 27.1  Approach to pediatric patients with photosensitivity

Step 1: What is the age of onset of the disease?

At birth < 1 year 1–5 years School age
Neonatal, LE RTS, BS AT Hartnup disease PLE, JSE, HV, SU, AP childhood LE
CEP & HEP XP, CS (type II), TTD EPP juvenile DM
phenylketonuria Kindler syndrome BS (cutaneous features)
neonatal LE CS (Type I) TTD
Phytophotodermatitis, pellagra

Step 2: What is the predominant clinical manifestation?

Pigment dilution Butterfly erythema Dermatitis Papulonodular lesions
Albinism, PKU BS, RTS, CS, SLE, pellagra, Pellagra, Hartnup disease, Actinic prurigo
Hartnup disease PLE, phytophotodermatitis
Vesicobullous lesions Wheals Telangiectasia Poikiloderma
SLE, RTS, BS, porphyrias, Solar urticaria RTS, BS, XP, AT, SLE, juvenile RTS, juvenile DM
hydroa vacciniforme DM
Hypertrichosis
porphyrias
juvenile DM

Step 3: What are the associated clinical features?

Neurological features Growth retardation Immunodeficiency

• Cerebellar ataxia with psychiatric disturbances – BS, PKU, porphyria, AT, BS, RTS, AT, PKU
Hartnup disease CS, XP
• Cerebellar ataxia with nystagmus – AT
• Mental retardation – Hartnup disease, PKU, AT, CS, XP
• Seizures, psychosis – SLE
Dark Urine Malignancies Ophthalmic involvement
Hepatoerythropoetic porphyria BS, XP, RTS XP, BS, RTS
staining of Diapers – CEP
Abbreviations: LE, lupus erythematosus; CEP, congenital erythropoietic porphyria; HEP, hepatoerythropoietic porphyria; XP, xeroderma pigmento-
sum; CS, Cockayne syndrome; TTD, trichothiodystrophy; RTS, Rothmund-Thomson syndrome; EPP, erythropoietic protoporphyria; PMLE, poly-
morphous light eruption; AP, actinic prurigo; JSE, juvenile spring eruption; HV, hydroa vacciniforme; DM, dermatomyositis; SU, solar urticaria.

Porphyria ●● Porphyrias with cutaneous involvement include the

●● Porphyrias are a group of disorders resulting from following (Table 27.2).
deficiency of various enzymes involved in heme (part of ●● Congenital erythropoietic porphyria (CEP)
haemoglobin) biosynthesis, resulting in accumulation ●● Erythropoietic protoporphyria (EPP)
and excretion of precursors of heme called porphyrins. ●● Porphyria cutanea tarda (PCT)

Table 27.2  Childhood porphyria of dermatological importance

Type of
Porphyria Age of onset Clinical features
Porphyria Type 1 (sporadic) – Third to fourth decade Cutaneous manifestations in sun-exposed areas, including
cutanea tarda of life, usually not before puberty skin fragility, erosions, crusts, vesicles and bullae, milia,
Type II – Adulthood scarring, hyperpigmentation and hypertrichosis
Type III – Childhood
Erythropoietic Early childhood (1 to 4 years) Erythema, edema, crusts, purpura, skin thickening and waxy
protoporphyria scars, primarily on dorsal hands and face
Congenital Infancy/first decade of life Vesicles, bullae, erosions, ulcerations, crusts, milia, scarring,
erythropoietic hyperpigmentation, hypertrichosis, mutilation, hemolytic
porphyria anemia, hepatosplenomegaly, porphyrin deposition in the
teeth (erythrodontia), pink, red, or violet staining of diapers
616  Photosensitivity in children

●● Hereditary coproporphyria ●● Ocular involvement results in photophobia, corneal

●● Variegate porphyria scarring, ulcerative keratoconjunctivitis, and
●● Varied clinical presentations occur because of the cataracts.
differences in water solubility of the intermediate ●● Bone involvement manifests as osteolysis with
porphyrins. Childhood onset of hereditary osteoporosis, bone fragility, and fractures.
coproporphyria and variegate porphyria are only rarely ●● Other common findings are hemolytic anemia,
reported and are outside the scope of this chapter. hypercellular bone marrow, and splenomegaly.
●● Congenital erythropoietic porphyria (Gunther disease) ●● DD: Porphyria cutanea tarda, variegate porphyria,
●● This is an autosomal recessive condition caused epidermolysis bullosa.
by deficiency of uroporphyrinogen III synthase.
It is associated with a significant decrease in life
expectancy.
●● The most disfiguring form of porphyrias is
mutilation of facial skin and cartilage.
●● It presents in infancy as discomfort on sun exposure
or generalized blistering on phototherapy. Dark-
colored urine (Figure 27.2a) and staining of diapers
with red fluorescence on Wood’s lamp is noted.
●● In childhood, patients exhibit severe
photosensitivity with blistering (tense) on the
photo-exposed areas, healing with scarring,
and mutilation. Red- or brown-colored teeth
(Figure 27.2b) that fluoresce red orange on Wood’s
lamp examination, hypertrichosis (Figure 27.2c),
and hyperpigmentation are additional findings.

Figure 27.2  (a) Dark-colored urine of a child with congenital erythropoietic porphyria. (b) Erythrodontia in congenital
erythropoietic porphyria. (c) Hypertrichosis in a case of porphyria. (Continued)
 Photosensitivity in children  617

Figure 27.2 (Continued)  (d) Atrophic scarring on the

face and erosions on the ear in a case of erythropoietic
protoporphyria. (e) Atrophic scarring and ulcer on the
dorsum of hand in erythropoietic protoporphyria.
(a,b – Courtesy: Dr. Sunil Kumar Gupta, AIIMS Gorakhpur,
India; c,e – Courtesy: Dr. Piyush Kumar, Katihar, India.)

●● Erythropoietic protoporphyria hyperpigmentation, atrophic scarring and milia

●● This is the most common porphyria in childhood. formation (Figure 27.3b). Periorbital hypertrichosis
●● It is an autosomal recessive condition caused by can occur.
partial deficiency of enzyme ferrochelatase. ●● Sclerodermoid changes can occur in photo exposed
●● EPP is characterized by an immediate painful and non-photo–exposed areas (Figure 27.3c).
reaction within minutes of sun exposure. Erythema ●● Ocular involvement may be seen and includes
and edema with severe burning pain over the ocular pain and photophobia.
exposed areas, especially on the dorsal surfaces of ●● Liver dysfunction may be noted.
the hands, feet, and face, are noted (Figure 27.2d).
The burning pain responds only to cold air and cold
water. Photo-onycholysis may be observed.
●● Vesicobullous lesions typical of other cutaneous
porphyrias are uncommon.
●● In chronic cases, thickening of skin on knuckles
with subtle scarring on the bridge of the nose is
observed (Figure 27.2e).
●● Liver dysfunction and hypochromic microcytic
anemia are major systemic features.
●● DD: Phototoxic drug reactions, contact dermatitis,
other cutaneous porphyrias.
●● Porphyria cutanea tarda (PCT)
●● PCT usually presents in adulthood; patients
with homozygous mutations (type III,
Hepatoerythropoietic porphyria) in enzyme
(uroporphyrinogen decarboxylase) may present in
childhood. In fact, it is the second most common
porphyria in childhood.
●● PCT presents with cutaneous symptoms only.
Erosions, vesicles or bullae on sun exposed
cutaneous surfaces, especially dorsa of hands and Figure 27.3  (a) Erosion on the dorsum of the finger in a case
forearms are noted (Figure 27.3a). Lesions heal with of porphyria cutanea tarda. (Continued)
618  Photosensitivity in children

Figure 27.3 (Continued)  (b) Multiple milia on the dorsa of the hand in a case of porphyria cutanea tarda. (c) Pinched nose and
pseudo rhagades in a case of porphyria cutanea tarda. (d) Keratotic lesions on the dorsum of hands in Rothmund-Thomson
syndrome. (e) Bird like facies of Rothmund-Thomson syndrome with sparse hairs and poikiloderma. (d,e – Courtesy:
Dr Hiral Shah, Baroda Medical College, Vadodara, India.)

●● DD: Epidermolysis bullosa, bullous systemic lupus ●● Skin changes develop in first year of life, usually in first
erythematosus, other cutaneous porphyrias. three to six months.
●● In infancy, children develop erythema, swelling,
Rothmund-Thomson syndrome (poikiloderma and blistering of face. Gradually, reticulated
congenitale) hyperpigmented and hypopigmented patches,
●● This is an autosomal recessive condition caused and telangiectasia within areas of punctate
by mutations in the RECQL4 gene, which encodes atrophy (poikiloderma) appear over face, extensor
an enzyme RecQ DNA helicase, involved in DNA extremities, and buttocks. The trunk and abdomen
replication and repair. are usually spared.
 Photosensitivity in children  619

●● Keratotic lesions most commonly occur on elbows, poikiloderma (Figure 27.4c), and premalignant lesions
knees, hands, and feet (Figure 27.3d). Palmoplantar such as actinic keratosis and Bowen’s disease.
keratoderma can occur. ●● The most dreaded cutaneous complications are
●● Additional findings include sparse hair, sparse accelerated photoaging and a 1000-fold increased
eyelashes, and bird-like facies (Figure 27.3e). risk of development of cutaneous malignancies.
●● Systemic features include skeletal abnormalities Squamous cell carcinoma, basal cell carcinoma, and
(short stature, disproportionately small hands and
feet, bilateral thumb aplasia, etc.), juvenile cataracts,
sexual abnormalities, and increased risk of cancers
(osteosarcomas, non-melanoma skin cancers, etc.).
●● DD: Bloom’s syndrome, Cockayne syndrome,
dyskeratosis congenita, Kindler syndrome.

Bloom syndrome (congenital telangiectatic erythema)

●● This is an autosomal recessive condition caused

by mutations in gene BLM, which encodes an

enzyme (RecQ family of helicases) involved in DNA
recombination and repair.
●● The affected child presents with failure to thrive and

stunted growth in infancy and early life. Cutaneous

features develop late in childhood.
●● Characteristic facies is a long and narrow face,

an underdeveloped malar area, retrognathia or

micrognathia, and prominent nose and/or ears.
●● Patients develop erythematous patches and

telangiectasia on sun-exposed areas of face in a

butterfly distribution. The rash spreads to other photo-
distributed areas – ears, extensor of forearms, and dorsa
of the hands. In severe cases, blisters, hemorrhage and
crusts may develop. In dark-skinned individuals, skin
findings may be subtle or absent.
●● Other mucocutaneous findings include cheilitis (lower

lip), poikiloderma, and eyebrow or eyelash hair loss.

●● Severe pre- and post-natal growth retardation, stunted

growth with long limbs and disproportionately large hands

and feet, and sparseness of subcutaneous tissue (wasted
appearance) are among the most prominent features.
●● Other important findings include type 2 diabetes, ocular

disease (scleral telangiectasias, bulbar conjunctival

telangiectasia), infertility (in males) and subfertility (in
females), deficiency of immunoglobulin A (leading to
recurrent respiratory and gastrointestinal infections),
and increased predisposition to cancers (lymphomas,
leukemia, oral/esophageal squamous cell carcinoma, etc.).
●● DD: Cockayne syndrome, childhood lupus erythematosus.

Xeroderma pigmentosum (XP)

●● This is an autosomal recessive condition caused by a

genetic defect in nucleotide excision repair, leading to

impaired repair of DNA damaged by UV radiation.
●● Presents at one to two years of age.

●● The disease starts as an acute sunburn-like reaction

with blistering or persistent erythema after minimal

UV exposure, and scaling, usually at six months
Figure 27.4  (a) Freckling on the photo-exposed areas
of life. Freckling is marked by the age of two years of the face, including the lips and the V of the neck, in a
(Figure 27.4a,b). case of Xeroderma pigmentosum. (b) Extensive freckling
●● As age advances, chronic actinic damage results in dry
in xeroderma pigmentosum. Note depigmented macules
and parchment-like skin with mottled pigmentation, causing a mottled appearance. (Continued)
620  Photosensitivity in children

Figure 27.4 (Continued)  (c) Dyspigmentation with hyper

and hypopigmented macules on the V area of the chest in
a case of xeroderma pigmentosum (XP). (d) Squamous cell
carcinoma on the scalp in a child with xeroderma pigmen-
tosum. Note extensive freckling on the sun-exposed areas.
(e) Multiple cutaneous malignancies and premalignant
lesions in an older child with xeroderma pigmentosum.
(f) Corneal scarring in a case of xeroderma pigmentosum.
(g) Keratoacanthoma in a case of xeroderma pigmentosum.
(b,d,e – Courtesy: Dr. Piyush Kumar, Katihar, India.)

melanoma may develop as early as three to five years ●● Pinguecula and pterygium
of life and are predominantly seen on sun-exposed ●● Atrophy of the skin of the eyelids, resulting in
areas (Figure 27.4d,e). ectropion, entropion, and in severe cases complete
●● Ophthalmologic involvement is very prominent and loss of eyelids
manifests as ●● Epithelioma, squamous cell carcinoma, and
●● Chronic UV-induced conjunctivitis and keratitis – melanoma of the UV-exposed portions of the eye
corneal opacification and vascularization (Figure 27.4f) (Figure 27.4g)
 Photosensitivity in children  621

●● Neurological involvement is seen in 30% of cases. ●● DD: Bloom syndrome, Rothmund-Thomson syndrome,
Spasticity, hyporeflexia, areflexia, ataxia, chorea, motor Werner Syndrome, xeroderma pigmentosum.
neuron signs, segmental demyelination and mental
retardation may be seen. Trichothiodystrophy (TTD)5
●● DD: Rothmund-Thomson syndrome, Werner syndrome, ●● This is an autosomal recessive condition caused by

LEOPARD syndrome. genetic abnormalities in the general transcription factor

IIH (TFIIH) complex, participating in the repair of
Cockayne syndrome (CS) UV-induced DNA damage.
●● This is an autosomal recessive condition caused by ●● TTD is a heterogenous group of disorders characterized

genetic cellular sensitivity to ultraviolet (UV) mediated by sulphur-deficient (cysteine and methionine) brittle
DNA damage. hairs and neuroectodermal manifestations. A detailed
●● Two types are recognized: Type I (classical, presents in discussion of different types of TTD is beyond the scope
childhood, death by second or third decade of life) and of this book.
Type II (severe, presents at birth/in infancy, death by six ●● The cutaneous manifestations include ichthyosis

to seven years of age). (including lamellar ichthyosis), dry skin, and

●● Characteristic facies includes microcephaly, deep-set photosensitivity.
eyes, prominent ears, and a shrivelled facial appearance ●● Hairs are characteristically sparse, short, dry, and

– Mickey Mouse facies (Figure 27.5a). brittle, or there may be complete loss of hairs. Light
●● Loss of subcutaneous fat gives a wasted appearance. microscopy of hairs shows a wavy, irregular outline
●● Photosensitivity is manifested as erythema and scales and a flattened shaft that twists like a folded ribbon.
in acute cases and as hyperpigmentation, telangiectasia, Polarizing microscopy shows characteristic alternating
and atrophy in chronic cases. dark and light bands “tiger tail appearance.”
●● Other prominent features include postnatal growth ●● Nail findings include onychodystrophy, splitting, and

failure and cachexic dwarfism, long limbs with large ridging or thickening.
hands and feet, absent or hypoplastic teeth and delayed ●● Systemic findings include growth retardation,

teeth eruption, neurological degeneration, pigmentary skeletal manifestations (peripheral osteopenia and
retinopathy, cataracts, sensorineural hearing loss, etc. central osteosclerosis), neurological manifestations
●● Metronidazole administration in CS patients may cause (developmental defects, microcephaly, intellectual
potentially fatal liver failure. impairment and ataxia), and decreased fertility.
●● Rare cases with combined clinical features of XP and ●● XP-TTD overlap is called PIBIDS syndrome –

CS (XP-CS complex) are known (Figure 27.5b). photosensitivity, ichthyosis, brittle hairs, impaired

  

Figure 27.5  (a) Pinched narrow face and a beaked thin

nose and large ears suggestive of Mickey Mouse facies in
a case of Cockayne syndrome. (b) Cockayne XP overlap
syndrome presenting with freckling of the photo-exposed
areas with facial features of Cockayne syndrome.
622  Photosensitivity in children

physical and mental development, decreased fertility ●● Children assume a characteristic posture – stooping,
and short stature. Unlike XP, patients with TDD do not with the shoulders drooped and the head sunk forward
show freckle-like pigmentation and the high frequency and usually tilted to one side.
of light-induced skin cancer. ●● Increased risk of lymphoid malignancies (Hodgkin and
●● DD: Congenital alopecias. non-Hodgkin lymphoma, leukemia) and solid tumors
(stomach cancer, breast cancer, medulloblastoma, basal-
Phenylketonuria (PKU) cell carcinoma, hepatoma, etc.) is noted.
●● This is an autosomal recessive disorder of amino acid ●● DD: Hartnup disease, Refsum disease, Gaucher disease.
metabolism caused by deficiency of phenylalanine
hydroxylase enzyme, causing accumulation of Hartnup disease
phenylalanine. ●● This is an autosomal recessive metabolic disorder

●● The clinical manifestations of the disease may caused by impaired gastrointestinal and renal transport
be minimal if diagnosed and treated early (low of neutral amino acids, including tryptophan, leading
phenylalanine diet). to a consequent decrease in nicotinic acid synthesis
●● There is widespread pigmentary dilution of skin, eventuating in a pellagra-like presentation.
hairs, and eyes. Patients often exhibit photosensitivity ●● Onset of the disease is usually at three to nine years

and may develop sclerodermoid plaques, if left of age.

untreated. ●● The affected children may be asymptomatic or may

●● Developmental delay and mental retardation are other develop episodic worsening of the cutaneous and
prominent features. Other findings include mousy odor, neurological symptoms. The symptoms progress
eczematous lesions, seizures, self-mutilation, and severe over several days and last one to four weeks before
behavioral disorders. spontaneous resolution. The frequency of episodes
●● DD: Tetrahydrobiopterin deficiency, tyrosinemia. decreases with age.
●● With milder sun exposures, erythema and scaling
Ataxia telangiectasia develop over the sun-exposed areas. In severe cases, dry
●● This is an autosomal recessive condition caused by scaly well-marginated patches (resembling pellagra)
mutations in ATM gene, which encodes protein kinase develop over the forehead, cheeks, periorbital regions,
ATM. Protein kinase ATM plays a key role in repair of dorsal surfaces of the hands, and other light-exposed
double-strand breaks in DNA. areas. Lesions on the face may resemble the malar
●● Ataxia, the hallmark of disease, has its onset in the first rash of childhood LE. The skin lesions resolve with
year of life and becomes appreciable as the child grows. dyspigmentation.
Oculocutaneous telangiectasia starts appearing at the ●● Other pellagra-like features include photophobia;

three to six years (sometimes delayed till adolescence). gingivitis, stomatitis, and glossitis; and diarrhea.
●● The clinical findings are heterogenous – four groups ●● Neurological symptoms may be observed and include

have been identified. Also, the rate of progression of the ataxia, spasticity, anxiety, mood changes and mental
disease varies. retardation.
●● Facies is relaxed, dull, sad, and seemingly inattentive, ●● DD: Pellagra, phototoxic reaction, childhood LE,

contrasting with cheerful, alert appearance during the ataxia-telangiectasia.

slow-spreading smile.
●● Ocular telangiectasia is a prominent finding and starts Kindler syndrome
at the angles of both eyes, spreading toward the cornea. ●● This is an autosomal recessive subtype of epidermolysis

Eyelids too may be affected. bullosa

●● Cutaneous telangiectasia may appear on the face, ●● It is characterized by poikiloderma (Figure 27.6a),

internal ears, cubital and popliteal fossae, and, less trauma-induced acral blistering, and subsequent
commonly, elsewhere. Gradually patients develop cigarette paper thinning of the skin (Figure 27.6b),
atrophic, sclerodermoid skin changes and poikiloderma mucosal fragility, photosensitivity, and an increased
(most prominent on facial skin) in adolescence. Hairs predisposition to cancer.
may turn gray, and ears may become inelastic.
●● Patients often have a prematurely aged appearance Polymorphous light eruption (PLE)
–wasted face, scattered gray hairs, oculocutaneous ●● This presents from childhood to late adulthood.

telangiectasia, and stooped posture. ●● The prevalence is inversely related to latitude – high

●● Neurological changes (ataxia, abnormal eye movements, prevalence in Scandinavia, the U.K., and northern U.S.,
choreoathetosis, etc.) appear before cutaneous and low in Australia and equatorial regions.
telangiectasia and progress relentlessly, making the ●● There is seasonal variation – maximally seen at the

child wheelchair-bound by the age of ten to eleven beginning of the summer season and becoming
years. Deep tendon reflexes may be preserved till seven less severe as the summer season progresses (photo
to eight years and are lost thereafter. hardening).
 Photosensitivity in children  623

Figure 27.6  (a) Atrophy, dyspigmentation and telangiectasia suggestive of poikiloderma in a case of Kindler syndrome.
(b) Cigarette paper thinning of the skin on the dorsum of the hand in a case of Kindler syndrome.

●● Appears within 20–30 minutes or one to two days after ●● Other presentations include eczematous lesions,
sun exposure. variably sized papules, large plaques, papulovesicles,
●● The lesions appear in a patchy manner over sun-exposed vesicles, urticarial lesions, hemorrhagic, insect-bite–like
areas of the extensors of forearms and nape of the neck and erythema multiforme-like lesions.
and upper back. The face, which is always exposed to ●● DD: Atopic dermatitis (AD), photoallergic reactions.
sunlight, is usually, but not always, spared.
●● The most common presentation in dark skin is pinhead- Juvenile spring eruption (JSE)
sized, grouped, pruritic papules (Figure 27.7a,b). ●● This is a variant of PLE seen mostly in young boys (5 to

These may coalesce to form a plaque (Figure 27.7c). 12 years).

These lesions typically heal without scarring, leaving ●● Lesions are classically seen on the top of the ears as

hypopigmentation and mild scaling (Figure 27.7d). pruritic erythematous papules that are usually confined to

Figure 27.7  (a) Multiple pinhead-sized skin colored papules tending to coalesce on the forearm in a case of polymorphous
light eruption. (b) Lichen-nitidus–like hypopigmented micropapules over the forearm and arm in a case of polymorphous
light eruption.(Continued)
624  Photosensitivity in children

Figure 27.7 (Continued)  (c) Pinhead-sized papules

coalesced to form a hypopigmented plaque-like poly-
morphous light eruption. (d) Case of polymorphous light
eruption healed with post-inflammatory hypopigmenta-
tion on the face of a schoolgirl.

the helices of ears and evolve into vesicles and crusts. The
lesions heal with minimal or no scarring (Figure 27.8a,b).
●● Rarely, systemic symptoms such as fever, general
malaise, and headache may be seen.

Hydroa vacciniforme (HV)

●● HV is a rare, idiopathic photodermatosis principally

affecting children. Rarely, adults may be affected. Figure 27.8  (a) Multiple skin-colored micropapules
The mean age of onset is eight years (range is three to located on the external pinna in a case of juvenile spring
fifteen years). The disease resolves spontaneously in eruption. (b) Multiple papulovesicular lesions on the pinna
adolescence or young adulthood. with a few lesions topped with hemorrhagic crusts and
●● A history of summer aggravation is seen. healing with scarring in a case of juvenile spring eruption.
 Photosensitivity in children  625

●● Lesions appear within hours to a day after exposure to sun. ●● There is seasonal variation – summer exacerbation, with
●● There may be an itching and burning sensation with persistence of pruritus throughout the year.
mild constitutional symptoms preceding the eruption. ●● Clinically, it presents as intensely itchy papules,
●● HV is characterized by recurrent crops of discrete 2- to plaques, and nodules with excoriation and scars on
3-mm sized erythematous macules and papules on the the sun-exposed areas. Sometimes, in long-standing
face and dorsa of hands. The lesions progress to blisters cases the covered areas too may be affected. Secondary
and umbilicated, necrotic papules and eventually heal eczematization and lichenification is common.
with pitted varioliform scarring. ●● Very shallow linear, flat, or punctate scars may occur on
●● Keratoconjunctivitis, uveitis, blistering of lips and the face. There can be associated cheilitis, conjunctivitis,
photo-onycholysis can accompany it. and pseudopterygium.
●● DD: EPP, vesicular PLE, bullous SLE, porphyria cutanea ●● DD: Atopic dermatitis, prurigo nodularis, lichen planus.
tarda (PCT).
Childhood systemic lupus erythematosus (CSLE)
Solar urticaria ●● CSLE appears to be less common in whites than in

●● The age of is variable; it can present as early as one week non-whites.

to as late as the eighth decade. ●● It presents during childhood, between 11 and 15 years

●● There is seasonal variation: it is aggravated in summer. of age. CSLE is rare under five years of age.
●● It appears within five to ten minutes after exposure to sun. ●● The cutaneous and systemic manifestations are similar

●● It is clinically characterized by the appearance of wheals to adulthood onset SLE (discussed in Chapter E35).
and flares associated with pruritus and a burning Some key differences have been highlighted here.6
sensation appearing soon after sun exposure; it resolves ●● In general, CSLE has a more severe and more
within 24 hours without any residual changes. aggressive course than adulthood SLE.
●● Headache, nausea, wheezing, syncope, and anaphylactic ●● Raynaud’s phenomenon is less common in CSLE.
shock can accompany the eruption. On the other hand, avascular necrosis is more
●● DD: Acute urticaria, acute cutaneous lupus common in affected children.
erythematosus, cholinergic urticaria, urticarial ●● Renal diseases and central nervous system
vasculitis, polymorphous light eruption. involvement is more frequent in CSLE.
●● Compared to adults, children with discoid LE are
Actinic prurigo (Hutchinson’s summer prurigo) more likely to progress to SLE.
●● The disease appears to be common in dark-skinned ●● Malar rash (Figure 27.9a) is the most common

populations and people living at higher altitudes. cutaneous manifestation of SLE in children. Rarely
●● Childhood cases have onset before puberty, but it can photo-exacerbated annular and psoriasiform lesions
appear anytime between 2 to 43 years of age. Spontaneous of subacute cutaneous lupus erythematosus and
remission may occur in adolescence, but persistence is lesions of discoid lupus erythematosus, though
common. Childhood cases have equal gender distribution, uncommon, may be seen in the pediatric age group
but adult onset cases show female preponderance. (Figure 27.9b).

Figure 27.9  (a) Erythematous well-demarcated eruption

that occurs on bridge of the nose and cheeks, classically
sparing the naso labial fold in a case of systemic lupus
erythematosus. (b) Discoid lupus erythematosus lesions
on the face in a case of systemic lupus erythematosus.
626  Photosensitivity in children

Figure 27.10  (a) Violaceous erythema with edema over the

periorbital area, suggestive of the heliotrope rash in a case
of juvenile dermatomyositis. (b) Violaceous flat-topped
papules over the knuckles and the metacarpophalangeal
joints, suggestive of Gottron papules in a case of juvenile
dermatomyositis.

●● DD: Juvenile dermatomyositis, acute rheumatic fever, after exposure to furocoumarin and sunlight.
leukemia. Mild erythema to severe blistering eventuate in a
characteristic hyperpigmentation. Linear streaks of
Juvenile dermatomyositis (JDM) hyperpigmentation on the face, chest, hands, and
●● The common age of presentation is seven years. lower legs are characteristic. Hyperpigmentation
●● The clinical features of JDM are similar to those of adult can occur over one to two weeks and can persist
DM. Some salient differences include the following:7 over 6 to 12 months. Phototoxic reactions are also
●● The cutaneous rash of JDM can occur anywhere commonly seen due to topical applications of
on the body and is more frequently associated with psoralens prescribed in vitiligo (Figure 27.11a–c).
ulcerative change.
●● Interstitial lung disease and malignancy are less
common in JDM. Conversely, calcinosis cutis is
more common in children.
●● JDM appears to have a better prognosis compared to
adult DM.
●● Cutaneous findings include photo-distributed rash

on the face with periorbital violaceous erythema

and edema (Heliotrope rash) (Figure 27.10a), with
involvement of V of the neck, Gottron papules
(Figure 27.10b), and calcinosis cutis.
●● DD: Childhood SLE.

Photosensitivity to exogenous chemicals

●● Photosensitivity caused by exogenous agents

is relatively rare in children. It can manifest as a

phototoxic or a photoallergic reaction.
●● Phototoxic reactions

●● Plant-induced photosensitivity
(phytophotodermatitis) caused by furocoumarins Figure 27.11  (a) Phototoxic reaction due to use of
is the most common cause of phototoxic reactions topical psoralen followed by sun exposure in a case of
in the pediatric age group. It usually begins a day vitiligo. (Continued)
 Photosensitivity in children  627

Figure 27.11 (Continued)  (b) Severe phototoxic reaction due to excessive sun exposure after topical psoralen applica-
tion in a case with vitiligo. (c) Bulla in phototoxic reaction following topical PUVA therapy. (b – Courtesy: Dr. Piyush
Kumar, Consultant Dermatologist, Katihar, India; c – Courtesy: Dr. PC Das, Consultant Dermatologist, Katihar, India.)

●● Systemic phototoxicity, though rare, can be caused

by systemic antibacterial agents (doxycycline,
tetracycline, sulphonamides, nalidixic acid and
fluoroquinolones), antifungal agents (griseofulvin),
sulfonylurea antidiabetics, furosemide, non-
steroidal anti-inflammatory drugs, amiodarone,
quinine, isoniazid, and the thiazide group of drugs.
●● Photoallergic reactions
●● Photoallergic reactions are rare in the pediatric
age group, and organic components of
sunscreens, such as octocrylene, benzophenone
-3, and butyl methoxydibenzoylmethane,
are known photosensitizers in the western
population.

Pellagra
●● Pellagra is caused by an acquired deficiency of niacin or

its precursor, tryptophan. It is primarily a disorder of

adults but may be seen in children too.
●● The clinical findings in pediatric pellagra are similar to

those in adult disease.

●● Cutaneous lesions are characterized by sharply

demarcated and symmetric, tender erythematous

edematous plaques over sun-exposed areas
especially the dorsa hands, forearms, face, and neck, Figure 27.12  Burned skin-like appearance on the photo-
accompanied by pruritus and burning (Figure 27.12). exposed areas in a case of pellagra.
628  Photosensitivity in children

Lesions may develop vesicles and erosions, and 4. Grossberg AL. Update on pediatric photosensitivity disorders.
Curr Opin Pediatr 2013;25(4):474–479.
eventually the affected skin thickens and becomes
5. Faghri S, Tamura D, Kraemer KH, DiGiovanna JJ.
hyperpigmented. Trichothiodystrophy: A systematic review of 112 published cases
●● DD: Pediatric SLE, phototoxic reaction. characterises a wide spectrum of clinical manifestations. J Med
Genet 2008;45:609–621.
6. Papadimitraki ED, Isenberg DA. Childhood-and adult-onset lupus:
REFERENCES An update of similarities and differences. Expert Rev Clin Immunol
2009;5(4):391–403.
1. Inamadar AC, Palit A. Photosensitivity in children: An approach to
7. Tansley SL, McHugh NJ, Wedderburn LR. Adult and juvenile
diagnosis and management. Indian J Dermatol Venereol Leprol
dermatomyositis: are the distinct clinical features explained by our
2005;71:73–79.
current understanding of serological subgroups and pathogenic
2. Chantorn R, Lim HW, Shwayder TA. Photosensitivity disorders in
mechanisms? Arthritis Res Ther 2013;15(2):211.
children: Part I. J Am Acad Dermatol 2012;67(6):1093.e1–18.
3. Chantorn R, Lim HW, Shwayder TA. Photosensitivity disorders in
children: Part II. J Am Acad Dermatol 2012;67(6):1113.e1–15.
 E56
Photosensitivity in adults

SANTOSHDEV P RATHOD, KALGI BAXI

ABSTRACT
Photosensitivity refers to precipitation or aggravation of skin disorders by ultraviolet rays and sometimes by visible light too.
Photosensitive skin disorders may be congenital or acquired later in life. This chapter focuses on clinical aspects of acquired
photosensitive disorders.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-88 629

 E57
Maculopapular rash

PREETI SHARMA

ABSTRACT
The differential diagnosis for maculopapular rash is extensive. Evaluating the patients with such a rash can be challenging
because the differential diagnosis is extensive and includes benign self-limiting (roseola) as well as life-threatening illnesses
(Kawasaki disease). The key features that help in clinical diagnosis are mode of onset (acute/chronic/relapsing), distribution
(localized or generalized, central or peripheral), evolution (some conditions begin with morbilliform rash but evolve into dif-
ferent morphology, e.g., vesicles in varicella), and presence or absence of systemic features, along with family history and drug
history.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

630 DOI: 10.1201/9781351054225-89

 E58
Fever with rash in children

BHUMESH KUMAR KATAKAM

ABSTRACT
Fever with rash in children is seen by dermatologists as well as pediatricians. Usually the most common cause is viral exan-
them, which is self-resolving, but it is important to know the signs and symptoms of other serious illnesses for timely diagnosis
and treatment.

The text of this chapter is available online at https://routledgetextbooks.com/textbooks/9781032399669/

DOI: 10.1201/9781351054225-90 631

Index

Note: Locators in italics represent figures and bold indicate tables in the text

A friction melanosis, 53 lymphogranuloma venereum (LGV),

hori nevus/acquired bilateral nevus of 338–339
Abdominal trauma, 342
Ota-like macules (ABNOM), 47 malakoplakia of the skin, 341
Abscesses, 228, 231, 339, 397, 435
lichen planus pigmentosus (LPP), 39 mammary duct fistula, 335
Acanthosis nigricans (AN), 20, 31, 44,
macular amyloidosis, 53 metastatic fistula, 341–342
66–67, 192, 199, 397
maturational pigmentation, 40–42 Milker’s sinus, 335
Acetaminophen, 604
melasma, 39 mycetoma, 336–338
Acitretin, 112
Nevus of OTA (nevus fusco caeruleus non-metastatic fistula, 342
Acne, 79, 81, 84
ophthalmo-maxillaris), 46–47 oral squamous cell carcinoma, 341
conglobata, 336
periorbital melanosis, 42–44 pancreatic panniculitis, 341
excoriée, 500, 501
pigmentary demarcation lines (PDLs), pilonidal sinus, 341
fulminans, 341
47–48 pyoderma faciale, 341
inversa, 207
poikiloderma of civatte, 50 scrofuloderma, 335–336
keloidalis, 84, 555–556
post-chikungunya pigmentation, 50 severe acne, 340
keloidalis lesions, 85
post-inflammatory Acquired smooth-muscle hamartoma, 170
keloidalis nuchae, 85, 282
hyperpigmentation (PIH), 53 Acquired tufted angioma, 218
lesions, 102
Riehl’s melanosis, 44–46 Acral erythema, 606
preadolescent, 81
seborrheic melanosis, 49–50 Acral fibromyxoma, 232
Acneiform
vitamin B12 deficiency, 51–52 Acral lentiginous melanoma (ALM),
eruption, 601
Acquired focal facial melanosis, 40 32–33, 33
follicular mucinosis, 115, 115
Acquired melanocytic nevus, 76, 85 Acral purpuric lesions, 325
lesions, 601
Acquired perforating disorders, 212 Acral skin, 58
Acne vulgaris (AV), 3, 81, 100–103, 104,
Acquired pigmentary disorders, 39, 58 Acremonium species, 336, 355
106–109, 116–117, 119, 123,
Acquired sinus and fistula Acroangiodermatitis, 213, 218
280–281
actinomycosis, 339 of Mali, 585–586
Acquired bilateral nevus of Otalike
antitrypsin deficiency panniculitis, of Mali-Kuiper, 213
macules (ABNOM), see Nevus
340–341 Acrochordons, see Skin tags
of Hori
atypical mycobacterial infections, 340 Acrocyanosis, 303, 325
Acquired brachial cutaneous
botryomycosis, 338 Acrodermatitis
dyschromatosis (ABCD), 33, 75
chronic osteomyelitis, 335 continua, 285–286
Acquired facial melanosis, 39
coccidioidomycosis, 338 enteropathica, 413, 430, 431
acanthosis nigricans (AN), 44
Crohn’s disease (cutaneous Crohn’s Acrokeratosis
Addison’s disease, 50–51
disease), 341 verruciformis, 86, 86
drug induced pigmentation, 52–53
cutaneous amoebiasis, 339 verruciformis of Hopf, 109
erythema dyschromicum perstans
cutaneous myiasis, 339 Acropachy, 451–452
(EDP), 39–40
dental sinus or fistula (odontogenic Acropigmentation of Dohi, 70
erythromelanosis follicularis faciei et
fistula), 335 Acroreticulate pigmentation of Dohi, 69
colli (EFF), 48
enterocutaneous fistula (ECF), 342 Actinic cheilitis, 401, 497
erythrose péribuccale pigmentaire
epidermoid cyst (infundibular cyst), Actinic granuloma, 526
de Brocq (peribuccal
334–335 Actinic keratoses (AK), 30, 70, 87,
pigmentation of Brocq), 48–49
hidradenitis suppurativa, 340 167–168, 207, 297
exogenous ochronosis (EO), 44
Hodgkin’s disease, 341 inflammation, 608
freckles (ephelides) and lentigines, 47

632
 Index 633

Actinic lichen planus (LP), 19, 30, dissecting cellulitis (DC), 568 Amyloidosis cutis dyschromica,
514–516, 516 erosive pustular dermatosis of scalp 72–74, 73
Actinic prurigo (Hutchinson’s summer (EPDS), 569 Anagen effluvium (AE), 560–562, 564
prurigo), 30, 625 folliculitis decalvans (FD), 568–569 due to azathioprine, 561–562
Actinic purpura, 317 frontal fibrosing alopecia (FSA), Anal fistula, 341
Actinic reticuloid, 480 551–553 Analgesics, 606
Actinomyces, 336 hereditary vitamin D-resistant rickets ANCA-associated vasculitis, 322
Actinomycetoma, 336, 337, 338, 338 with alopecia, 564 Androgenetic alopecia (AA), 548–551,
Actinomycosis, 214, 336, 339–341, 435 keratosis follicular spinulosa 553, 560, 562, 564, 567
Active lesions, 596 decalvans, 565 Anemia, 116, 394
Acute cutaneous lupus lichen planopilaris, 564–565 Aneurysmal dermatofibroma, 218
erythematosus, 581 lipedematous Alopecia, 554 Angioblastoma of Nagakawa, 96
Acute febrile neutrophilic loose anagen hair syndrome Angioedema, 397, 435, 607
dermatosis, 210 (LAHS), 562 Angiofibroma, 114
Acute generalized exanthematous lupus hair, 551 Angiogenesis inhibitor, 306
pustulosis (AGEP), 605 mucinosa (follicular mucinosis), Angioinvasive fungal infections, 324
Acute graft versus host disease, 430 152–153, 567–568 Angiokeratomas, 95–96, 418, 427, 577,
Acute irritant contact dermatitis (ICD), neonatal occipital alopecia (NOA), 581–584
245–246 554–555 circumscriptum, 582
Acute paronychia, 467, 468 nevus sebaceous, 556 circumscriptum neviforme, 95,
Acute phototoxic drug reactions, pressure alopecia, 555 176–177, 576
271–272 pseudopelade of Brocq, 569 corporis diffusum, 418, 583
Acute rheumatic fever, 626 secondary scarring alopecia, 558 of fordyce, 583
Acute self-limiting viral infection, 376 short anagen syndrome (SAS), 562 of Mibelli, 582–583
Acute urticaria, 209 syphilitic alopecia (SA), 547–548 of scrotum, 583
Addison’s disease, 50–51, 52, 199, 389 telogen effluvium (TE), 559–560 Angioleiomyoma, 207–208
Adenoma sebaceum (facial temporal triangular alopecia, Angiolipoma, 208
angiofibroma), 84, 106 553–554 Angiolupoid type psoriasis, 152
Adrenocorticotropic hormone tinea capitis, 541–546 Angiolymphoid hyperplasia with
(ACTH), 39 totalis, 541 eosinophilia (ALHE), 96, 97,
Adult-onset PRP, 180 traction alopecia (TA), 550–551 116, 128, 218, 585
Aedes mosquitoes, 50 trichotillomania, 556–558 Angioma serpiginosum, 576
Aeromonas hydrophila, 348 tumor alopecia, 558 Angioneurotic edema, 402
Afatinib, 210 universalis, 541 Angiosarcoma, 196, 299, 587
African endemic Kaposi sarcoma, 196 Alopecia areata (AA), 503, 541, 548, Angular cheilitis (AC), 401, 401, 473,
African tick bite fever (ATBF), 347 553–555, 558, 564, 569 475, 479
AIDS-related epidemic Kaposi diffuse, 544, 544 Annular atrophic plaque, 191
sarcoma, 196 lesion of, 542 Annular elastolytic giant cell granuloma
Air hostess dermatitis, see Periorificial regrowing hairs within the (AEGCG), 526–527
dermatitis (perioral dermatitis) lesions, 543 Annular lesions, 514
Alcohol, 366 Alpha-1 antitrypsin deficiency actinic lichen planus, 514–516
Allergic contact dermatitis, 194, panniculitis, 341 annular elastolytic giant cell
244–245, 412–413, 417, 417 Alphalinolenic acid (ALA) granuloma (AEGCG), 526–527
Allergic/irritant contact dermatitis, 421 deficiency, 478 annular lichenoid dermatitis of youth
Allopurinol, 594, 604 Alpha tissue growth factor (TGF), 227 (ALDY), 520
Alopecia, 541 Amalgam tattoo, 388 annular lichen planus, 530–532
acne keloidalis, 555–556 Ambiguous genitalia, 437 annular pustular psoriasis, 539
anagen effluvium (AE), 560–562 Amblyomma ticks, 346 annular sarcoidosis, 530
androgenetic alopecia, 548–550 Amebiasis, 434 annular tufted angioma, 527
aplasia cutis congenita (ACC), 559 Amebic infections, 355 basal cell carcinoma (BCC), 532–533
atrichia with papular lesions Amelanotic melanoma, 217, 217, 218, bullous impetigo, 519
(APL), 564 225, 577 circinate balanitis, 520
central centrifugal cicatricial alopecia Amicrobial pustulosis of the folds clinical clues for diagnosis, 515
(CCCA), 554, 569 (APF), 288 clinical diagnosis, 515
clinical approach, 542 Aminoaciduria, 180 cutaneous larva migrans (creeping
diffuse alopecia areata, 562–564 Aminopenicillins, 604 eruption), 537–538
discoid lupus erythematosus, 565–567 Amiodarone, 53 dermatophyte infection, 417, 520–521
634   Index

elastosis perforans serpiginosa Anthrax, 215–216, 301, 348, 349 Atrichia with papules, 565
(EPS), 523 Anticoagulants, 559 Atrophic glossitis (AG), 473, 479
erythema annulare centrifugum Antiepileptics, 594 Atrophic lichen planus, 191, 370
(EAC), 516–518 Antifungal agents, 604 Atrophic or hypertrophic scars, 102
erythema gyratum repens, 518 Anti-inflammatory drugs, 53 Atrophic plaques
erythema marginatum, 518 Antimalarial-induced lichen sclerosus et atrophicus (LSA),
erythema migrans (Erythema hyperpigmentation, 600 164–165
chronicum migrans), 518 Antimalarials, 53 morphea, 164
erythrokeratodermia variabilis, 540 Antineutrophil cytoplasmic antibodies Atrophoderma of pierini and pasini, 20
figurate erythema (gyrate (ANCA), 607 Atypical genital nevus, 407
erythema), 516 Anti-phospholipid antibody/lupus Atypical juvenile PRP, 182
fixed drug eruption (FDE), 518–519 anticoagulant syndrome Atypical mycobacterial infections, 159,
granuloma annulare, 521–523 (APLS), 323, 325, 350–351 210, 214, 295, 307, 340
Hansen’s disease, 528–529 Antithyroid, 559 Atypical mycobacterial lesions, 295
ichthyosis linearis circumflexa (ILC), Antitrypsin deficiency panniculitis, Atypical mycobacteriosis, 214, 295
539–540 340–341 Atypical pyoderma gangrenosum, 210
Jessner lymphocytic infiltration of the Aphthous stomatitis, 377 Auspitz phenomenon, 143
skin, 529–530 Aphthous ulcers, 378, 383, 434 Auspitz sign, 76, 145
keratoacanthoma centrifugum (aphtha, aphthous stomatitis, or Autoimmune blistering diseases, 68
marginatum, 536–537 canker sore), 431–432 Autoimmune destruction (vitiligo), 5
in leprosy, 528 clinical patterns of, 381 Autosomal dominant disease, 70
linear IgA bullous dermatosis Aplasia cutis congenita (ACC), 293–294, AV, see Acne vulgaris
(LABD), 537 295, 559
livedo reticularis, 519–520 Aplasia cutis with focal alopecia, atrophy,
B
lupus vulgaris (LV), 530 and crusted ulcer, 560
necrobiosis lipoidica, 527 Aplastic anemia, 394 Bacillary angiomatosis, 95, 116, 427,
necrolytic migratory erythema Apocrine (cystic proliferation of apocrine 577, 581
(NME), 538 glands) hidrocystoma, 114, 114 Bacilli Calmette-Guérin (BCG)
neonatal lupus erythematosus, 525 Apophysomyces elegans complex, 344 vaccine, 294
neutrophilic figurate erythema Apoptosis of melanoblasts, 5 Bacillus anthracis, 301, 347
(NFE), 538 Appendageal tumors, 114, 124–126, 174 Bacterial abscesses, 214, 226
with papules, 417 clinical characteristics, 127 Bacterial adenitis, 214
pityriasis rosea, 523–524, 524 Arciform-to-annular plaques, 154 Bacterial folliculitis, 81, 102, 104, 569
porokeratosis, 534–536 Arcuate lesions, 514 and impetigo, 276–277
secondary syphilis, 525 Argasidae, 345 Bacterial osteomyelitis, 226
Sneddon-Wilkinson disease, 538 Arrector pili muscle, 207 Balanitis, 420
subacute cutaneous lupus Arterial and venous disease, 304 Balanitis xerotica obliterans, 421
erythematosus (SCLE), 524 Arterial anomalies, 580 Balanoposthitis, 409–410, 412, 421
tuberculosis verrucosa cutis, 536 Arterial insufficiency (ischemic) ulcer, Bannayan-Riley-Ruvalcaba syndrome, 28
urticaria, 525–526 350, 351 Bartonella bacilliformis, 577
Annular lichenoides dermatitis of youth Arterial thromboembolism, 349 Basal cell carcinoma (BCC), 70, 76, 85,
(ALDY), 15, 520 Arterial ulcer, 304 95, 108, 110, 114, 124–125,
Annular lichen planus, 424, 530–532, Arteriovenous malformation, 574, 588 127–128, 171, 171–172, 207,
532, 533 Arthritis, 116, 185, 209 217–219, 228, 297, 297, 421, 425,
Annular plaques, 181 Ash-leaf macules (ALMs), 5, 12–13, 532–533, 588
of leprosy, 529 13, 589 Basal cell nevus syndrome, 112, 135
Annular psoriasis, 417, 516 Ashy dermatosis of Ramirez, 39 Basaloid follicular hamartoma
Annular pustular psoriasis, 539 Aspergillosis, 348–349, 384 (BFH), 135
Annular sarcoidosis, 523, 530 Aspergillus (flavus, fumigatus, niger), Bat disease, 123
Annular tufted angioma, 527, 527 324, 347–348 Bathing trunk, 583
Annular urticaria, 516 Asymptomatic lesions, 5 Battered baby syndrome, 320
Anogenital wart, 420 Ataxia-telangiectasia, 622 Bazex-Dupré-Christol syndrome,
Anonychia/micronychia, 446–447 Atopic dermatitis (AD), 103, 111, 140, 112, 135
Anonychia of great toenails in 167, 194, 412–413, 496, 500, BCC, see Basal cell carcinoma
epidermolysis bullosa, 447 614, 623, 625 B-cell lymphoma, 164
Anorectal malformations, 580 Atopic dirty neck, 31 Beare-Stevenson syndrome, 124
Anorexia, 116 Atrichia with papular lesions (APL), 564 Beau’s lines, 457, 457, 457
 Index 635

Becker’s nevus, 19, 24, 34, 170 Blue neuronevus, see Blue nevus Burns, 353, 379
brownish patch with lesional Blue nevus, 23, 204–205, 588 chemical burns, 353–354, 368, 372,
hypertrichosis, 34 Blue or purple toe syndrome, see 375, 391
Beckwith-Wiedemann syndrome Cholesterol embolization classfication, 353
(BWS), 574 syndrome Buruli ulcer (Bairnsdale ulcer), 303,
Behçet’s disease, 379, 382–383, 394, Blue rubber bleb nevus syndrome, 202, 306–307
430–432, 432, 434 202, 208 Buschke-Lowenstein tumor, 425, 425
Bejel (endemic syphilis), 75 Body dysmorphic disorder, 504 Buschke-Ollendorff syndrome, 176
Benign adnexal tumor, 125 Bone marrow failure, 64
Benign angiofibroma, 76 Bony deformities, 580
C
Benign cephalic histiocytosis, 88 Borderline borderline (BB), 11
Benign cystic lesion, 558 Borderline lepromatous (BL), 11, 529 CADR, see Cutaneous adverse drug
Benign dermatosis, 228 leprosy, 182 reactions
Benign genital lentiginosis, 407 Borderline tuberculoid (BT), 11, 528 Café-au-lait macules (CALMs), 20–23,
Benign hamartomatous tumor, 108 leprosy, 144, 182, 183, 193 23, 24
Benign juvenile melanoma, 219 Borrelia infection, 193 Calcific uremic arteriolopathy, see
Benign lichenoid keratosis (lichen Botryomycosis, 338, 537 Calciphylaxis
planus-like keratosis), 163, 217 Bowenoid papulosis, 84, 169, 297, Calcifying epithelioma, 221
Benign mesenchymal melanoma, see 414–415 of Malherbe, 125
Blue nevus Bowen’s disease, 84, 168, 172–174, 297, Calcinosis cutis, 216, 222, 426
Benign neoplasm of the skin, 163 410, 415, 533 Calciphylaxis, 300, 304, 306, 325
Benign vascular tumor, 96 of glans penis, 438 calcific uremic arteriolopathy,
Benoxaprofen, 112 Brachyonychia/racquet nail, 447 351–352
Beta thalassemia, 480 Branchial cleft cyst, 331 with reticulate purpura, 351
Bevacizumab, 306 Branchial cyst, 334 CAMN, see Common acquired
Bier spots, 15 Brittle nails, 459, 459–460 melanocytic nevi
Bilateral lesions, 213 Brocq, peribuccal pigmentation of, 50 Cancer, 342
Bilateral nodules of tophi, 222 Brooke-Spiegler syndrome, 109, 109, Candida, 304, 348
Bilateral periorbital lichen planus 112, 218 Candida albicans, 366, 373, 475
pigmentosus, 42 Brown-black macules of lentigines, 28 Candidal balanoposthitis, 409
Bilateral pigmentary demarcation Brown or gray-brown reticulated Candida species, 409
line, 49 pigmentation, 60 Candidial folliculitis, 287–288
Bilateral symmetrical distribution Brown recluse spider bite, 347, 354 Candidial intertrigo, 413
(ichthyosis hystrix), 174 Bruise, 19 Candidiasis, 372–375, 410, 412, 417, 420,
Bilateral temporal recession of anterior Bubble hairs, 563 429, 429–430, 473
hairline, 548 Bubonulus, 427 Capecitabine, 210
Biotin deficiency, 482 Buccal mucosa, 365, 375, 377 Capnocytophaga (formerly dysgonic
Bipolaris spicifera, 355 Buerger’s disease, see Thromboangiitis fermenter type 2 [DF2]), 355
Birt-Hogg-Dubé syndrome, 106 obliterans Carbamazepine, 604
Bitot’s spots, 478 Bullae, 169 Carbuncle, 207
Black Death, 347 Bullous acrokeratotic poikiloderma of Carcinoma, 422
Black dot tinea capitis with alopecia, 546 Kindler and Weary, 58 Carney complex, 28
Black patch with lesional Bullous cellulitis, 249 Carotenemia and carotenoderma, 485
hypertrichosis, 25 Bullous erythema multiforme, 210 Catastrophic antiphospholipid antibody
Blanchable wheals, 189 Bullous fixed drug eruption, 242–243 syndrome, 347
Blaschkitis, 135 Bullous impetigo, 241–242, 514, 519 Cat scratch disease, 295, 302, 307
Blastomycosis, 159, 295, 341, 384 Bullous insect-bite reaction, 243 Cavernous hemangioma, 202
Bleeding disorders, 485 Bullous leukocytoclastic vasculitis, 240 Caver’s and miner’s fever, 123
Blistering distal dactylitis, 240–241 Bullous lichen planus (BLP), 263, 371 Cayenne pepper spots, 410
Blistering of lips, 625 Bullous pemphigoid antigen (BP180), 380 CD8+ T cell-mediated autoimmune
Bloom syndrome (congenital Bullous pemphigoid (urticarial phase), phenomena, 368
telangiectatic erythema), 58, 190, 259, 379–380, 428, 430 Cell-mediated immunity (CMI), 11
70, 614, 619, 621 Bullous scabies, 237–239 Cellulitis, 152, 155–156, 156, 210, 231,
Blowfly strike and fly-blown maggot Bullous SLE, 272, 625 302, 306
infestation, see Furuncular Burkholderia cepacia, 348 Central centrifugal cicatricial alopecia
myiasis Burkholderia mallei, 307 (CCCA), 554
Blue-black nodules, 202 Burned skin-like appearance, 627 Central nervous system (CNS), 122
636   Index

Cephalosporins, 604 Chronic cutaneous lupus erythematosus Condylomata accuminata, 418, 427
Cerebriform intradermal nevus, 124 (CCLE), 77, 183, 184 Confluent and reticulated papillomatosis
Cerebro-oculo-facial syndrome, 70 Chronic eczema, 74 (CRP), 68
Cervical idiopathic poikiloderma, 31 Chronic erythematous vulvitis without Congenital alopecias, 622
Cervical lymph nodes, 214 vaginitis, 143 Congenital bullous poikiloderma, 58
Chancres, 430–431, 432 Chronic glucocorticoid use and Congenital erythropoietic porphyria
on inner prepuce, 431 abuse, 289 (CEP), 616
Chancroid (soft chancre, ulcus molle), Chronic granulomatous dermatitis, 172 Congenital hemangiomas, 178, 580
432–433 Chronic kidney disease, 155 Congenital livedo reticularis, 520
Chancroid ulcers, 429 Chronic liver disease, 155 Congenital medial fronto-facial capillary
Cheilitis, 365, 400–401 Chronic local friction, 366 malformation, 571
actinic, 401 Chronic osteomyelitis, 335 Congenital melanocytic nevus (CMN),
angular, 401 Chronic paronychia, 468 19, 24, 34
contact, 400 Chronic plaque psoriasis, 162, 180, Congenital melanocytic nevus on
exfoliating, 401 188–189 scalp, 170
glandularis, 401–402 Chronic telogen effluvium (CTE), 559 Congenital milia, 112
granulomatosa, 402 Churg-Strauss syndrome, 322 Congenital or developmental sinus, 331
granulomatous, 402 Cicatracial pemphigoid, 380 branchial cleft cyst (lateral cervical
Chemical burns, 353–354, 368, 372, 375, Cicatricial pemphigoid (mucous cyst), 334
391; see also Burns; Thermal membrane pemphigoid), bronchogenic cysts (subcutaneous
burns 379–380, 421, 430 bronchogenic cysts), 334
Chemical leukoderma, 9 Cicatricial scarring alopecia, 554 dermal sinus tract (dermal spinal
Chemotherapy agents and ulcer, 306 Cimetidine, 559 tract), 334
Cherry angioma (Campbell de Morgan Circinate balanitis, 411–412, 412, 520, 520 dermoid cysts, 331
spots), 95–96, 576–577 C-kit gene mutation, 224 preauricular cysts and pits, 331
Cherry hemangioma, 418 C-kit proto-oncogene, 8 thyroglossal duct cyst, 331–332
Chewing pads, 497 Classical tinea lesions, 522 umbilical fistula, 334
Chikungunya fever, 30 Classic Kaposi sarcoma, 196 Congenital penile curvature, 427, 436
Child abuse, 485 Clear cell acanthoma, 217 Congenital pigmentary disorders, 58
Childhood Clofazimine, 53 Congenital pigmentary mosaic
granulomatous periorificial Clofazimine-induced condition, 5
dermatitis (CGPD), 111 hyperpigmentation, 600 Congenital plaques
LE, 622 Closed comedones, 120, 131 connective tissue nevus, 176
porphyria of dermatological Clostridium perfringens, 306 hemangioma, 177–178
importance, 615 Clouston syndrome, see Hidrotic Inflammatory linear verrucous
SLE, 626 ectodermal dysplasia epidermal nevus (ILVEN), 176
systemic lupus erythematosus (CSLE), Clubbing (Hippocrates fingers, milia-en-plaque, 174
625–626 acropachy), 451–452 nevus lipomatosis superficialis,
Chlamydia trachomatis, 338 Coagulation-factor deficiency, 318 175–176
Chloracne (metabolizing acquired Coalescing erosions on soft palate, 380 nevus sebaceous, 174
dioxin-induced skin Cobblestones, 109 verrucous epidermal nevus, 174–175
hamartomas/MADISH), 116, Cobb syndrome, 589 verrucous hemangioma, 176–177
117, 119 Coccidiodes immitis, 338 Congenital syphilis, 273–274
Chlormezanone, 604 Coccidioidomycosis, 338, 384 Connective tissue nevus, 176
Cholesterol embolization syndrome, 206, Coccydynia, 504 Contact dermatitis, 103, 140, 195, 496
323, 325, 349–350 Coccygeal dimple, 334 Contagious pustular dermatitis,
Chondrodermatitis nodularis helicis, 207 Cockayne syndrome (CS), 619, 621 see Orf
Chondroid syringoma, 221 Cold panniculitis, 215 Contagious viral infection, 377
Chromatophoroma, see Blue nevus Collagenomas, 170, 176, 176 Coral injuries, 355
Chromoblastomycosis, 159–160, 220 Colloid milium, 136–138, 138, 139, 231 Cordylobia, 339
Chromomycosis Coma blister, 240 Cordylobia anthropophaga, 230, 307
(chromoblastomycosis), 159 Comedones, 86, 102, 102–103 Cornu cutaneum, see Cutaneous horn
Chromonychias, 462–467, 465 Common acquired melanocytic nevi Corporis, 521
Chromosomal mosaicism, 37 (CAMN), 85, 123–125 Corpus alienum, 437–438
Chronic arsenicism, 70–71, 72 Compound nevus, 123, 123–124, 134 Corticosteroids, 112, 604
Chronic arsenicosis, 74 Condyloma acuminata, 395–396, induced hypopigmentation, 16
Chronic chewing, 368 415, 419 purpura, 317
 Index 637

Coumadin (coumarin, warfarin) linear IgA bullous dermatoses Cutaneous lesions, 196, 210, 222, 308
necrosis, 352 (LAD), 605 in PSEK, 189
Cowper’s and Tyson’s glands, 437 morbilliform (exanthematous) drug in sarcoidosis, 151
Cowpox, 215, 355 reaction, 597–599 Cutaneous lipoma, 425
COX-2 inhibitors, 604 neutrophilic eccrine hidradenitis, 606 Cutaneous lymphoid hyperplasia/
Coxsackievirus A16, 377 palmar-plantar lymphocytoma cutis, 152, 530
Crazy pavement dermatosis, 476 erythrodysesthesia, 606 Cutaneous lymphomas, 218
Crohn’s disease (cutaneous Crohn’s photo recall phenomenon, 609 Cutaneous mastocytosis, 169, 196
disease), 304, 334, 341, 402, PRIDE complex, 608 Cutaneous melanoma, 226, 407
433–435 psoriasiform drug reaction, 602 Cutaneous metastases of visceral
Crusted scabies, 194 radiation recall dermatitis, 609 neoplasms and melanoma, 232
Cryoglobulinemia, 325 SJS-TEN overlap (Lyell’s syndrome), Cutaneous mucormycosis, 343
Cryptococcosis, 122, 122–123, 131, 603–605 Cutaneous myiasis, 339
227, 384 Stevens-Johnson syndrome (SJS), Cutaneous necrotizing vasculitis, 198
Cryptococcus neoformans, 227 603–605 Cutaneous polyarteritis nodosa
Curth’s angle, 451 symmetrical drug-related (CPAN), 215
Cushing disease, 14 intertriginous and flexural Cutaneous pseudolymphoma, 210, 231
Cutaneous adverse drug reactions exanthema (SDRIFE), 596–597 Cutaneous schwannomas (CS), 220
(CADR), 594 toxic epidermal necrolysis (TEN), Cutaneous small vessel vasculitis
acneiform eruption, 601 603–605 (CSVV), 322
acral erythema, 606 Cutaneous amoebiasis, 339 Cutaneous sporotrichosis, 206
actinic keratoses, inflammation, 608 Cutaneous angiofibroma, 106 Cutaneous squamous cell carcinoma, 217
acute generalized exanthematous Cutaneous angiosarcoma, 218 Cutaneous tuberculosis, 226, 335
pustulosis (AGEP), 605 Cutaneous anthrax, 251, 301–302, Cutaneous vasculitis, 206, 296
chemotherapeutic agents, 595–596 306, 354 Cuterebra species, 339
clinical approach, 595 Cutaneous aspergillosis, 306 Cuticle, 445
drug hypersensitivity syndrome Cutaneous B-cell lymphoma, 210 Cutis rhomboidalis nuchae, 74
(DHS), 599–600 Cutaneous calcinosis (calcinosis Cyclosporine, 112
drug-induced bullous cutis), 230 Cylindromas, 109, 109, 218
pemphigoid, 605 Cutaneous candidiasis, 412 Cystic fibrosis, 402
drug-induced eccrine Cutaneous Crohn disease, 308 Cystic hygroma, 334
hidradenitis, 606 Cutaneous cryptococcosis, 122, 414
drug-induced erythema Cutaneous hallmarks of tuberous
D
nodosum, 606 sclerosis complex (TSC), 106
drug-induced erythroderma/ Cutaneous heparin necrosis, 352 Dabska tumor, 581, 587
exfoliative dermatitis, 600 Cutaneous horn, 357–359 Darier disease, 82, 67, 81–82, 109,
drug-induced lupus erythematosus, benign, 357 191–192, 192, 360, 397
602–603 Bowen’s disease on the abdomen, 358 Darier-Roussy sarcoidosis, 231
drug-induced pemphigus, 605 conditions, 357 Darier’s sign, 169, 224
drug-induced phototoxic on great toe, 358 Darier-White disease, 109, 173
reaction, 594 on lower lip, 358 Dark-brown macules on buccal mucosa
drug-induced pigmentation, 600–601 malignant, 357 and lip, 371–372
drug-induced pityriasis rosea-like on neck, 357 Dark circles, 31
reaction, 602 on penis, 358 Dark-walled (dematiaceous) fungi, 226
drug-induced pseudoporphyria, 605 premalignant, 357 Darling disease, 123
drug-induced urticaria/ on scalp, 358 Decubitus ulcers, 299–300
angioedema, 606 on tip of finger, 358 Deep-brown hyperpigmentation, 42
drug-induced vasculitis (DIV), 606 Cutaneous intraepithelial Deep fungal infections, 296, 340, 577
drug-induced xerosis, 606 adenocarcinoma, 173 Deep hemangiomas, 585
drug rash with eosinophilia Cutaneous larva migrans (creeping Deep mycoses, 232
and systemic symptoms eruption), 231, 339, 537–538 Deep xanthomas, 221
(DRESS), 599 Cutaneous leiomyomas, 207 Deficiency of various clotting factors, 484
erythema multiforme, 603 Cutaneous leishmaniasis (CL), 130, 130, Delusional parasitosis, 503–504
fixed drug eruption, 594–596 159–160, 207, 219, 219–220, Delusion of parasitosis, 339
flagellate dermatoses, 606 295–296, 301 Dematiaceous fungi, 159
hand-foot syndrome (HFS), 606 multiple crusted plaques and Demodex brevis, 107
lichenoid drug eruption, 602 ulcers, 198 Demodex folliculorum, 107
638   Index

Demodicosis, 81, 102, 107, 107 Diffuse poikiloderma, 58 Drug-induced eccrine hidradenitis, 606
gravis demodicosis, 107 Diffuse swelling of lower lip, 402 Drug-induced erythema nodosum, 606
Dental sinus, 335 Digital ulcerations, 302, 302 Drug-induced erythroderma/exfoliative
Denture-related stomatitis, 393 Dihydrotestosterone (DHT), 548 dermatitis, 600
Dentures, 373 Diiffuse bluish-black pigmented Drug-induced lupus erythematosus,
Depigmentation, 8 macule, 389 602–603
Depigmented macules and patches, 6 Dilated pore of winer, 125, 222 Drug-induced pemphigus, 605, 605
Dermacentor ticks, 346 Dimple, 204 Drug-induced photosensitivity, 594
Dermal melanocytic hamartoma, 19 Diogenes syndrome, 499 Drug-induced phototoxic reaction, 594
Dermal melanocytic nevus, 92 Diphtheria, 394 Drug-induced pigmentation, 52–53, 388,
Dermal melanocytoma, see Blue nevus Discharging sinuses, 341 480, 600–601
Dermatitis artefacta, 434, 499–500 in axilla in hidradenitis Drug-induced pityriasis rosea-like
Dermatitis herpetiformis, 190, 194–195, suppurativa, 207 reaction, 602
263, 380, 496, 504 or fistula, 333 Drug-induced pseudoporphyria, 605
Dermatitis of Jacquet, 413 Discoid lupus erythematosus (DLE), Drug-induced sclerosis, 190
Dermatitis passivata (dermatitis 77–78, 148, 148–149, 155, 183, Drug-induced ulceration, 304
neglecta), 499 218–219, 380, 393, 516, 530, Drug-induced urticaria/angioedema, 606
Dermatobia hominis, 230, 307, 339 565–567 Drug-induced vasculitis (DIV), 606
Dermatofibroma, 76, 96, 204, 204, 208, erythematous plaque, 149 Drug-induced xerosis, 606
216, 218, 220, 224, 226 healed lesion, 149 Drug rash with eosinophilia and
Dermatofibrosarcoma protuberans Linear lesion, 149 systemic symptoms
(DFSP), 218, 225–226, 226 Dissecting cellulitis (DC), 568–569 (DRESS), 599
Dermatopathia pigmentosa reticularis Disseminated cutaneous Drug reaction with eosinophilia
(DPR), 60–64 leishmaniasis, 198 and systemic symptoms
Dermatophyte infections, 289–290, Disseminated histoplasmosis, 180 (DRESS), 594
520–521 Disseminated intravascular Dyschromatosis symmetrica hereditaria
Dermatophytosis, 415–417, 429 coagulation, 206 (DSH), 69, 70
Dermatoses, 514 Disseminated sporotrichosis, 206 Dyschromatosis universalis hereditaria
with halo phenomenon, 9 Disseminated superficial actinic (DUH), 69–70, 72
papulosa nigra (DPN), 82, 90, 113, porokeratosis (DSAP), 536 Dyshidrotic eczema/pompholyx, 287
113–114, 134 Disseminated superficial PK (DSP), 536 Dyskeratosis congenita (DKC), 60,
papulosa nigra seborrheic Distal hypospadias, 437 63–64, 375, 619
keratosis, 169 Distal splinter hemorrhage, 470 Dyspigmentation, 620
Dermoid cyst, 127, 205, 334, 427 Distorted penis in lymphogranuloma of median raphe, 409
Desmoid tumor, 220 venereum, 439 Dysplastic nevus, 407
Developmental and acquired sinuses and DLE, see Discoid lupus erythematosus
fistulae, 332 DNA
E
Diabetes mellitus, 112, 155, 172 parapox virus, 215
Diabetic bulla/bullous diabeticorum, 240 poxvirus, 130 Earlobe keloid, shiny nodule in, 216
Diabetic dermopathy, 32 Dome-shaped translucent swelling, 398 Ecchymosis, 316
Diaminodiphenyl sulfone, 600 Donovanosis, 425, 433 in sun-exposed area, 318
Diaper dermatitis, 167, 413, 430 Dorsal pterygium, 462, 462 Eccrine
Dietzia papillomatosis, 67 Double-stranded DNA virus, 215 angiomatous hamartoma, 232
Diffuse AA, 541, 562 Dovitinib, 112 (cystic dilatation of intradermal sweat
Diffuse acanthosis nigricans, 198–199 Dowling-Degos disease (DDD), 66, 69 ducts) hidrocystoma, 114, 114
Diffuse alopecia areata, 544, 550, 560, Down syndrome, 112, 374 poroma, 217
562–564 Doxycycline-induced phototoxic spiradenoma, 208
Diffuse cutaneous mastocytosis, 265 reaction, 596 Ecthyma, 300
Diffuse dark-brown irregular pigmented Drug eruptions, 194, 210, 377, 409 contagiosum, see Orf
macules, 389 Drug hypersensitivity syndrome (DHS), gangrenosum, 250–251, 300, 302, 306,
Diffuse erythema, 400 599–600 346, 346–348
Diffuse facial hyperpigmentation, 40 Drug-induced bullous Eczema, 423
Diffuse lepromatous leprosy, 323 disorder, 421 molluscatum, 216
Diffuse normolipemic plane pemphigoid, 605 Edematous swelling, 437
xanthoma, 221 Drug-induced delayed multiorgan Ehlers-Danlos syndrome, 112, 124
Diffuse patches of scaling and hair loss, hypersensitivity syndrome Ekbom syndrome, see Delusional
544, 544 (DIDMOHS), 599 parasitosis
 Index 639

Elastolytic giant cell granuloma, 526 Eruptive histiocytosis, 88 Erythematous nodules, 208, 211, 215, 568
Elastosis perforans serpiginosa Eruptive lobular capillary with central umbilication seen in
(EPS), 523 hemangioma, 116 giant molluscum, 216
Embolia cutis medicamentosa, see Eruptive LP, 92 of tufted angioma, 581
Nicolau syndrome Eruptive vellus hair cyst, 86, 92 with verrucous surface, 584
Enalapril, 559 Eruptive xanthoma, 112, 523 Erythematous noduloulcerative
Endemic typhus, 347 Erysipelas, 152, 153, 156, 156, 169, 209, lesions, 225
Endogenous chromonychia, 463 249–250 Erythematous papular lesions, 418
Endogenous pigments, 387 Erysipeloid, 152, 156, 251 Erythematous papules, 102, 104, 128,
Endometrioma, 208 Erythema, 8, 81, 308 195, 608
Entamoeba histolytica, 339 ab igne, 68, 520 of juvenile xanthogranuloma, 137
Enterocutaneous fistula (ECF), 342 annulare centrifugum (EAC), 193, Erythematous papulonodular
Environmental mycobacteria, 214 516–518, 517, 527 lesions, 120
Eosinophilic cellulitis, 213 clinical forms, 518 of progressive nodular
Eosinophilic fasciitis, 190 chronicum figuratum histiocytosis, 137
Eosinophilic granulomatosis, 322 melanodermicum, 39 Erythematous patches, 481
Eosinophilic ulcer, 384 dyschromicum perstans (EDP), 30, of psoriasis, 420
Epidemic typhus, 347 39–40 Erythematous plaques, 120, 181–182,
Epidermal atrophy, 68 elevatum diutinum, 152, 155, 186, 184, 186
Epidermal cysts, 88, 221 218, 221 alopecia mucinosa, 152–153
Epidermal growth factor (EGF), 227 gyratum repens (EGR), 193, 518 cellulitis, 155–156
Epidermal growth factor receptors induratum, 209–210, 232, 341 cutaneous lymphoid hyperplasia/
(EGFR), 306 induratum of Bazin, 214–215 lymphocytoma cutis, 152
Epidermal inclusion cyst, 125, 138, 222, 427 marginatum, 193, 518 discoid lupus erythematosus (DLE),
Epidermal nevus, 86 migrans (Erythema chronicum 148–149
Epidermal or synovial cysts, 226 migrans), 518 erysipelas, 152
Epidermodysplasia verruciformis (EV), migrans of Lyme disease, 354 erythema elevatum diutinum, 152
86, 199–200 multiforme (EM), 180, 186, 189, 190, granuloma annulare, 154
Epidermoid cysts, 110, 114, 126, 126, 201, 216, 235, 376, 378, 411, 430, granuloma multiforme, 154
126–127, 131, 218, 221, 228, 232, 476, 526, 603, 608 Jessner’s lymphocytic infiltrate, 151
331, 334–335, 425–426 multiforme-like lupus, 201 leprosy, 143–148
with central punctum, 334 multiforme major, 379 pagetoid reticulosis, 153
Epidermolysis bullosa (EB), 235, 381 totelangiectatic rosacea, 104–105 polymorphic light eruption (PLE),
acquisita (EBA), 261, 379–381 Erythema nodosum (EN), 186, 208–210, 149–151
simplex, 61, 69 215, 341, 581, 608 psoriasis, 143
with mottled pigmentation, 68 leprosum (ENL), 196–198, 209–210, sarcoidosis, 151–152
Epidermolytic hyperkeratosis, 199 215, 230 tumid lupus erythematosus, 155
Epidermolytic ichthyosis (bullous necroticans, 210 xanthoma, 154–155
congenital ichthyosiform Erythematous, 93, 181, 192, 198 Erythematous scaly papules, 105
erythroderma), 272, 540 papules, 79, 81, 97 Erythematous scaly plaque in Bowen’s
Epiphora, 64 shiny papules, 80 disease, 172
Epithelial cysts, 220 shiny subcutaneous nodules, 209 Erythema with erythematous
Epithelial hyperplasia, 395 subcutaneous nodules, 209 papules, 105
Epithelioid Erythematous and purpuric lesion, 604 Erythrokeratodermia variabilis, 189,
hemangioma, 585 Erythematous and skincolored 516, 540
sarcoma, 229 infiltrative plaques, 199 Erythromelalgia, 303
and spindle-cell nevus, 219 Erythematous atrophic plaque, 393 Erythromelanosis follicularis faciei
Epithelioma of Malherbe, 205 Erythematous crusted plaque, 160 (EFF), 104, 105
Epstein–Barr virus, 368, 394 Erythematous edematous plaques, 197 Erythromelanosis follicularis faciei et
Erosions Erythematous macules, 182, 183 colli (EFFC), 48, 50, 104
in child with scabies, 416 and patches, 576 Erythroplakia, 393
on tongue, 378 Erythematous monomorphic Erythroplasia of Queyrat, 143, 297, 410,
Erosive lichen planus, 369–370, 378, 380, papules, 119 420–421, 421
414, 423, 430 Erythematous mucosal lesions, vascular Erythropoietic protoporphyria, 616–617
Erosive mucositis, 306 causes of, 391 Erythrose péribuccale pigmentaire
Erosive pustular dermatosis of scalp Erythematous nodular lesions of nodular de Brocq (peribuccal
(EPDS), 276, 569 scabies, 416 pigmentation of Brocq), 48–49
640   Index

Eschar, 343 Exfoliation, 477 erythromelanosis follicularis faciei

amebic infections, 355 Exfoliative dermatitis, 599 (EFF), 104
anthrax, 348 Exogenous chromonychia, 463 facial angiofibroma, 106–107
antiphospholipid antibody/lupus Exogenous ochronosis (EO), 44, 44 facial idiopathic granulomas with
anticoagulant syndrome Exophiala jeanselmei, 355 regressive evolution (FIGURE),
(APLS), 350–351 Extracellular matrix protein 1 107–108
arterial insufficiency (ischemic) (ECM1), 139 fibrous papule of the nose, 124
ulcer, 350 Extracutaneous abnormalities, 70 follicular mucinosis (FM), 115
aspergillosis, 348–349 Extrafacial melasma, 19 granuloma annulare, 120
burns, 353 Extragenital lichen sclerosus, 191 granuloma faciale, 130
calciphylaxis/calcific uremic Extramammary Paget’s disease, 420 hidrocystoma, 114
arteriolopathy, 351–352 Exudative discoid, see Sulzberger-Garbe in histiocytoses, 136, 136
capnocytophaga (formerly dysgonic disease histoplasmosis, 123
fermenter type 2 [DF2]), 355 infantile hemangioma, 128–129
chemical burns, 353–354 keratoacanthoma, 129–130
F
cholesterol embolization syndrome, Keratosis follicularis, 109
349–350 Facial acanthosis nigricans, 44 keratosis follicularis spinulosa
clinical approach to diseases, 344 Facial actinomycosis, 339 decalvans, 135–136
clues to diagnosis, 344 Facial Afro-Caribbean childhood keratosis pilaris atrophicans
coumadin (coumarin, warfarin) eruption (FACE), 111 (KPA), 135
necrosis, 352 Facial angiofibromas, 84, 106, 106, lepromatous leprosy, 119–120
cowpox, 355 106–107 lipoid proteinosis, 139
ecthyma gangrenosum, 346–348 Facial atopic dermatitis, 103 lobular capillary hemangioma (LCH),
flap necrosis, 352 Facial idiopathic granulomas with 127–128
Fournier gangrene, 345 regressive evolution (FIGURE), lupus miliaris disseminatus faciei
frostbite, 353 107–108 (LMDF), 107–108
heparin necrosis, 352–353 Facial lesions, 571 milia, 112–113
hyalohyphomycosis, 355 Facial melanosis, 39 molluscum contagiosum (MC),
injuries caused by fire coral and coral Facial melasma, 39 130–131
cuts, 355 Facial molluscum contagiosum, 130 neurofibroma, 131
loxosceles, 354 Facial papules, 100 nevus sebaceous, 131–132
Lucio’s phenomenon, 350 acne vulgaris (AV), 100–103 papular scar, 114–115
Milker’s nodule, 355–356 age of presentation, 101 periorificial dermatitis (perioral
mucormycosis (zygomycosis), angiolymphoid hyperplasia with dermatitis), 110–111
343–344 eosinophilia (ALHE), 116 pilomatricoma, 125–126
necrotizing fasciitis, 344 atopic dermatitis, 103 post-kala azar dermal leishmaniasis
phaeohyphomycosis, 355 basal cell carcinoma (BCC), 124–125 (PKDL), 116
plague, 348 basaloid follicular hamartoma pseudofolliculitis barbae (PFB)
pressure ulcer (decubitus ulcer), 352 (BFH), 135 (shaving bumps), 118
purpura fulminans, 349 chloracne (metabolizing acquired rosacea, 104–106
rat-bite fever (sodoku, spirillum dioxin-induced skin sarcoidosis, 115
minor), 355 hamartomas/MADISH), 116 sebaceous gland hyperplasia (SH), 110
rickettsia infection, 345–346 clinical approach, 100–101 seborrheic keratosis (SK), 134
snakebite, 354 clinical characteristics, 101–102 secondary syphilis, 121–122
tegenaria, 355 colloid milium, 136–138 steroid acne, 119
tularemia, 346 common acquired melanocytic nevus sycosis barbae, 117
Escherichia coli, 345, 348 (CAMN) (mole), 123–124 syringoma, 111–112
Essential fatty acids (EFA) deficiency, cryptococcosis, 122–123 tinea barbae, 117–118
478, 482 cutaneous leishmaniasis (CL), 130 Tinea faciei, 139–140
Etiopathological processes, 100 cylindroma, 109 traumatic anserine folliculosis,
Eumycetoma, 338 Darier disease, 109 118–119
Excoriated papules Darier-White disease, 109 trichoepithelioma, 108
of prurigo simplex, 496 demodicosis, 107 trichofolliculoma, 127
of scabies, 416 dermatosis papulosa nigra (DPN), Trichostasis spinulosa (TS), 116–117
Excoriations with secondary infection in 113–114 verrucous epidermal nevus, 134–135
scabies, 416 dilated pore of winer, 125 viral warts, 132–133
Exfoliating cheilitis, 401 epidermoid cyst, 126–127 Facial picking disorder, 500
 Index 641

Factitious cheilitis, 497, 497 Follicular nodule, 207 Genital psoriasis, 143
Factitious panniculitis, 341 Follicular papules, 153 Genital warts, 84, 84, 228, 425
Familial cutaneous collagenoma Follicular plugging, 148 Genitourinary tuberculosis, 421–422
(FCC), 176 Folliculitis, 118, 207 Genodermatoses, 112
Familial dysbetalipoproteinemia, 155 Folliculitis decalvans (FD), 276, 568–569 Geographic-shaped ulcer, 499
Familial dyskeratotic comedones, 94 Foreign bodies Geographic tongue, 370–372, 372, 394
Familial Mediterranean fever, 209 cysts, 226 Gianotti-crosti syndrome, 97, 98
Fanconi anemia, 64–65 granuloma, 126, 434 Giant acrochordon, 227
Farcy, see Glanders disease inflammatory reaction, 118 Giant cauliflower-like plaques, 425
Fatigue, 116 Fournier gangrene, 345, 345, 434–435 Giant condyloma, 424
Favre-Racouchot syndrome, 114, 222 Fragile X syndrome, 124 Giant molluscum, 222, 230
Favus, 546 Francisella tularensis, 346 contagiosum, 216, 216
Female androgenetic alopecia, 549 Freckles, 388 Gingival fibroma, 399
Female pattern hair loss, 554 ephelides, 19, 26, 31, 36, 47, 48 Gingival hyperplasia, 365, 399–400
FERMT1 gene, 58 and lentigines, 388 causes, 400
Fever, 180, 209 Friar Tuck type of trichotillomania, Glanders disease, 307
Fibrin, 375 556, 557 Glans penis, 419
Fibroepithelial polyps, see Skin tags Frictional melanosis, 53, 53 in candidiasis, 409
Fibrofolliculoma, 114 Friction blister, 243 Glomus tumors, 205, 208, 470, 587
Fibromas, 226, 228, 398–399 Frontal fibrosing alopecia (FSA), 551, Glomuvenous malformations
Fibrous histiocytoma, 434 551–553, 552 (glomangioma) (GVMs),
Fibrous papule of nose, 76, 78, 124, 124 Frostbite, 302, 353 581, 587
Fibrous papules of face, 125 Frostnip, 302 Glossodynia, 504
Fiddle-back spiders bite, 354 5-FU, 594 Gloves-and-socks syndrome, 323
Figurate erythema (gyrate erythema), Fungal eumycetoma, 336 GM1 gangliosidosis, 20
193, 516, 516 Fungal infections, 120, 219, 412 Goltz-Gorlin syndrome, 114
Filiform warts, 132 cryptococcus, 434 Goltz syndrome, 223
Fire stains, 68 Furuncle (boils), 207, 419 Gonococcemia, 206
Fish tank granuloma, see Swimming pool Furuncular myiasis, 230–231, 339 Gonorrhoea, 437
granuloma Furunculosis, 207, 231, 339 with purulent discharge, 437
Fissured tongue, 372, 374 Fusarium (solani, oxysporum, Gorlin syndrome, 135
with geographic tongue, 375 verticillioides), 324, 348 Gottron papules, 96
Fistula (odontogenic fistula), 335 Futcher’s line, 47 Gougerot-Blum syndrome, 318
Fitzpatrick sign, 204 Gout, 335
Fitzpatrick skin type, 224 multiple tophi on toes, 222
G
Fixed drug eruption (FDE), 3, 30, 219, nodulosis, 222
388, 410–411, 413, 430–431, Galli-Galli disease, 66–67 Gouty nodulosis, 152
476, 518–519, 594–596 Gardner-Diamond syndrome, see Gouty tophi, 91, 216, 228
Flagellate dermatoses, 606 Psychogenic purpura Graft-vs-host disease, 608
Flap necrosis, 352 syndrome Gram negative folliculitis, 283, 341
Flea-borne zoonosis, 347 Gaucher disease, 622 Granular cell tumor, 208, 434
Flexors, 66 Gefitinib, 210, 306 Granular parakeratosis, 81
Flexural psoriasis, 420, 430 Generalized exanthematous pustulosis Granuloma
Flu-like syndrome, 338 (AGEP), 594 gluteale infantum, 413
Fluoroquinolones, 594 Generalized morphea, 190, 191 inguinale, 340, 422, 433
Fluorouracil, 112 Genetic predisposition, 29 multiforme, 154, 187
Focal dermal hypoplasia, 223 Genetic syndromes, 28 pyogenicum, 127, 217, 418,
Focal epithelial hyperplasia (Heck’s Genital aphthosis, 430 574–576, 585
disease), 396–397 Genital foreign body (corpus alienum), telangiectaticum, 127
Focal scarring alopecia, 559 437–439 Granuloma annulare (GA), 30, 79, 120,
Focal ulceration, 215 Genital herpes, 409, 410, 411, 428, 121, 148, 154, 180, 186–188,
Focal vitiligo, 9 429, 433 193, 221, 516, 521–523, 522, 523,
Folate deficiency, 478–479, 482 Genital involvement in riboflavin 527, 530
Follicular blockage, 100 deficiency, 476 Granuloma faciale (Lever type), 130, 130,
Follicular degeneration syndrome, 554 Genital leiomyoma, 207–208 218–219, 585
Follicular infundibulum, tumour of, 15 Genital lesions, 190, 410, 422 Granulomatosis with polyangiitis (GPA),
Follicular mucinosis (FM), 115 Genital lymphedema, 435 296, 322
642   Index

Granulomatous cheilitis, 402, 402 β-Hemolytic streptococci infection, 475 Hospital addiction, see Munchausen
Granulomatous eruption, 80 Hemophagocytic syndromes, 434 syndrome
Granulomatous periorificial Hemorrhagic necrosis, 308 Hot comb alopecia, 554
dermatitis, 115 Henoch-Schönlein purpura, 322, 322 Human immunodeficiency virus (HIV)
Granulomatous secondary syphilis, 186 Heparin-dependent antiplatelet infection, 368, 422
Gravitational purpura, 213 antibody, 353 Human papillomavirus, 395
Grayish macular pigmentation, 51 Heparin-induced necrosis, 306 Human papilloma virus (HPV), 84,
Gray patches of tinea capitis, 545 Heparininduced thrombocytopenia and 162, 415
Greither syndrome, 540 thrombosis (HITT), 306 Human scabies, 194
Griseofulvin-induced phototoxic Heparin-induced thrombocytopenia Hunter syndrome, 98
reaction, 597 (HIT), 315–317, 352 Hutchinson–Gilford syndrome, 74
Grover’s disease, 254 Heparin necrosis, 352–353 Hutchinson’s sign, 469
Gumma, 222–223 Heparin skin necrosis, 325 Hyalohyphomycosis, 355
Gumma lesion, 383 Hepatoerythropoietic porphyria, 69 Hydroa vacciniforme (HV), 624–625
Gummatous syphilis, 214 Herald patch of pityriasis rosea, 167 Hydrochlorothiazide, 594
Guttate, 5 Hereditary acrokeratotic Hydroxyurea, 306
Guttate macules, 6 poikiloderma, 58 Hypergammaglobulinemic purpura of
Guttate psoriasis, 96, 193, 525 Hereditary vitamin D-resistant rickets Waldenstrom, 318
with alopecia, 564 Hyperhidrosis, 116
Herpangina, 376–378 Hyperkeratinization, 100
H
Herpes genitalis, 428–431, 433 Hyperkeratotic eczema, 143, 153
Haber’s syndrome, 66 in immunosuppressed patient, 433 Hyperkeratotic papules, 109, 162
Habit tic deformity, 458, 458 Herpes labialis, 376 Hyperkeratotic plaques, 177
Haemophilus influenzae, 347 with secondary infection, 376 benign lichenoid keratosis (lichen
Hailey–Hailey disease (HHD), 81, 173, Herpes simplex, 246, 279–280, 376, 378, planus-like keratosis), 163
269, 430 383, 391, 434 chromomycosis
Hair loss, 146 infection, 376 (chromoblastomycosis), 159
objective assessment, 560 virus infection, 375–376 cutaneous leishmaniasis (CL),
short broken, 564 Herpes simplex virus (HSV) type 1, 375 159–160
in trichotillomania, 557 Herpes simplex virus (HSV) type 2, keloids/hypertrophic scar, 163–164
Hair shaft disorders, 562, 563 375, 428 lichen planus hypertrophicus,
Hairy leukoplakia, 368, 375 Herpes zoster (HZ), 3, 246–247, 288–289, 161–162
Hairy tongue, 372–373, 373 299, 376, 376, 438 lichen simplex chronicus, 161
Half and half nails, 464 Herpetic gingivostomatitis, 375 lupus vulgaris, 157–158
Halogens, 606 Herpetic whitlow, 215 sporotrichosis, 159
Halo nevus, 8–9, 516 Herpetiform aphthous ulcer, 376 tuberculosis verrucosa cutis, 156–157
Halo phenomenon, dermatoses, 9 Hidradenitis suppurativa, 207, 214, 226, verruca vulgaris, 162
Hamartoma of dermal melanocytes, 19 288, 336, 340–341, 433 Hyperkeratotic verrucous plaque, 161
Hamartomatous disorder, 221 Hidrocystoma, 114, 114, 252–253 Hyperlipidemia, 116
Hamilton-Norwood classification, 548 Hidrotic ectodermal dysplasia, 61 Hyperoxaluria, 352
Hand, foot, and mouth disease, 237, Hippocrates fingers, 451–452 Hyperpigmentation, 19, 37, 44, 51, 58, 61
377–378 Histiocytoid hemangioma, 585 dystrophic nails, 70
Hand-foot syndrome (HFS), 606–607 Histiocytoses, 136 of friction, 52
Hansen’s disease, 9, 80, 119, 130, 180, 516, facial papules in, 136 of nails, 116
528–530 Histoid Hansen’s disease, see Histoid reticulated, 64, 66
Hartnup disease, 622 leprosy Hyperpigmented macules and patches,
Hartnup syndrome, 482 Histoid leprosy, 221, 229–230 localized, 19
Healed lesions, 596 Histoid nodules, see Histoid leprosy acquired brachial cutaneous
Heavy-metal deposition, 388 Histoplasma capsulatum, 227 dyschromatosis (ABCD), 33
Hemangioblastoma, 299 Histoplasmosis, 123, 227, 384 acral lentiginous melanoma (ALM),
Hemangioma, 95–96, 177, 177–178, 217, HIV infection/AIDS, 155 32–33
223, 417–418, 418 Hobnail hemangioma, 96, 587, 587 actinic lichen planus (LP), 30
Hemangiopericytoma, 581 Hodgkin’s disease, 341 atopic dirty neck, 31
Hematologic malignancies, 485 Hoffman disease, 568 Becker’s nevus, 34
Hemochromatosis, 480 Homogentisic acid accumulation, 44 Café-au-lait macules (CALMs),
Hemolytic streptococci, 347 Hori nevus/acquired bilateral nevus of 20–23
Hemolytic-uremic syndrome (HUS), 317 Ota-like macules (ABNOM), 47 clinical approach, 20
 Index 643

congenital melanocytic nevus corticosteroid induced Idiopathic thrombocytopenic

(CMN), 24 hypopigmentation, 16 purpura, 315
diabetic dermopathy, 32 halo nevus, 8–9 IgA pemphigus, 258–259
erythema dyschromicum perstans hypomelanosis of ITO Imatinib-induced phototoxic
(EDP), 30 (incontinentia pigmenti reaction, 597
freckles (ephelides), 26 achromicans), 15–16 Imidazoles, 594, 604
lentigo simplex, 27–28 idiopathic guttate hypomelanosis Immune thrombocytopenia (ITP), 484
lichen planus pigmentosus (LPP), (IGH), 14 Immunoglobulin G (IgG) antibodies, 190
24–26 leprosy, 11 Impetigo, 417, 429
Lifa disease, 35 nevus anemicus, 7–8 Impotence, 116
Linea nigra, 36 nevus depigmentosus (nevus Inclusion cyst, 205
linear and whorled nevoid achromicus), 5–7 Incontinentia pigmenti (verrucous stage),
hypermelanosis (LWNH), 37 phylloid hypomelanosis, 13 64, 201, 247–248
macular amyloidosis (MA), 34–35 physiologic anemic macules (Bier achromicans, 15–16
melasma, 29–30 spots), 15 Indeterminate leprosy (IL), 11
Mongolian spot, 19–20 piebaldism, 8 Infantile acropustulosis, 283–284
Nevus of Hori, 31 pityriasis alba, 5 Infantile fibrosarcoma, 585
Nevus of Ito, 19 pityriasis versicolor (Tinea Infantile hemangioma (IH), 128–129,
Nevus of Ota, 19 versicolor), 13 177, 177, 211, 294, 527, 574,
Nevus spilus (speckled lentiginous post-inflammatory 576, 576, 577–580, 578–579,
nevus), 23–24 hypopigmentation, 16 580–581
notalgia paresthetica (NP), 34 post kala-azar dermal Infections, 304
periorbital hyperpigmentation, 31 leishmaniasis, 12 Infectious mononucleosis, 394
pityriasis versicolor (tinea tumour of follicular infundibulum, 15 Infestation, 504
versicolor), 33 vitiligo, 9 Inflamed seborrhoeic keratosis, 217
poikiloderma of civatte, 31–32 Woronoff’s ring, 9 Inflammation of pilosebaceous units, 100
post-chikungunya pigmentation Hypohidrosis, 60 Inflammatory bowel disease (IBD), 342
(PCP), 30–31 Hypomelanosis, 58 Inflammatory disorders, 304
post-inflammatory of ITO (incontinentia pigmenti Inflammatory lesions, 100, 102
hyperpigmentation (PIH), achromicans), 15–16, 16 Inflammatory linear verrucous
35–36 Hyponychium, 445 epidermal nevus (ILVEN),
progressive cribriform and Hypopigmentation 92–93, 176
zosteriform hyperpigmentation atrophic, 64 Inflammatory skin disease, 68
(PCZH), 36–37 macules, 120 Inflammatory tinea capitis, 546, 546
regional distribution, 62 mycosis fungoides, 15 Infundibular cysts, 86
Schamberg’s disease, 32 Woronoff ring, 9 Infundibular folliculitis, 119
segmental lentigines, 28–29 Hypoplastic dermatoglyphics, 60 Ingrown nails/onychocryptosis, 469
solar lentigo, 26–27 Hypospadias, 437 Inherited epidermolysis bullosa, 273
Hyperpigmented macules of Hypotrichosisdeafness syndrome, 61 Injuries caused by fire coral and coral
lentigines, 409 Hypozincemia (Zinc deficiency), cuts, 355
Hyperpigmented nodules on the 480–482 Insect-bite reactions, 224, 231, 300,
dorsum, 212 339, 496
Hyperpigmented patch, 33 Insect bites, 209
I
Hyperpigmented solitary papule, 78 Insect dermatitis, 96, 98
Hyperpigmented verrucous papules, 174 Iatrogenic KS, 217 Integrin B4, 380
Hypertension (Martorell ulcer), 304 Ichthyosis follicularis alopecia Internal malignancy, 134
Hyperthyroidism, 112 photophobia (IFAP) Intertrigo, 173, 410
Hypertrichosis, 116 syndrome, 136 Intestinal polyps (hamartomas), 390
Hypertrophic lichen planus, 371, 496, 536 Ichthyosis linearis circumflexa (ILC), Intradermal melanocytic nevus, 124
Hypertrophic scar, 163, 163–164, 180, 216 539, 539–540 Intradermal nevus, 84, 114, 124–125
Hypervitaminosis A, 478 Ichthyosis linearis circumflexa in Ipsilateral sclera, pigmentation, 19
Hypoaesthesia, 146 Netherton syndrome, 540 Iritis, 209
Hypo/depigmented macules, localized, 5 Idiopathic cutaneous hyperchromia, 42 Iron deficiency, 451
annular lichenoides dermatitis of Idiopathic guttate hypomelanosis (IGH), Irradiation, 342
youth (ALDY), 15 14, 15 Irritant contact dermatitis (ICD),
ash-leaf macules (ALMs), 12–13 Idiopathic livedo reticularis, 520 412–413, 498
clues, clinical, 6 Idiopathic sclerosis, 190 Ischemic ulcers, 350
644   Index

Isolated acral hemorrhagic, 109 Keratosis of unknown significance Lesional leucotrichia, 12

Isoniazid toxicity, 482 (KUS), 368 Lesional skin, 7
Isotretinoin-induced xerosis, 607 Keratosis pilaris, 86, 90, 117, 119 Lesions, 28, 31, 67, 100–101, 207
Itching purpura, 318 Keratosis pilaris atrophicans (KPA), 135 color, 180, 365
Itchy red papules, 414 Keratotic papules, 83 of drug-induced erythema
Itraconazole, 594 Kerion, 545, 546, 568 multiforme, 603
Ixodidae, 345 Ketoprofen, 594 by environmental (nontuberculous)
Ketron–Goodman pagetoid reticulosis, mycobacteria, 336
153, 187 of granuloma, 523
J
Kimura’s disease, 96, 116, 585 in lepromatous pole, 143
Janeway lesions, 206 Kindler syndrome, 58, 70, 619, 622 of lobular capillary hemangioma, 128
Jaundice, 485 Kindler–Weary syndrome, 58 oral mucosal, 365
Jessner’s lymphocytic infiltration, 79, 81, Kissing melanocytic nevus on penis, 409 oral pathology based on morphology
151–152, 155 Klebsiella granulomatis, 433 of, 366
of the skin, 529–530 Klippel-Trenaunay and Proteus pigmented, 365
Junctional melanocytic nevus, 27, syndrome, 589 red, 365
123, 407 Klippel-Trenaunay syndrome (KTS), 213, white, 365
Junctional nevus, 134 572, 574, 582 Leucoderma syphiliticum, 74
Juvenile dermatomyositis (JDM), 626 Knife cut ulcers in Crohn disease, 308 Leukemia/bone marrow failure, 315
Juvenile-onset PRP, 180 Koebnerization, 9, 143, 145 Leukemias, 394, 626
Juvenile spring eruption (JSE), 623–624 Koebner phenomenon, 90, 514 cutis, 210
Juvenile xanthogranuloma (JXE), 87–88, Koilonychia (spoon nails), 451, 451 infiltrates, 186
137, 155, 196, 219–220, 220, 221 KRT5 gene, 66, 68 Leukocytoclastic vasculitis, 152
Juxtaclavicular beaded lines, 110 KRT14 gene, 68 Leukoedema, 368
Kyrle’s disease, 83, 212 acquisitum centrifugum, 8
of scleroderma, 74
K
Leukonychia, 464–465
L
Kangri basket, 300 in onychomycosis, 464
Kangri ulcer, 300 Lamellar nail dystrophy, 460 Leukoplakia, 366–368, 375, 401
Kaposiform hemangioendothelioma, Laminin 5, 380 Lichen
95–96, 211, 218, 527, 581 Lanceolate-shaped alopecic patch, 553 amyloidosis, 83
Kaposi sarcoma (KS), 95–96, 178, 196, Langerhans cell histiocytosis, 88, 137, aureus, 320, 321
211, 213, 217–218, 226, 299, 425, 167, 220, 269, 413, 433–434, 614 myxedematosus, 139
527, 576–577, 585–586 Large-cell lymphoma, 210 nitidus, 90, 92, 414, 419
AIDS related, 217 Large-plaque parapsoriasis, 193 planopilaris, 554, 564–565,
classic type, 217 Leiomyoma, 207–208, 227, 231 566, 568
endemic type, 217 Leishmania major, 295 scrofulosorum, 90
iatrogenic immunosuppression, Leishmaniasis, 159, 219, 231, 294–295 simplex, 174
196, 217 recidivans, 160 spinulosus, 90, 119, 414
Kaposi varicelliform eruption, 267 Leishmania tropica, 295 striatus, 92–94, 414
Kasabach-Merritt phenomenon Lentigines, 23, 28, 28, 31, 36, 47, 48, 87, Lichenified plaque, 496
(KMP), 581 407–409 Lichenoid
Keloidal lesions, 556 Lentigo, 388, 588 chronic dermatitis, see Sulzberger-
Keloids, 84, 163, 163–164, 208, 216, 230 Lentigo simplex, 27–28 Garbe disease
Keratinization, 67 LEOPARD syndrome, 28, 621 drug eruption, 30, 602
disorder of, 168 Lepra reaction, 180 drug reaction, 370, 602
Keratinocytes (pityriasis alba), 5 Lepromatous leprosy (LL), 11, 14, 116, keratosis, 27
Keratinous debris, 499 119–120, 131, 182, 183, 198, papules, 30
Keratoacanthoma, 125, 129–130, 383, 434 sarcoidosis, 88
207, 424 Lepromatous nodules, 230 Lichen planus, 76, 78, 79, 83–86, 96, 112,
centrifugum marginatum, Leprosy (Hansen’s disease), 11, 115, 122, 148, 153, 174, 183, 186, 368–370,
536–537, 537 143–148, 152, 154, 182–183, 372, 401, 410, 414–415, 420–421,
Keratoconjunctivitis, 625 186–187, 422, 523, 530 473, 532, 625
Keratosis follicularis, 109 Leprosy with type 1 reaction, 153 actinicus, 514–516
Keratosis follicularis spinulosa decalvans Leprous histiocytoma, see Histoid hypertrophicus, 152, 161–162, 162,
(KFSD), 101–102, 135–136, leprosy 163, 497, 498
564–565 Leser-Trélat SK, 134 like syringoma, 112
 Index 645

pemphigoides (LPP), 24–26, 39, 41, Lobomycosis, 164, 216 Lymphoma, 120, 123, 131, 214, 218,
263–264 Lobular capillary hemangioma 423, 434
subtropicus, 514–516 (granuloma pyogenicum) Lymphoma-associated FM follicular
tropicus, 514–516 (LCH), 94, 127–128, 210, 211, mucinosis, 115
Lichen sclerosus 574–576, 575 Lymphomas, 220
(atrophy), 9, 190, 409, 423 Localized cicatricial pemphigoid, 569 Lymphomatoid papulosis, 96
et atrophicus (hypoplastic dystrophy), Localized lepromatous leprosy, 146
164–165, 180, 190–191, 407, Localized leukoplakia, 367
M
410, 423, 430 Localized papular mucinosis, 92
Lichen simplex chronicus (LSC), 76, 143, Localized pigmentation, 35 Macerated plaques, 192
161–162, 496–497, 497, 536 Localized Sweet’s syndrome (SS), 210 with fissures, 173
Lifa disease, 35 Longitudinal grooves, nails, 454–455 Macro acrochordon, 227–228, 228
Linea nigra, 36 Longitudinal melanonychia, 467, 467 Macronychia, 447, 448
Linear and whorled nevoid Longitudinal ridges, 455 Macular amyloidosis (MA), 34–35, 53
hypermelanosis (LWNH), 37 and beads, nails, 455–456 Macules of freckles, 48
Linear Blaschkitis, 92 Loose anagen hair syndrome Madurella grisea, 336
Linear Darier, 175 (LAHS), 562 Madurella mycetomomatis, 336
Linear erythematous scaly plaque, 176 Loose anagen syndrome, 550, 562 Majocchi disease, 68
Linear hypopigmentation, 6 Loss of papilla, 475 Majocchi granuloma, 536
Linear IgA bullous dermatoses (LABD), Loss of sweating, 146 Malaise, 180, 209
537, 605 Lovibond’s angle, 451 Malakoplakia, 434
Linear IgA dermatosis/chronic bullous Low-grade angiosarcoma, 96 of skin, 341
disease of childhood, 261–262 Low-molecular-weight heparin (LMWH) Malassezia, 14
Linear IgA disease, 379–380 injections, 325 Malassezia furfur, 67
Linear lichen planus, 92, 135 Loxosceles, 354 Male genitalia, 407
Linear porokeratosis, 92–94, 94, 536 Lucio-Latapí leprosy, 323 acrodermatitis enteropathica, 430
Linear psoriasis, 92–93 Lucio leprosy, 198 actinomycosis, 435
Linear VEN, 93 Lucio phenomenon (LPh), 323 allergic contact dermatitis, 417
Linear verruca vulgaris, 175 Lucio reaction, 197 angiokeratoma, 418
Linear verrucous epidermal nevus Lucio’s leprosy (LuLp), 350, 350 aphthous ulcer (aphtha, aphthous
(linear VEN), 93, 93 Lucio’s phenomenon, 350, 350 stomatitis, or canker sore),
Linear vesiculobullous, 109 LUMBAR syndrome, 580 431–432
Linear vitiligo, 16 Lunula, 467, 468 balanoposthitis, 409–410
Linoleic acid (LA) deficiency, 478 Lupus erythematosus, 96, bowenoid papulosis, 415
Lipedematous Alopecia, 554 393–394, 401 bullous pemphigoid, 430
Lip lesions tumidus, 530 Buschke-Lowenstein tumor, 425
actinic cheilitis, 401 Lupus hair, 551 candidiasis, 429
angular cheilitis, 401 Lupus miliaris disseminatus faciei chancre, 430–431
cheilitis, 400 (LMDF), 79–81, 107–108, chancroid (soft chancre, ulcus molle),
cheilitis glandularis, 401–402 108, 115 432–433
contact cheilitis, 400 Lupus panniculitis, 341 cicatricial pemphigoid (mucous
exfoliating cheilitis, 401 Lupus pernio, 115, 152 membrane pemphigoid), 430
granulomatous cheilitis, 402 Lupus vulgaris (LV), 79–81, 130, 155, 157, circinate balanitis, 411–412
lip-licking dermatitis, 400 157, 157–158, 158, 160, 207, 219, clinical approach to dermatoses, 408
Lip-licking dermatitis, 50, 400 301, 530, 537 Crohn’s disease, 433
Lipodermatosclerosis, 303, 527 Lymphadenitis, 209 dermatitis artefacta, 434
Lipodystrophy (MDPL) syndrome, 74 Lymphadenopathy, 307 dermatophytosis, 415–417
Lipoid proteinosis, 139, 397 Lymphangioma, 223, 402 diaper dermatitis, 413
Lipomas, 92, 127, 218, 220, 226, 228, 232, 426 Lymphangioma circumscriptum, 248, donovanosis, 433
intraoperative visualization, 229 427, 435 dyspigmentation of median
subcutaneous nodules of, 229 Lymphedema, 435 raphe, 409
Lipomatosis, 221 Lymphocutaneous sporotrichosis, 206 epidermoid cyst, 425–426
Liposarcomas, 228 Lymphocytic infiltrate of Jessner, 219 Erythroplasia of Queyrat, 420–421
Livedoid vasculopathy, 325–326 Lymphocytoma cutis, 151, 218, 585 fixed drug eruption, 410–411
Livedo racemosa, 520 Lymphogranuloma venereum (LGV), genital foreign body (corpus
Livedo reticularis, 68, 325, 519–520 338–340, 429 alienum), 437–439
causes of, 519 with inguinal bubo and sinus., 338 genitourinary tuberculosis, 421–422
646   Index

gonorrhoea, 437 Marginal TA, 550 Miliary calcinosis cutis, 113

hemangioma, 417–418 Marshall-White syndrome, 15 Milium-lik syringoma, 112
herpes genitalis, 428–429 Martorell ulcer, 303 Milker’s nodule, 215–216, 355–356
hypospadias, 437 Mast cells, 169 Milker’s sinus, 335
irritant contact dermatitis, 412–413 Masticatory mucosa, 365 Millium, 419
lentigines, 407–409 Mastocytoma, 169, 224 Minerals of dermatological importance
leprosy (Hansen’s disease), 422 Mastocytosis, 220 copper, 486
lichen nitidus, 414 Maturational pigmentation, 40–42, 43 iron, 486–487
lichen planus, 414–415 Median canaliform dystrophy of Heller selenium, 485–486
lichen sclerosus et atrophicus (MCDH), 455 zinc, 485
(hypoplastic dystrophy), 423 Median raphe cysts, 427–428, 428 Minocycline induced pigmentation, 601
lymphangioma circumscriptum, 427 Median rhomboid glossitis, 392–393, 473 Molluscipox viruses 1–4, 216
lymphedema, 435 Mediterranean spotted fever, 347 Molluscum contagiosum (MC), 84,
lymphoma, 423 MEDNIK syndrome, 540 88–89, 89, 103, 104, 113, 120,
malakoplakia, 434 Mees’ lines, 463 123, 125, 130–131, 138, 227,
median raphe cyst, 427–428 Melanin, 52 230, 413–414, 419, 427, 577
melanocytic nevus, 407 Melanoacanthoma, 134 like umbilicated papules, 230
molluscum contagiosum, 413–414 Melanoacanthoma seborrheic on penis, 413
mucoid penile cyst, 427 keratosis, 169 virus (MCV), 130
paraphimosis, 436–437 α-Melanocyte-stimulating hormone Mongolian spots, 19–20, 22
pearly penile papule, 419 (α-MSH), 39 Monilethrix (beaded hairs), 563, 564
pemphigus vulgaris, 430 Melanocytic nevus, 8, 19, 23, 125, 170, Monkey facies, 476
penile fracture, 436 219, 223–224, 228, 407, 583, 588 Monomorphic papules, 80
penile thrombophlebitis, 426–427 Melanofibroma, see Blue nevus Monosodium urate (MSU) crystals, 221
Peyronie’s disease, 427 Melanogenesis, 5 Morbilliform (exanthematous) drug
phimosis, 436 Melanoma, 70, 85, 96, 124–125, 172, 217, reaction, 597–599
plasma cell balanitis (Zoon’s 219, 224, 388–389, 588 Morphea, 15, 20, 164–165, 165, 172, 180
balanitis), 410 Melanoma-associated leukoderma, 9 Mosaic skin, 476
porokeratosis, 423–424 Melanosomes, 5 Moth-eaten alopecia, 547, 547
priapism, 435–436 Melanotic macule, 387 Mottled pigmentation, 58, 69, 71
pseudoepitheliomatous, keratotic, and Melasma, 19, 29, 29–31, 36, 39, 40–41 acquired brachial cutaneous
micaceous balanitis, 424–425 Melioidosis, 307 dyschromatosis, 75
psoriasis, 420 Melkersson-Rosenthal syndrome, amyloidosis cutis dyschromica, 72–74
pyoderma gangrenosum, 433–434 374, 402 causes of, 59
scabies, 415 Meltzer’s triad, 325 chronic arsenicism, 70–71
scrotal calcinosis, 426 Meningeal signs, 122 disorders causing, 60–61
sebaceous hyperplasia, 418–419 Meningococcemia, 206 dyschromatosis symmetrica
seborrheic dermatitis, 412 Merkel cell carcinoma, 218, 299 hereditaria (DSH), 69
skin tags, 420 Metastasis, 205 dyschromatosis universalis
squamous cell carcinoma, 425 Metastatic cancer, 299 Hereditaria (DUH), 69–70
Stevens-Johnson syndrome and toxic Metastatic fistula, 341–342 epidermolysis bullosa simplex with
epidermal necrolysis, 430 Metastatic lesions, 308 mottled pigmentation, 68
verrucous carcinoma, 425 Metastatic ulcer, 307 mycosis fungoides (MF), 71–72
vitiligo, 407 Michelin tire baby syndrome, 124 onchocerciasis, 75
Male pattern hair loss, 554 Microcystic lymphatic malformation, photoaging, 74
Malignancy, 127 584–585 porphyria cutanea tarda, 68–69
Malignancy, risk of, 64 Micronychia, 447, 447 Progeria, 74
Malignant lymphoma, 198 Microscopic polyangiitis, 322 regional distribution of disorders, 63
Malignant lymphoproliferative Mid-borderline (BB) leprosy, 182, 529 Rothmund-Thomson syndrome, 70
process, 115 Migratory glossitis, 473 treponematoses, 75
Malignant melanoma, 204–205, 407, 583 Migratory myiasis, 339 vagabond’s leukomelanoderma, 74–75
Fitzpatrick sign, 204 Mild (and chronic) ICD, 412, 412 xeroderma pigmentosum (XP), 70
nodule of, 225 Milia, 89, 89, 92, 103, 112–113, 113, 131 Mucinous degeneration of follicles, 115
Malignant T cells, 152 Milia-en-plaque, 112, 174 Mucinous LE, 92
Mammary duct fistula, 335 Milia-like idiopathic calcinosis cutis Mucocele (mucous cyst or ranula),
Mandibular hypoplasia deafness, 74 (MICC), 88, 88 397–398
Marfan syndrome, 112 Miliaria crystallina, 254 Mucocutaneous candidiasis, 374
 Index 647

Mucocutaneous lesions, 123 Nails, 445–446, 445–446 nail plate thickness, abnormalities of
Mucoid penile cyst, 427 ABCDEF rule, 467 nail-plate thickening, 448–450
Mucormycosis (zygomycosis), 343–344 abnormalities, 61 nail-plate thinning, 450
Mucosa, 365 apparatus, 445 peeling of, 460
Mucosal candidiasis, 421 bed, 445 pigmentation, 467
Mucosal damage, 373 bed glomus tumor, 471 pitting, 459
Mucosal inflammation, 599 brittle, 459 plate, 445, 446
Mucosal lesions, 127, 194 clubbing, 452, 452, 453 plate splitting, 471
Mucous membrane cuticle, 445 signs, 446–447
erosions, 604 folds, 445 subungal changes, 470
pemphigoid, 370, 423, 430 fragility, 109 thickening, 449
Muehrck’s nails, 464 hyponychium, 445 Naked granuloma, 115
Multicentric reticulohistiocytosis, 96 increased nail plate fragility, brittle Nappes claires, 180
Multifocal cutaneous sporotrichosis, 159 nails, 459–460 Necrobiosis lipoidica (NL), 221, 229,
Multifocal systemic disease, 196 longitudinal grooves, 454–455 303, 527
Multiple apocrine hidrocystomas, 114 longitudinal overcurvature of the diabeticorum, 172, 187
Multiple endocrine neoplasia type 1 nail plate diabeticorum/necrobiosis
(MEN-1), 13, 106 habit tic deformity, 458 lipoidica, 303
Multiple eruptive milia, 112 longitudinal ridges and beads, Necrobiotic xanthogranuloma, 187, 220,
Multiple hyperpigmented papules, 195 455–456 220–221
Multiple lentigines syndrome, 28 nail beaking (parrot beak Necrolytic migratory erythema (NME),
Multiple longitudinal grooves, 455 nails), 454 482, 538
Multiple papillomas, 396 pitting (pits, erosions, onychia Necrotizing fasciitis (NF), 304–306,
Multiple skin-colored micropapules, 624 punctata), 458–459 305, 344
Multiple tense cystic lesions on the trachyonychia, 456–457 with eschar and ulcer, 345
scrotum, 426 transverse grooving (TG), 457–458 Neisseriagonorrhoeae, 437
Munchausen by proxy, 502 matrix, 445 Neisseria meningitidis, 347
Munchausen syndrome, 501–502 melanocytes, 467 Neonatal acne/infantile acne, 280
Musculoskeletal abnormalities in nail folds changes Neonatal cephalic pustulosis, 102
hypomelanosis of ITO, 16, 16 Hutchinson’s sign, 469 Neonatal hemangiomatosis, 580
Mycetoma, 226–227, 230, 335–338 ingrown nails/onychocryptosis, 469 Neonatal lupus erythematosus (NLE),
nodule and discharging sinuses on paronychia, 467–468 525, 614, 614–615
heel, 226 Pseudo-Hutchinson’s sign, 469 Neonatal occipital alopecia (NOA),
Mycobacterial infections, 219, 296, 434 nail plate adhesions, abnormalities of 554–555, 555
Mycobacteria other than tuberculosis onycholysis, 460–461 Neotestidina, 336
(MOTT), 214 onychomadesis, 461 Nephrotic syndrome, 585
Mycobacterium abscessus, 214 onychoschizia (lamellar nail Nerve palsies, 122
Mycobacterium chelonae, 214 dystrophy, peeling of Nerve trunks on face, 120
Mycobacterium fortuitum complex, 214 nails), 460 Nestor-Guillermo progeria syndrome, 74
Mycobacterium leprae, 119, 143, 182 pterygium, 461–462 Nestor-Guillermo syndrome, 74
Mycobacterium leprae histiocytoma, see nail plate color, abnormalities of Neurilemmoma, 208, 220
Histoid leprosy chromonychia, 462–467 Neuritis, 209
Mycobacterium marinum, 214 longitudinal melanonychia, 467 Neurocutaneous melanosis (NCM), 24
Mycobacterium ulcerans, 214 nail plate curvature, abnormalities of Neurodermatitis, 174
Mycoplasma pneumoniae, 378 clubbing (Hippocrates fingers, Neurodermatitis circumscripta, see
Mycosis fungoides (MF), 71–72, 174, acropachy), 451–452 Lichen simplex chronicus
186–188, 188, 195 koilonychia (spoon nails), 451 Neurofibromas, 92, 131, 223, 227
Myelopathy, 580 platonychia, 451 Neurofibromatosis, 221
Myiasis, 230, 307 transverse overcurvature of nail, Neurofibromatosis type 1 (NF1), 22,
452–453 65–66, 228
nail plate size, abnormalities of Neurological abnormalities in
N
anonychia/micronychia, 446–447 hypomelanosis of ITO, 16, 16
Naegeli–Franceschetti–Jadassohn (NFJ) brachyonychia/racquet nail, 447 Neurological manifestations in vitamin
syndrome, 60–61, 64 macronychia, 447 B12 deficiency, 478
Nail beaking (parrot beak nails), 454 onychoatrophy, 447 Neuromas, 205, 208
Nail-plate thickening, 448–450, 450 onychodystrophy/dystrophic Neuropsychiatric abnormalities, 109
Nail-plate thinning, 450 nail, 448 Neuropsychiatric manifestations, 482
648   Index

Neurotic excoriations, 434, 500 blue nevus, 204–205 osteoma cutis, 230
Neutrophilic dermatosis of dorsal hands blue rubber bleb nevus syndrome, 202 pilomatricoma, 205
(NDDH), 210 cavernous hemangioma, 202 prurigo nodularis, 211–212
Neutrophilic eccrine hidradenitis, 606 chondrodermatitis nodularis pyogenic granuloma (PG), 210–211
Neutrophilic figurate erythema helicis, 207 rheumatoid nodules, 228
(NFE), 538 chondroid syringoma, 221 sarcoidosis, 231–232
Nevi, 427 clear cell acanthoma, 217 schwannoma, 220
Nevoid acanthosis nigricans, 170 clinical approach to, 203 scrofuloderma, 213–214
Nevoid basal cell carcinoma clinical characteristics, 202 sebaceous hyperplasia, 228
syndrome, 135 clinical clues to diagnose, 204 spitz nevi, 219
Nevoid hyperkeratosis of nipple, 174 cryptococcosis, 227 sporotrichosis (rose gardener’s
Nevoid psoriasis, 135 cutaneous calcinosis (calcinosis disease), 206–207
Nevus anemicus, 7–8, 588 cutis), 230 steatocystoma multiplex, 221, 221
with hypopigmented patch, 8 cutaneous leishmaniasis, 219–220 subcutaneous granuloma annulare,
Nevus comedonicus, 94, 94 cutaneous polyarteritis nodosa, 215 228–229
Nevus depigmentosus (nevus cylindroma, 218 Sweet’s syndrome (SS), 210
achromicus), 5–7, 7, 8, 589 dermatofibroma, 204 tertiary syphilis (gumma), 222–223
Nevus fusco-ceruleus ophthalmo- dermatofibrosarcoma protuberans, tophus (podagra), 221–222
maxillaris, 19 225–226 Wells syndrome (Eosinophilic
Nevus lipomatosis superficialis, 175, dilated pore of Winer, 222 cellulitis), 213
175–176 eccrine poroma, 217 xanthoma (tendinous, tuberous,
Nevus lipomatosus, 176, 223 erythema nodosum, 208–209 eruptive, normolipemic), 221
classical type, Hoffman and Zurhelle erythema nodosum leprosum (ENL), Nodules of prurigo nodularis, 498
type, 223 209–210 Nodules of scrotal calcinosis, 427
cutaneous superficialis, 155 furuncle (boils), 207 Nodulocystic acne, 206
solitary form, 223 furuncular myiasis, 230–231 Nodulocystic lesions, 104
Nevus of Hori, 31 giant molluscum contagiosum, 216 Noduloulcerative syphilis, 340
Nevus of Ito, 19, 24 glomus tumors, 205 Noma/cancrum oris, 294
Nevus of OTA (nevus fusco caeruleus granuloma faciale (Lever type), Noma caused by Fusobacterium, 347
ophthalmo-maxillaris), 19, 218–219 Non-cicatricial alopecia of scalp, 60
20–21, 24, 46, 46–47, 389 hidradenitis suppurativa, 207 Non-genital warts, 219
Nevus sebaceous, 87, 94, 131–132, 153, histoid leprosy, 229–230 Non-homogeneous lesions, 367
174–176, 223, 556 histoplasmosis, 227 Non-homogenous leukoplakia, 367
Nevus spilus (speckled lentiginous juvenile xanthogranuloma (JXG), 220 Non-inflammatory lesions, 100
nevus), 23–24, 24 kaposi sarcoma, 217–218 Noninvoluting congenital hemangioma
Nevus unius lateralis, 175 keloid, 216 (NICH), 178
Nicolau-Balus syndrome, 112 leiomyoma, 207–208 Nonmelanoma skin cancer, 297
Nicolau syndrome, 303 leukaemia cutis, 210 Non-metastatic fistula, 342
Nicotinic stomatitis, 375 lipoma, 228 Non-scarring alopecia, 548
Nijmegen breakage syndrome (NBS), 65 macro acrochordon, 227–228 Non-steroidal antiinflammatory drugs
Nikolsky’s sign, 379 mastocytoma, 224 (NSAIDs), 594, 604
Nipple melanocytic nevi, 223–224 Nontuberculous mycobacteria
eczema, 174 Merkel cell tumor, 218 (NTM), 214
nevoid hyperkeratosis of, 174 Milker’s nodule, 215–216 Non-vasculitic vessel occlusion, 323
Nocardiosis, 301, 341 mycetoma, 226–227 Noonan syndrome, 124
Nodular amyloidosis, 85, 221, 231 necrobiotic xanthogranuloma, Norwegian scabies, 194
Nodular basal cell carcinoma, 124 220–221 palm involvement, 195
Nodular BCC, 125 neurofibroma, 227 scaly plaque in, 194
Nodular candidiasis, 373–374 nevus lipomatosus, 223 Notalgia paresthetica (NP), 34–35, 504
Nodular melanoma, 225 nodular amyloidosis, 231 NSAIDs, 39
Nodular scabies, 212–213 nodular melanoma, 225 Nummular dermatitis, 417
Nodular vasculitis (erythema induratum nodular scabies, 212–213 Nummular eczema, 77, 193
of Bazin), 214–215 nodular vasculitis (erythema Nutrients required for humans, 473
Nodules, localized, 182, 202 induratum of Bazin), 214–215 Nutritional deficiency dermatosis, 430
acroangiodermatitis, 213 nodulocystic acne, 206 Nutritional deficiency disorders, 473
amelanotic melanoma, 217 orf, 215 angular cheilitis (AC), 475
atypical mycobacteria infection, 214 osler nodes, 205–206 atrophic glossitis (AG), 473
 Index 649

biotin deficiency, 482 Open comedones, 115, 117 herpes simplex virus infection,
carotenemia and carotenoderma, 485 Ophiasis type AA, 541 375–376
clinical approach to dermatoses Ophthalmopathy, 116 herpes zoster, 376
seen in, 474 Oral candidiasis, 373, 373 linear IgA disease, 380
clues for diagnosis, 474 Oral cavity, 365 pemphigoid gestationis, 380
essential fatty acids (EFA) Oral contraceptives, 210, 606 pemphigus vulgaris, 379
deficiency, 478 Oral leukokeratosis, 375 Stevens-Johnson syndrome,
factors responsible, 475 Oral manifestations, 376 378–379
folate deficiency, 478–479 Oral mucosa, 30, 58, 82, 365 toxic epidermal necrolysis, 379
hemochromatosis, 480 lip lesions ulcerative lesions without preceding
hypervitaminosis A, 478 actinic cheilitis, 401 with vesicobullous lesions
hypozincemia (Zinc deficiency), angular cheilitis, 401 Behçet’s disease, 382–383
480–482 cheilitis, 400 eosinophilic ulcer, 384
oral-ocular-genital syndrome, cheilitis glandularis, 401–402 oral ulcer, rarer causes, 384
475–476 contact cheilitis, 400 oral ulcer caused by fungal
pellagra (vitamin B3 or Niacin exfoliating cheilitis, 401 infection, 384
deficiency), 482 granulomatous cheilitis, 402 recurrent aphthous stomatitis,
protein energy malnutrition lip-licking dermatitis, 400 381–382
(PEM), 476 pigmented lesions, 387 squamous cell carcinoma, 384–387
pyridoxine (vitamin B6) Addison’s disease, 389 syphilis, 383–384
deficiency, 482 amalgam tattoo, 388 traumatic ulcers, 381
riboflavin (vitamin B2) deficiency, drug-induced pigmentation, 388 verrucous lesions, lumps and
475–476 freckles and lentigines, 388 swellings
scurvy, 484–485 heavy-metal deposition, 388 abscesses, 397
vitamin A deficiency, 476–478 melanoma, 389 angioedema, 397
vitamin B12 deficiency, 478 melanotic macule, 387 fibroma, 398–399
vitamin K deficiency, 483–484 nevus of ota, 389 gingival hyperplasia, 399–400
Peutz-Jeghers syndrome, 390 mucocele (mucous cyst or ranula),
pigmented nevi, 389 397–398
O
smoker’s melanosis, 388 pyogenic granuloma, 399
Obsessive-compulsive disorder (OCD), red lesions, acquired disorders verrucous lesions, papilloma due to
497–498, 500 anemia, 394 chronic irritation, 395
Ocular abnormalities, 70 denture-related stomatitis, 393 acanthosis nigricans, 397
Ocular rosacea, 105 erythroplakia, 393 condyloma acuminatum, 395–396
Oculomucodermal melanocytosis, 19 Infectious mononucleosis, 394 Darier disease, 397
Ohio Valley disease, 123 lupus erythematosus, 393–394 focal epithelial hyperplasia
Onchocerca volvulus, 74 median rhomboid glossitis, (Heck’s disease), 396
Onchocerciasis, 75 392–393 verruca vulgaris, 395
Onfluent and reticulated papillomatosis plasma cell gingivitis, 393 verruciform xanthoma, 396
(CRP), 67–68 radiation mucositis, 391–392 verrucous carcinoma, 396–397
Onychoatrophy, 447, 447 Reiter’s disease, 394 white lesions, 365–366
Onychochauxis, 449, 449 thermal burn, 391 candidiasis, 373–374
Onychocryptosis, 469 thrombocytopenic purpura, 394 chemical burn, 375
Onychodystrophy/dystrophic nail, 448 traumatic erythema, 390–391 dyskeratosis congenita, 375
Onychodystrophy of toenail, 448 red lesions, hereditary disorders, 390 fissured tongue, 374
Onychogryphosis, 449 ulcerative lesions preceding with geographic tongue, 370–372
Onycholysis, 460–461, 461 vesico-bullous lesion hairy leukoplakia, 368
Onychomadesis, 461, 461 bullous pemphigoid, 379–380 hairy tongue, 372–373
Onychomatricoma, 450 cicatracial pemphigoid, 380 leukoedema, 368
Onychomycosis, 466 dermatitis herpetiformis, 380 leukoplakia, 366–368
Onychophagia, 503 epidermolysis bullosa, 381 lichen planus, 368–370
Onychorrhexis, 455 epidermolysis bullosa nicotinic stomatitis, 375
Onychoschizia (lamellar nail dystrophy, acquisita, 381 pachyonychia congenita, 375
peeling of nails), 460, 460 erythema multiforme, 378 white sponge nevus, 375
Onychotemnomania, 503 hand, foot, and mouth disease, Oral mucosal lesions, 365
Onychotillomania, 503 377–378 Oral-ocular-genital syndrome, 475–476
Opaque trachyonychia, 456 herpangina, 377 Oral squamous cell carcinoma, 341
650   Index

Oral ulceration, causes of, 384, 385 Papules, 182 milia, 89

Oral ulcer caused by fungal infection, 384 benign cephalic histiocytosis, 88 Milia-like idiopathic calcinosis
Orange palm in carotenemia, 485 bowenoid papulosis, 84 cutis, 88
Orf, 215–216, 251–252, 286–287 forehead in steatocystoma molluscum contagiosum, 89
Orofacial granulomatosis, 374 multiplex, 91 morphologic characteristics, 77
Oropharyngeal ulcers, 227 of granuloma annulare, 523 multicentric reticulohistiocytosis, 96
Osler nodes, 205–206 of lichen nitidus, 414 neurofibromas, 92
Ossified hair follicle, 230 molluscum contagiosum, 89, 131 nevus comedonicus, 94
Osteochondroma, 230 neurofibroma, 92 nevus sebaceous, 87
Osteoma, 124 of neurofibromatosis type 1, 131 papular sarcoidosis, 79
cutis, 230, 231 Papules, localized, 76 papulopustular rosacea, 81
Oxicam, 604 acne keloidalis, 84 pearly penile papules, 90
Oyster-like onychogryphosis, 449 acne vulgaris, 81 piezogenic pedal papules, 91
acquired melanocytic nevus, 85 prurigo nodularis, 84–85
acrokeratosis verruciformis, 86 psoriasis, 76
P
adenoma sebaceum (facial reactive perforating collagenosis, 83
Pachyonychia, 449, 449 angiofibroma), 84 seborrheic keratosis, 86–87
congenita, 61, 375 angiokeratomas, 95 skin tags, 90
congenita type II syndrome, 112 angiolymphoid hyperplasia with steatocystoma multiplex, 91–92
Paecilomyces, 355 eosinophilia (ALHE), 96 subacute cutaneous lupus
Paederus dermatitis, 248–249 bacillary angiomatosis, 95 erythematosus, 77
Pagetoid reticulosis, 153, 187 basal cell carcinoma, 76 targetoid hemosiderotic
Paget’s disease, 173–174 benign cephalic histiocytosis, 88 hemangioma, 96
on nipple, 173 bowenoid papulosis, 84 tufted angioma, 96
in perianal area, 174 cherry angioma, 96 verruca vulgaris, 86
Palatal papules, 191 darier disease, 81–82 Papulonecrotic lesions, 322
Palmar hyperkeratotic papules, 72 defined, 76 Papulonecrotic tuberculid, 422
Palmar-plantar erythrodysesthesia, 606 dermatosis papulosa nigra (DPN), 82 Papulopustular rosacea, 81, 105
Palmar xanthomas, 155 discoid lupus erythematosus (DLE), Papulo-squamous plaques, 184
Palmoplantar psoriasis, 143, 153 77–78 Papulosquamous syphilids, 193–194
Palmoplantar pustulosis (PPP), 287 eruptive vellus hair cyst, 86 Paracoccidioidomycosis, 384
Palpable purpura, 316, 322, 322 fibrous papule of nose, 76 Parakeratosis pustulosa, 284–285
ANCA-associated vasculitis, 322 Gianotti-crosti syndrome, 97 Paraneoplastic pemphigus (PNP),
cutaneous small vessel vasculitis gottron papules, 96 257–258, 428, 430
(CSVV), 322 granular parakeratosis, 81 Paraphimosis, 436, 436, 436–437
gloves-and-socks syndrome, 323 granuloma annulare, 79 Parapoxviru, 215
Henoch-Schönlein purpura, 322 Inflammatory linear verrucous Parapsoriasis, 169, 193–194
urticarial vasculitis, 322 epidermal nevus (ILVEN), 93 Parasite infection (leishmaniasis), 434
Pancreatic fat necrosis, 341 Jessner’s lymphocytic infiltration, 81 Parkes Weber syndrome, 574, 589
Pancreatic panniculitis, 341 juvenile xanthogranuloma, 87–88 Paronychia, 467–468
Panniculitis, 341 lichen amyloidosis, 83 Parrot beak nails, 454
Papillary hyperplasia of palate, 397 lichen nitidus, 90 Partial unilateral lentiginosis, 24
Papillary intralymphatic lichen planus, 76 Patch stage, mycosis fungoides (MF), 72
angioendothelioma, 587 lichen scrofulosorum, 90 Patchy hyperpigmentation, 479
Papillomas, 395–397 lichen striatus, 94 Patchy/localized AA, 541
on labial mucosa, 395 linear lichen planus, 92 Pathomechanisms, 19
Papillomatosis, 67, 109 linear porokeratosis, 94 Pearly penile papules, 90, 419, 419
of Gougerot, 67 linear psoriasis, 92 Pediatric SLE, 628
Papular angiokeratoma, 583 linear verrucous epidermal nevus Pediculus humanus corporis
Papular histiocytosis of head, 88 (linear VEN), 93 infestation, 74
Papular lesion of lupus vulgaris, 80 lobular capillary hemangioma Peeling of nails, 460
Papular mucinosis, 90 (granuloma pyogenicum), 94 Pellagra (vitamin B3 or Niacin
Papular sarcoidosis, 79, 79, 81 localized papular mucinosis, 92 deficiency), 482, 483, 622,
Papular scar, 114, 114, 114–115 lupus miliaris disseminatus faciei 627–628
Papular urticaria, 496 (LMDF), 80–81 PELVIS syndrome, 580
insect bite hypersensitivity, 169 lupus vulgaris, 79 Pemphigoid, 379
with papulovesicles, 98, 98 lymphomatoid papulosis, 96 gestationis, 259–261, 380
 Index 651

Pemphigus, 379–380 primary systemic amyloidosis, 318 Rothmund-Thomson syndrome

Pemphigus foliaceus, 77, 167, 269 scurvy, 317 (poikiloderma congenitale),
Pemphigus vegetans, 81, 173, 192, 397 thrombotic thrombocytopenic 618–619
Pemphigus vulgaris, 254–257, 378–379, purpura (TTP), 317 solar urticaria, 625
428, 430 valsalva-associated petechiae, 318 trichothiodystrophy (TTD), 621–622
Penicillamine, 112 vitamin K deficiency bleeding xeroderma pigmentosum (XP),
Penicillin, 606 (VKDB) disorder, 320 619–621
Penicillium, 355 Peutz-Jeghers syndrome, 28, 390 Photosensitizers, 39
Penile; see also Male genitalia Peyronie’s disease, 427, 436 Phototoxic and photoallergic
dorsal vein thrombosis, 427, 436 Phaeohyphomycosis, 226, 355 reaction., 597
foreign body, 438 Phaeomycotic cyst, 355 Phototoxic reaction, 482, 622,
fracture, 427, 436 Phakomatosis 626–627, 628
horn, 424 cesioflammea, 572 Phycomycosis, 384
implant, 436 pigmentovascularis, 28, 574, 575 Phylloid hypomelanosis, 5, 13
lichen planus, 414 Phenobarbital, 604 Phymatous rosacea, 105
papules, 90 Phenothiazines, 594 Physiological livedo reticularis, 520
psoriasis, 424 Phenylketonuria (PKU), 622 Physiologic anemic macules (Bier
swelling, 435 Phenytoin, 39, 604 spots), 15
thrombophlebitis, 426–427 Phimosis, 436–437 Phytophotodermatitis, 39
warts, 439 Phimosis in lichen sclerosus, 436 PIBIDS syndrome, 621
Penttinen syndrome, 74 Photoaging, 74 Piebaldism, 8, 8
Perifollicular petechiae in scurvy, Photoallergic reactions, 594, 623 Piezogenic pedal papules, 91, 91
317, 484 Photolichenoid drug eruption, 185 Pigmentary changes, 5
Perifolliculitis capitis abscedens et Photo-onycholysis, 625 Pigmentary demarcation lines (PDLs),
suffodiens, 568 Photo recall phenomenon, 609 19, 47–48
Perino, 302 Photosensitivity, 185 Pigmentary mosaicism, 37
Perioral dermatitis, 115, 281 dermatosis, 184 Pigmentation, 29–30
Periorbital hyperpigmentation, 31 disorders, 68, 149 Pigmentatio reticularis faciei et colli, 66
Periorbital melanosis, 42–44, 43 eczema, 185 Pigmented basal cell carcinoma, 124–125
Periorbital purpura, 319 to exogenous chemicals, 626–627 Pigmented contact dermatitis, see Riehl’s
Periorificial dermatitis (perioral skin lesions, 180 melanosis
dermatitis), 110–111, 111 Photosensitivity in children, 614 Pigmented dermatofibroma, 205
Periorificial tuberculosis, 174 actinic prurigo (Hutchinson’s Pigmented lesions, 387
Peripheral neuropathy, 116 summer prurigo), 625 Addison’s disease, 389
Peripheral papules, 186 approach to, 615 amalgam tattoo, 388
Perirectal abscess, 341 ataxia telangiectasia, 622 drug-induced pigmentation, 388
Periumbilical thumbprint parasitic Bloom syndrome (congenital freckles and lentigines, 388
purpura (PTPP), 323–324 telangiectatic erythema), 619 heavy-metal deposition, 388
Petechiae, 316 childhood systemic lupus melanoma, 389
in idiopathic thrombocytopenic erythematosus (CSLE), melanotic macule, 387
purpura, 317 625–626 of mucosa, 387
Petechiae and ecchymoses Cockayne syndrome (CS), 621 nevus of ota, 389
actinic purpura, 317 hartnup disease, 622 Peutz-Jeghers syndrome, 390
battered baby syndrome, 320 hydroa vacciniforme (HV), 624–625 pigmented nevi, 389
coagulation-factor deficiency, 318 juvenile dermatomyositis (JDM), 626 smoker’s melanosis, 388
corticosteroid purpura, 317 juvenile spring eruption (JSE), Pigmented macules
hemolytic-uremic syndrome 623–624 dorsum of feet, 65
(HUS), 317 kindler syndrome, 622 face and neck, 65
heparin-induced thrombocytopenia, neonatal lupus erythematosus (NLE), neck, 65
315–317 614–615 Pigmented nevi, 388–389
hypergammaglobulinemic purpura of pellagra, 627–628 Pigmented papules of bowenoid
Waldenstrom, 318 phenylketonuria (PKU), 622 papulosis, 415
idiopathic thrombocytopenic photosensitivity to exogenous Pigmented peribuccal erythrosis of
purpura, 315 chemicals, 626–627 Brocq, 104
leukemia/bone marrow failure, 315 polymorphous light eruption (PLE), Pigmented purpuric dermatoses, 213, 318
pigmented purpuric 622–623 Pigmented xerodermoid, 71
dermatoses, 318 porphyria, 615–618 Pilar cyst, 221
652   Index

Pili torti, 563 erythema nodosum leprosum (ENL), erythematous

Piloleiomyoma, 207 196–198 alopecia mucinosa, 152–153
Pilomatricoma, 125–126, 126, 127, 205, figurate erythemas, 193 cellulitis, 155–156
220, 230 generalized morphea, 190 cutaneous lymphoid hyperplasia/
Pilomatrixoma, 125 granuloma annulare, 186 lymphocytoma cutis, 152
Pilonidal cyst, 427 granuloma multiforme, 187 discoid lupus erythematosus
Pilonidal sinus, 341 incontinentia pigmenti (verrucous (DLE), 148–149
Pilosebaceous follicle, 108 stage), 201 erysipelas, 152
Pincer nail, 453 Kaposi sarcoma (KS), 196 erythema elevatum diutinum, 152
Pinhead-sized papules, 624 leprosy, 182–183 granuloma annulare, 154
Pinpoint papules in lichen nitidus, 90 lichen sclerosus et atrophicus (LSA), granuloma multiforme, 154
Pinta, 75 190–191 Jessner’s lymphocytic infiltrate, 151
Pitting (pits, erosions, onychia punctata), mycosis fungoides (plaque stage), leprosy, 143–148
458–459 187–188 pagetoid reticulosis, 153
Pityriasis alba (scales), 5, 6, 9, 14 necrobiotic xanthogranuloma, 187 polymorphic light eruption (PLE),
Pityriasis amiantacea, 143 Norwegian scabies, 194 149–151
Pityriasis folliculorum-like papulosquamous syphilids, 193–194 psoriasis, 143
(spinulate demodicosis) parapsoriasis, 193 sarcoidosis, 151–152
demodicosis, 107 pityriasis rubra pilaris, 180–182 tumid lupus erythematosus, 155
Pityriasis lichenoides, 193 progressive symmetric xanthoma, 154–155
Pityriasis lichenoides et varioliformis erythrokeratoderma (PSEK), hyperkeratotic
acuta (PLEVA), 96, 496 188–189 benign lichenoid keratosis (lichen
Pityriasis rosea, 167, 169, 193–194, 417, pruritic urticarial papules and planus-like keratosis), 163
523–525 plaques of pregnancy (PUPPP), chromomycosis
Pityriasis rotunda, 168–169 195–196 (chromoblastomycosis), 159
Pityriasis rubra pilaris (PRP), rupioid psoriasis, 180 cutaneous leishmaniasis (CL),
180–182, 189 sarcoidosis, 186 159–160
Pityriasis versicolor (Tinea versicolor), seborrheic keratosis, 195 keloids/hypertrophic scar, 163–164
5, 13, 14, 15, 33, 68, 165, 200, subacute cutaneous lupus lichen planus hypertrophicus,
525, 589 erythematosus, 184–185 161–162
Pityrosporum folliculitis, 14, 283 Sulzberger-Garbe disease, 194–195 lichen simplex chronicus, 161
Plagues, 348, 586 sweet syndrome, 185–186 lupus vulgaris, 157–158
Plane warts, 86, 88, 90 systematized verrucous epidermal sporotrichosis, 159
Plaque-like syringoma, 112 nevus, 200–201 tuberculosis verrucosa cutis,
Plaques, 231 tinea corporis, 192–193 156–157
in erythema multiforme, 201 urticaria, 189 verruca vulgaris, 162
parapsoriasis, 180 urticarial vasculitis, 189–190 plaques without scaling
psoriasis, 421 urticaria pigmentosa, 196 mastocytoma, 169
of secondary syphilis, 194 Plaques, localized, 110, 143, 153 melanocytic nevus, 170
stage, mycosis fungoides (MF), 72 atrophic pruritic urticarial papules and
ulcerated, 297 lichen sclerosus et atrophicus plaques of pregnancy, 169
Plaques, generalized, 180 (LSA), 164–165 seborrheic keratosis, 169
bullous pemphigoid (urticarial morphea, 164 shagreen patch, 170–171
phase), 190 clinical approach, 144 scaly
chronic cutaneous lupus congenital actinic keratosis (AK), 167–168
erythematosus, 183 connective tissue nevus, 176 herald patch of pityriasis rosea,
chronic plaque psoriasis, 180 hemangioma, 177–178 167
clinical approach to diseases Inf lammatory linear pityriasis rotunda, 168–169
presenting, 181 verrucous epidermal nevus seborrheic dermatitis (SD),
Darier disease, 191–192 (ILVEN), 176 165–167
diffuse acanthosis nigricans, 198–199 milia-en-plaque, 174 ulcerated
discoid lupus erythematosus, 183 nevus lipomatosis superficialis, basal cell carcinoma, 171–172
disseminated cutaneous 175–176 Bowen’s disease, 172–173
leishmaniasis, 198 nevus sebaceous, 174 necrobiosis lipoidica
epidermodysplasia verruciformis verrucous epidermal nevus, diabeticorum, 172
(EV), 199–200 174–175 Paget’s disease, 173–174
erythema multiforme (EM), 201 verrucous hemangioma, 176–177 pemphigus vegetans, 173
 Index 653

Plaques without scaling Post-kala azar dermal leishmaniasis Pseudofolliculitis barbae (PFB) (shaving
mastocytoma, 169 (PKDL), 12, 12, 12, 15, 115–116, bumps), 118, 118
melanocytic nevus, 170 116, 120, 130–131, 160, 198, Pseudo glucagonoma syndrome, 482
pruritic urticarial papules and 198, 230 Pseudo-Hutchinson’s sign, 469
plaques of pregnancy, 169 Post-pemphigus acanthoma, 195 in Laugier-Hunziker-Baran
seborrheic keratosis, 169 Post-transfusion purpura, 317 syndrome, 469
shagreen patch, 170–171 Potassium iodide, 606 Pseudo-Kaposi sarcoma, 213
Plaque-type polymorphous light Preadolescent acne, 81 Pseudo-Kaposi’s sarcoma, 196
eruption, 530 Premalignant fibroepithelial tumor Pseudo-knuckle pads, 497
Plasma cell balanitis (Zoon’s balanitis), (Pinkus tumor), 228 Pseudo-Koebnerization, 131, 162
143, 410, 411 Pressure alopecia, 554–555 Pseudolymphoma, 81, 210
Plasma cell gingivitis, 393 Pressure ulcer Pseudomembranous candidiasis, 373
Platanus orientalis, 300 covered with eschar, 352 Pseudomonas, 338
Platonychia, 451 decubitus ulcer, 352 Pseudomonas aeruginosa, 306, 345, 346
Pleomorphic adenoma, 96, 97 Pretibial epidermolysis bullosa Pseudomonas infection, 294
Plicated nail, 453 pruriginosa, 162 Pseudoparasitica dysaesthesia, see
POFUT1 gene, 66 Priapism, 435–436 Delusional parasitosis
POGLUT1 gene, 66 PRIDE complex, 608 Pseudopelade of Brocq, 569
Poikiloderma, 64 Primary herpes gingivostomatitis, 377 Pseudoporphyria, 69, 271
atrophicans vasculare, 68 Primary herpes simplex, 379 Pseudopyogenic granuloma, 585
of civatte, 31–32, 50 Primary milia, 112 Pseudo-scleroderma, 190
face and neck, 64 Primary or metastatic tumors, 304 Pseudo wart, 413
Poikiloderma of Civatte, 104 Primary rectal adenocarcinoma, 425 Pseudoxanthoma elasticum, 176
Poikiloderm congenitale, 618 Primary systemic amyloidosis, 318 Psoriasiform drug reaction, 602
POLA1, 58 Professional patient syndrome, see Psoriasiform papules, 79
Polyarteritis nodosa, 323 Munchausen syndrome Psoriasiform syphilid, 180
Polycyclic lesions, 514 Progeria, 74 Psoriasis, 76–78, 96, 122, 143, 148,
Polymorphous light eruption (PLE), Hutchinson-Gilford syndrome, 74 151–153, 157, 167, 172–174, 180,
149–151, 150, 151–152, 622–623 Nestor-Guillermo syndrome, 74 183, 185–186, 193–194, 217,
facial lesion of, 151 Werner syndrome, 74 410, 412–413, 420, 422, 496,
Polypoid lesions, 134 Progressive cribriform, 28 536, 540
Polysurgical addiction, see Munchausen Progressive cribriform and zosteriform annular plaques of, 145
syndrome hyperpigmentation (PCZH), arcuate lesions of chronic plaque
Pompholyx/dyshidrosiform eczema, 235 36–37 psoriasis, 145
Poor heat intolerance, 60 Progressive symmetric on glans penis, 420–421
Porokeratosis, 172, 423–424, 424, 532, erythrokeratoderma (PSEK), koebnerization, 145
534–535, 534–536 180, 188–189, 540 over glans penis, 146
of Mibelli, 523, 536 Proliferative leukoplakia, 367 papules, 78
palmaris, 536 Proliferative verrucous leukoplakia, scalp involvement, 145
ptychotropica, 536 367, 368 silvery scales in, 145
Porphyrias, 70, 615–618 Prostaglandin E2 injection, 9 vulgaris lesions, 181
cutanea tarda (PCT), 68–69, 116, Protein energy malnutrition (PEM), Psychocutaneous disorders, 495
269–271, 482, 605, 617, 625 476, 476 acne excoriée, 500
Port-wine stains (PWS), 571, 571, PRP, see Pityriasis rubra pilaris body dysmorphic disorder, 504
571–574 Prurigo nodularis, 83–85, 85, 162, clinical approach, 495
syndrome associated, 574 211–212, 497, 498, 625 delusional parasitosis, 503–504
Post-chikungunya nasal tip Prurigo pigmentosa, 68 dermatitis artefacta, 499
pigmentation, 31 Prurigo simplex, 495–496, 500 dermatitis passivata (dermatitis
Post-chikungunya pigmentation (PCP), Pruritic papules, 176 neglecta), 499
30–31, 50 Pruritic urticarial papules and plaques factitious cheilitis, 497
Posterior cervical lymphadenopathy, 545 of pregnancy (PUPPP), 169, lichen simplex chronicus, 496–497
Post-infectious purpura fulminans 195–196 munchausen by proxy, 502
(PF), 323 Pruritus, 504 munchausen syndrome, 501–502
Post-inflammatory hypopigmentation Pseudo-atrophy, 187 neurotic excoriations, 500
(PIH), 16, 17, 19, 31–32, 35, Pseudoclubbing, 452 obsessive-compulsive disorder
35–36, 35–36, 39, 49, 50, 53, 54, Pseudoepitheliomatous, keratotic, and (OCD), 497–498
102, 407, 409, 589 micaceous balanitis, 424–425 onychophagia, 503
654   Index

onychotemnomania, 503 thrombotic thrombocytopenic perioral dermatitis, 281

onychotillomania, 503 purpura (TTP), 317 pityrosporum folliculitis, 283
prurigo nodularis, 497 valsalva-associated petechiae, 318 pyoderma gangrenosum (pustular
prurigo simplex, 495–496 vitamin K deficiency bleeding type), 286
pseudo-knuckle pads, 497 (VKDB) disorder, 320 rosacea (papulopustular type),
psychogenic dysesthesia, 504 retiform purpura 282–283
psychogenic pruritus, 504 angioinvasive fungal scabies, 283
psychogenic purpura syndrome, 500 infections, 324 subcorneal pustular dermatosis
trichoteiromania, 502–503 anti-phospholipid syndrome (SCPD), 288
trichotemnomania, 502–503 (APLS), 325 sycosis barbae, 277–278
trichotillomania, 502–503 calciphylaxis, 325 tinea barbae, 278–279
when to suspect, 495 cholesterol emboli, 325 Pyoderma, 307
Psychogenic dysesthesias, 504, 504 cryoglobulinemia, 325 Pyoderma faciale, 341
Psychogenic pruritus, 504 heparin skin necrosis, 325 Pyoderma gangrenosum (PG), 96, 127,
Psychogenic purpura syndrome, 500 livedoid vasculopathy, 325–326 210–211, 216–217, 219, 286,
Psychotropic drugs, 53 lucio phenomenon (LPh), 323 295–296, 300, 303–304, 304,
Pterygium, 461–462 periumbilical thumbprint parasitic 306–307, 341, 354, 399, 425,
Punctate excoriations, 500 purpura (PTPP), 323–324 433–434
Punctate leukonychia, 463 polyarteritis nodosa, 323 of penis, 434
Punctate porokeratosis, 536 post-infectious purpura fulminans Pyogenic granulomas, 577, 580
Purple (blue) toe syndrome, 352 (PF), 323 Pyridoxine (vitamin B6) deficiency, 482
Purpura, 315 purpura fulminans with DIC
annularis telangiectodes, 320, 321 (septic vasculitis), 323
Q
clinical characteristics, 315–316 warfarin necrosis, 325
clinical diagnosis, 317 Purpuric annular dermatoses, 516 Quinolones, 604
and epidermal necrosis, 324 Purpuric annular lesions, 517
on face, 318 Purpuric dermatosis, 576
R
fulminans, 349, 349 Purpuric lesions, 164, 501
fulminans with DIC (septic Purpuric lesions in polyarteritis Racquet nail, 448
vasculitis), 323 nodosa, 323 Radiation mucositis, 391–392
palpable purpura Pus, 207 Radiation recall dermatitis, 609
ANCA-associated vasculitis, 322 Pustular psoriasis, 374, 412, 429, 605 Ram’s horn dystrophy, 449
cutaneous small vessel vasculitis Pustules, localized, 102, 104, 276 Rapidly involuting congenital
(CSVV), 322 acne keloidalis nuchae, 282 hemangioma (RICH), 178
gloves-and-socks syndrome, 323 acne vulgaris, 280–281 Rat-bite fever (sodoku, spirillum
Henoch-Schönlein purpura, 322 acrodermatitis continua, 285–286 minor), 355
urticarial vasculitis, 322 amicrobial pustulosis of the folds Raynaud’s phenomenon, 302–303, 325
petechiae and ecchymoses (APF), 288 Reactive angioendotheliomatosis, 581
actinic purpura, 317 bacterial folliculitis and impetigo, Reactive arthritis, 180
battered baby syndrome, 320 276–277 Reactive perforating collagenosis, 83, 83
coagulation-factor deficiency, 318 candidial folliculitis, 287–288 Reactive skin disorder, 187
corticosteroid purpura, 317 chronic glucocorticoid use and Reckling, 619
hemolytic-uremic syndrome abuse, 289 RecQ DNA helicase, 618
(HUS), 317 dermatophyte infections, 289–290 RecQ family of helicases, 619
heparin-induced dyshidrotic eczema/pompholyx, 287 RECQL4 gene, 618
thrombocytopenia, 315–317 erosive pustular dermatosis of RECQL2 (WRN) gene, 74
hypergammaglobulinemic scalp, 276 Recurrent aphthous stomatitis, 381–382
purpura of Waldenstrom, 318 folliculitis decalvans, 276 Redder spots, 410
idiopathic thrombocytopenic gram-negative folliculitis, 283 Red lesions, acquired disorders
purpura, 315 herpes simplex, 279–280 anemia, 394
leukemia/bone marrow herpes zoster, 288–289 denture-related stomatitis, 393
failure, 315 hidradenitis suppurativa, 288 erythroplakia, 393
pigmented purpuric infantile acropustulosis, 283–284 hereditary disorders, 390
dermatoses, 318 neonatal acne/infantile acne, 280 Infectious mononucleosis, 394
primary systemic orf, 286–287 lupus erythematosus, 393–394
amyloidosis, 318 palmoplantar pustulosis (PPP), 287 median rhomboid glossitis, 392–393
scurvy, 317 parakeratosis pustulosa, 284–285 plasma cell gingivitis, 393
 Index 655

radiation mucositis, 391–392 Rheumatoid arthritis (RA), 228 in Norwegian scabies, 194
Reiter’s disease, 394 Rheumatoid nodules, 152, 220, pityriasis rotunda, 168–169
thermal burn, 391 228–229, 232 seborrheic dermatitis (SD), 165–167
thrombocytopenic purpura, 394 Rhinocerebral disease, 343 Scaly varieties of tinea corporis, 194
traumatic erythema, 390–391 Riboflavin deficiency, genital Scar, 164
Reed nevus, 219 involvement in, 476 sarcoidosis, 231
Refsum disease, 622 Riboflavin (vitamin B2) deficiency, Scarring, 114, 114
Regional dermatology, 360 475–476 alopecia
Reiter’s disease, 180, 394, 411 Rickettsia infection, 345–346 in acne keloidalis, 556
Reiter’s syndrome, 459 Rickettsial diseases, 347 burn, 558
Renal anomalies, 580 Rickettsial pox, 347 Scedosporium prolificans, 336, 355
Reticular lichen planus, 368–369 Riehl’s melanosis, 44–46, 45, 104 Schamberg’s disease, 19, 32, 318, 321
Reticulate acropigmentation of Dohi, 72 Robinson type eccrine hidrocystoma, 114 Schamroth’s window, 451
Reticulated acropigmentation of Rocky Mountain spotted fever Schopf-Schultz-Passarge syndrome, 114
Kitamura (RAK), 65–66, 69 (RMSF), 347 Schwannoma, 220, 227
Reticulate hyperpigmentation, 58 Rombo syndrome, 112 Scleroderma, 69
causes of, 59 Rosacea, 79, 81, 102, 104–108, 111, 167 Scleromyxedema, 92
confluent and reticulated erythematotelangiectatic rosacea, Sclerosing lymphangitis, 427
papillomatosis (CRP), 67–68 104–105 Scrofuloderma, 213–214, 295,
dermatopathia pigmentosa reticularis like demodicosis, 107 335–336, 340
(DPR), 61–63 ocular rosacea, 105 inguinal region, 214
disorders causing, 60–61 papulopustular rosacea, 105 Scrotal calcinosis, 426
Dowling-Degos disease (DDD), 66 papulopustular type, 282–283 Scrotal cyst, 426
dyskeratosis congenita (DKC), 63–64 phymatous rosacea, 105 Scrotum, 407
erythema ab igne, 68 Rose gardener’s disease, 206–207 Scrub typhus, 347
fanconi anemia, 64–65 Rothmund syndrome, 74 with erythematous plaque, 347–348
Galli-Galli disease, 66–67 Rothmund-Thomson syndrome Scurvy, 317, 484–485
hidrotic ectodermal dysplasia, 61 (poikiloderma congenitale), 58, perifollicular petechiae in, 484
incontinentia pigmenti, 64 70, 614, 618–619, 621 Sebaceous adenoma, 228
Kindler syndrome, 58 Rough nails, 456 Sebaceous cyst, 126
Naegeli-Franceschetti-Jadassohn Rupioid psoriasis, 180 Sebaceous gland hyperplasia (SH), 110
syndrome (NFJ), 60–61 Sebaceous hyperplasia, 102, 110, 138,
prurigo pigmentosa, 68 228, 418, 418–419
S
reticulated acropigmentation of Seborrhea, 413
Kitamura (RAK), 65–66 SACRAL syndrome, 580 Seborrheic dermatitis (SD), 14, 102–103,
X-linked reticulate pigmentary Saddle nose deformity, 322 107, 109, 111, 140, 143, 148,
disorder, 58 Saksenaea vasiformis, 344 151, 165–167, 174, 192, 412,
Reticuloendotheliosis, 123 Salmon-colored follicular 524–525, 614
Retiform purpura, 316 hyperkeratosis, 180 differential diagnosis, 167
angioinvasive fungal infections, 324 Salmon patch, 571 greasy, yellow scales, 166
anti-phospholipid syndrome Sarcoidosis, 79–81, 115, 115, 116, 120, Seborrheic keratosis (SK), 27, 84–87, 87,
(APLS), 325 122, 130, 148, 151–152, 155, 113, 125, 134, 169, 169, 172, 174,
calciphylaxis, 325 158, 180, 183, 186–188, 176, 195, 228, 415
cholesterol emboli, 325 209–210, 218, 223, 231–232, Seborrheic melanosis, 49–50
cryoglobulinemia, 325 295, 402, 422, 434, 516, 530, Seborrhoeic keratosis, 27
heparin skin necrosis, 325 581, 585 Secondary milia, 112
livedoid vasculopathy, 325–326 Sarcoma, 218 Secondary niacin deficiency, 482
lucio phenomenon (LPh), 323 Sarcoptes scabiei var. hominis, 194 Secondary scarring alopecia, 558, 558
periumbilical thumbprint parasitic Satellite lesions, 159 Secondary syphilis, 108, 120, 121–122,
purpura (PTPP), 323–324 Scabby mouth, see Orf 121–122, 167, 167, 180, 193, 394,
polyarteritis nodosa, 323 Scabies, 98, 103, 283, 415, 496 524–525, 530
post-infectious purpura fulminans Scald-like erythema, 480 Segmental lentigines, 28–29
(PF), 323 Scalp psoriasis, 143 Senile angioma, 96, 587
purpura fulminans with DIC (septic Scaly papules, 194 Senile comedones, 140
vasculitis), 323 Scaly plaques, 213 Septic arthritis, 335
warfarin necrosis, 325 actinic keratosis (AK), 167–168 Septic emboli, 306
Retinoids, 210, 559 herald patch of pityriasis rosea, 167 Severe acne, 340
656   Index

Severe (and acute) ICD, 412 Splinter hemorrhage, 470 Subcutaneous heparin, 325
Severe cutaneous adverse reactions Sporadic angiofibroma, 76 Subcutaneous nodules, 214
(SCARs), 594 Sporothrix schenckii, 158 of lipoma, 229
Severe forms of CADR (SCAR), 599 Sporotrichosis (rose gardener’s disease), Subcutaneous panniculitic T-cell
Severe rosacea, 341 157–161, 206–207, 214, 220, lymphoma, 215
Shagreen patches, 170–171, 176 295, 301, 307, 336, 384 Subcutaneous sarcoidosis, 228
Shallow ulcer, 377–378 Squamous cell carcinoma (SCC), 70, 168, Subungal changes, 470
Shaving bumps, 118 172, 172, 217–218, 297–300, Subungal hyperkeratosis, 470
Shiny trachyonychia, 457, 457 368, 384–387, 401, 410, Subungual hematoma, 470
Short anagen syndrome (SAS), 562 423–425, 426, 434, 436, 537 Subungual melanoma, 469, 469
Sickle cell anemia, 335 of lip, 58 Sucking blister, 240
Silvery white lamellate, 180 ulcerated nodule of, 298 Sulfonamides, 594, 604, 606
Sinonasal squamous-cell cancer, 299 Squamous epithelium, 365 Sulzberger-Garbe disease, 194–195
Sisaipho type AA, 541 Staphylococcal abscess, 216 Summertime actinic lichenoid eruption,
SJS-TEN, 394 Staphylococcal carbuncle, 347 514–516
SJS-TEN overlap (Lyell’s syndrome), Staphylococcal scalded skin syndrome Sunburn, 594
603–605 (SSSS), 68, 264–265, 430 Sunitinib, 306
Skin appendageal tumor, 76 Staphylococcal skin abscess, 302 Superficial hemangiomas, 177
Skin-colored asymptomatic papules, 91 Staphylococcus aureus, 117, 207, 338, 345, Superficial thrombophlebitis, 209
Skin-colored papules of verruca 347, 467, 475, 568 Superinfection, 303
plana., 133 Stasis dermatitis, 213 Suppurative inguinal lymphadenitis, 338
Skin in systemic diseases, 472 Stasis eczema, 303 Suppurative lymphadenitis, 341
Skin lesions, 187, 219, 227, 339 Stasis purpura, 213 Sutton’s nevus, 8
Skin pigmentation, increased, 19 Steatocystoma, 427 Sweet syndrome (SS), 152, 180, 185–186,
Skin tags, 84, 90, 92, 223–224, 227, 420 Steatocystoma multiplex, 91–92, 103, 110, 210, 341, 608
Slate-gray pigmentation, 45 138, 221, 425 Swimming pool granuloma, 301
Small-plaque parapsoriasis, 167, 193 Stellate purpura in purpura Sycosis barbae, 117, 117, 277–278
Smith and Chernosky type (solitary) fulminans, 324 Symmetrical drug-related intertriginous
eccrine hidrocystoma, 114 Steroid acne, 116, 119 and flexural exanthema
Smoker’s melanosis, 388 Steroid dermatitis, 81 (SDRIFE), 596–597
Snakebite, 354 Steroid-induced acne, 120 Syphilis, 193, 295, 339, 383–384, 422,
Sneddon-Wilkinson disease, 538 Steroid-induced hypopigmentation, 5 433–434, 548
Soft fibroma, 227 Steroid-induced perilesional Syphilis gumma, 295
Solar lentigo, 26–27, 27, 66, 85 hypopigmentation., 516 Syphilitic alopecia (SA), 547–548
seborrheic keratosis, 169 Steroid induced rosacea, 111 Syphilitic chancre, 429
Solar urticaria, 625 Steroid-modified tinea, 520, 522 Syphilitic gumma, 306
Solitary cylindroma, 109 Stevens–Johnson syndrome (SJS), Syphilitic ulcer, 422
Solitary/few macules, 6 201, 378–379, 430, 594, Syringocystadenoma papilliferum,
Solitary nodule of mastocytoma, 224 603–605, 604 87, 174
Solitary papule, 86 in toxic epidermal necrolysis, Syringomas, 84, 88, 102, 111–112,
Solitary trichoepithelioma, 125, 221 267–269, 430 112, 114
Solitary ulcer of chancroid, 433 Streptococcal infection, 300 Systematized verrucous epidermal nevus,
Sorafenib, 112, 306 Streptococcal species, 345 200–201
Sore mouth, see Orf Streptococcus pneumoniae, 347 Systemic amyloidosis with facial purpura
Southern tick-associated rash illness, 518 Stretching of the skin, 125 and macroglossia, 319
Space occupying lesions (SOL), 454 Stucco keratoses seborrheic keratosis, 169 Systemic lupus erythematosus (SLE), 167,
Sparse hair of scalp, 62 Stucco keratosis (keratosis alba), 134 393, 551
Speckled lentiginous nevus syndrome, 24 Stump dermatosis in amputees, 213 Systemic sclerosis, 190, 427
Spider angioma, 577 Sturge-Weber syndrome, 574
Spider bite, 302 Subacute cutaneous lupus erythematosus
T
Spindle cell hemangioma, 218 (SCLE), 77, 180, 184–185, 524
Spiradenoma, 218 Subcorneal pustular dermatosis Target/iris lesions, 514
Spirochaete bacterium, 430 (SCPD), 288 Targetoid hemosiderotic hemangioma,
Spitz nevi, 219, 219 Subcutaneous fat necrosis of 96, 587
Spitz nevus, 23, 88, 95, 204, 220 newborn, 581 Tattooing effect, 205
Spitz’s juvenile melanoma, 219 Subcutaneous granuloma annulare T-cell infiltrative disease, 529
Splashed paint appearance, 6, 7 (SGA), 215, 228–229 T cell lymphoma, 187
 Index 657

Teeth malformation, 64 Transient neonatal hair loss, 554 Tufted angioma, 96, 574, 580–581
Tegenaria, 355 Transverse grooving (TG), 457–458 Tufted folliculitis, 84, 568
Telangiectatic vessels, 76 Transverse overcurvature of nail, Tufted hemangioma, 580
Telogen effluvium (TE), 548, 550–551, 452–453 Tularemia, 295, 302, 307, 346
554–555, 559–560, 561, Trauma, 429, 437 Tumid LE, 81
562, 564 Traumatic anserine folliculosis, 118–119, Tumid lupus erythematosus, 152, 155
types, 560 118–119 Tumoral calcinosis, 228
Temporal arteritis, 299 Traumatic erythema, 390–391 Tumor alopecia, 558
Temporal triangular alopecia (TTA), Traumatic hair styling, 550 Tumor necrosis factor (TNF)–alpha
553–555 Traumatic lesions, 391 inhibitors, 603
Tendinous xanthoma, 154–155 Traumatic subungual hematoma, 470 Tumor stage, mycosis fungoides (MF), 72
Terra firma-forme dermatosis (TFFD), Traumatic ulcers, 303, 381, 383, Tumour of follicular infundibulum, 15
20, 67 433–434, 559 Turban tumors, 218
Terry’s nails, 463–464 Trench foot, 302 Turner syndrome, 124
Tertiary syphilis (gumma), 222–223, Treponema pallidum, 121, 193, 430, 547 Twenty-nail dystrophy, 456
295, 336 Treponematoses, 75 Tyndall effect, 20
Tetracyclines, 53, 604, 606 Trichilemmal cyst, 92, 127, 222 Tyrosinemia, 622
Tetrahydrobiopterin deficiency, 622 Trichoderma, 355 Tyson’s glands, 419
Thermal burns, 243–244, 391 Trichodynia, 504
Thromboangiitis obliterans (TAO), 304 Trichoepithelioma, 84, 103, 108, 115,
U
Thrombocytopenia, 206 218, 222
Thrombocytopenic purpura, 394 Trichofolliculoma, 127, 127 Ulcerated hemangioma, 294
Thrombophlebitis, 209, 427, 436 Trichophyton and Microsporum, 542 Ulcerated infantile hemangioma, 295
Thrombosed plantar wart, 205 Trichophyton concentricum, 520 Ulcerated lesion in mucormycosis, 308
Thrombotic thrombocytopenic purpura Trichophyton mentagrophytes, 545 Ulcerated plaques, 297, 301
(TTP), 317, 484 Trichophyton tonsurans, 544 basal cell carcinoma, 171–172
Thyroglossal cyst, 334 Trichophyton verrucosum, 545 Bowen’s disease, 172–173
Thyroglossal duct cyst, 334 Trichophyton violaceum, 544 necrobiosis lipoidica
Thyroid disorders, 39 Trichorrhexis invaginata (Bamboo diabeticorum, 172
Tick-borne rickettsial infections, 307 hairs), 563 Paget’s disease, 173–174
Tieche-Jadassohn nevus, see Blue nevus Trichorrhexis nodosa (TN), 563 pemphigus vegetans, 173
Tile-shaped nail, 453 Trichostasis spinulosa (TS), 116–117, 117 Ulcerating sarcoidosis, 434
Tinea barbae, 117–118, 278–279 Trichoteiromania, 502–503 Ulcerative lesions preceding with
Tinea capitis, 167, 503, 541–546, 548, Trichotemnomania, 502–503 vesico-bullous lesion
554, 558, 568 Trichothiodystrophy (TTD), 70, 621–622 bullous pemphigoid, 379–380
differential diagnosis of, 546 Trichotillomania, 502–503, 502–503, 548, cicatracial pemphigoid, 380
gray patches of, 545 551, 554–558, 562 dermatitis herpetiformis, 380
Tinea corporis, 76, 148, 154, 169, 185–187, diagnostic criteria (DSM-5), 558 epidermolysis bullosa, 381
192–193, 516, 523–524, 527, Trigeminal trophic syndrome, 500 epidermolysis bullosa acquisita, 381
532, 539 Tropical ulcer, 303 erythema multiforme, 378
Tinea cruris, 521 True knuckle pads, 497 hand, foot, and mouth disease,
Tinea faciei, 120, 139–140, 525 Truncal lesions, 166 377–378
Tinea imbricata (tokelau), 520 TT leprosy with reaction, 152 herpangina, 377
Tinea incognito, 520 Tubercular gumma, 296 herpes simplex virus infection,
Tinea manuum, 521 Tuberculid, 90 375–376
Tinea versicolor, 35, 524 Tuberculoid (TT), 528 herpes zoster, 376
Toasted skin syndrome, 68 leprosy, 11, 144 linear IgA disease, 380
Tobacco, 366 pole leprosy, 143 pemphigoid gestationis, 380
Tonsure trichotillomania, 556 Tuberculosis, 51, 341, 383, 434–435 pemphigus vulgaris, 379
Tophus (podagra), 221–222, 230 Tuberculosis verrucosa cutis, 156–163, Stevens-Johnson syndrome,
Total leukonychia, 463 339, 536 378–379
Toxic epidermal necrolysis (TEN), 379, Tuberculous chancre, 294–295 toxic epidermal necrolysis, 379
430, 594, 603–605 Tuberculous gumma, 295 Ulcerative lesions without preceding
Trachyonychia, 456–457 Tuberculous osteomyelitis, 226 with vesicobullous lesions
Traction alopecia (TA), 550–551, 551, 558 Tuberous sclerosis, 5 Behçet’s disease, 382–383
with fringe sign, 550 Tuberous sclerosis complex (TSC), 106 eosinophilic ulcer, 384
Transient acantholytic dermatosis, 192 Tuberous xanthoma, 154, 216, 222 oral ulcer, rarer causes, 384
658   Index

oral ulcer caused by fungal tularaemia, 307 lobular capillary hemangioma (LCH),
infection, 384 ulcerated hemangioma, 294 574–576
recurrent aphthous stomatitis, venous ulcer, 303 microcystic lymphatic malformation,
381–382 warfarin-induced skin necrosis, 306 584–585
squamous cell carcinoma, 384–387 Ultraviolet (UV) radiation exposure, nevus anemicus, 588
syphilis, 383–384 29, 614 port-wine stains (PWS), 571–574
traumatic ulcers, 381 Umbilicated papules of molluscum, 413 presentations of cutaneous, 571
Ulcers, 76, 303, 325, 580 Unfractionated heparin, 353 reactive angioendotheliomatosis, 581
in herpes zoster, 299 Unilateral foot swelling, 337 salmon patch, 571
in necrobiosis lipoidica, 303 Unilateral nevoid telangiectasia, 576 targetoid hemosiderotic
Ulcers, single or few, 293 Urethral diverticulum, 427 hemangioma, 587
angiosarcoma, 299 Urinary tract infection, 437 tufted angioma, 580–581
aplasia cutis congenita, 293–294 Urogenital anomalies, 580 venous lake, 587
arterial ulcer, 304 Urticaria, 152, 156, 169, 189, 190, 193, venous malformation, 587
basal cell carcinoma (BCC), 297 196, 525–526, 607 verrucous hemangioma, 584
buruli ulcer (Bairnsdale ulcer), Urticarial lesions, 325 Vascular malformations, 211, 572, 576, 580
306–307 Urticarial phase of bullous Vascular occlusion, 304
calciphylaxis, 300 pemphigoid, 189 Vascular papules on scrotum, 95
cat scratch disease, 307 Urticarial vasculitis, 180, 189–190, 322 Vascular plaques, 177
chemotherapy agents and ulcer, 306 Urticarial wheals, 169 Vascular tumors, 219
clinical approach to diagnosis, 294 Urticaria pigmentosa, 88, 189, 189, Vascular tumors and malformations, 580
clinical clue for diagnosis, 293 196, 196 Vasculitides, 322
crusts, 299 Uveitis, 625 Vasculitis, 213, 304, 581
cutaneous anthrax, 301–302 Vellous hair cysts, 110, 113–114, 131
cutaneous Crohn disease, 308 Venous lake, 587
V
cutaneous leishmaniasis, 295–296 Venous malformations (VM), 178, 202,
decubitus ulcers, 299–300 Vagabond’s leukomelanoderma, 74–75 581, 585, 587
ecthyma, 300, 300 Valproic acid, 604 syndromes, 588
ecthyma gangrenosum, 306 Valsalva-associated petechiae, 318 Venous stasis, 32
frostbite, 302 Variable sized macules, 6 Venous thrombosis, 435
Glanders disease, 307 Varicella, 265–267, 378 Venous ulcer, 303
granulomatosis with polyangiitis zoster, 376 Ventral pterygium, 462
(GPA), 296 Vascular anomaly syndrome, 202 Verneuil’s disease, 207
herpes zoster, 299 Vascular lesions, 571 Verruca, 418
kangri ulcer, 300 acroangiodermatitis of Mali, Verruca plana (pseudo koebnerization),
martorell ulcer, 303 585–586 90, 107, 112, 114, 132, 224
myiasis, 307 angiokeratoma, 581–584 Verruca vulgaris, 86–87, 95, 132, 134,
necrobiosis lipoidica diabeticorum/ angiolymphoid hyperplasia with 157, 159, 162–163, 169, 195, 368,
necrobiosis lipoidica, 303 eosinophilia, 585 395–397, 414
necrotizing fasciitis (NF), 304–306 angioma serpiginosum, 576 Verruciform xanthoma, 396
nicolau syndrome, 303 angiosarcoma, 587 Verrucous carcinoma, 162, 395–397,
noma/cancrum oris, 294 arteriovenous malformation, 588 424–425
pyoderma gangrenosum, 304, 304 bacillary angiomatosis, 577 on glans penis, 425
Raynaud’s phenomenon, 302–303 cherry angioma (Campbell de Verrucous epidermal nevus, 132,
scrofuloderma, 295 Morgan spots), 576–577 134–135, 169, 174–175, 175,
sporotrichosis, 301 clinical approach to, 572 176, 200
squamous cell carcinoma (SCC), congenital hemangioma, 580 Verrucous flat-topped papules, 162–163
297–298 dabska tumor, 587 Verrucous hemangioma, 176–177,
swimming pool granuloma, 301 glomus tumor, 587 418, 584
syphilitic gumma, 306 glomuvenous malformations Verrucous lesions, lumps and swellings
temporal arteritis, 299 (glomangioma), 587 abscesses, 397
thromboangiitis obliterans granuloma pyogenicum, 574–576 angioedema, 397
(TAO), 304 hemangiopericytoma, 581 fibroma, 398–399
trench foot, 302 infantile hemangioma (IH), 577–580 gingival hyperplasia, 399–400
tropical ulcer, 303 kaposiform hemangioendothelioma, 581 mucocele (mucous cyst or ranula),
tuberculous chancre, 294–295 kaposi sarcoma, 586 397–398
tuberculous gumma, 295 kimura disease, 585 pyogenic granuloma, 399
 Index 659

Verrucous lesions, papilloma due to Vesicobullous lesions, localized, 235 Volkmann’s cheilitis, 402
chronic irritation, 395 acute irritant contact dermatitis Von Recklinghausen’s disease/NF-1, 230
acanthosis nigricans, 397 (ICD), 245–246 Von Willebrand disease, 484
condyloma acuminatum, 395–396 allergic contact dermatitis, 244–245 Vulvodynia, 504
Darier disease, 397 blistering distal dactylitis, 240–241
focal epithelial hyperplasia (Heck’s bullous cellulitis, 249
W
disease), 396 bullous fixed drug eruption, 242–243
verruca vulgaris, 395 bullous impetigo, 241–242 Waardenburg syndrome, 8
verruciform xanthoma, 396 bullous insect-bite reaction, 243 Warfarin-induced skin necrosis
verrucous carcinoma, 396–397 bullous leukocytoclastic (WISN), 306
Verrucous plaques, 200, 582 vasculitis, 240 Warfarin necrosis, 325, 352
in tuberculosis verrucosa cutis, bullous scabies, 237–239 Warts, 87–88, 124, 132, 200, 207, 414,
156–157 coma blister, 240 419, 536; see also Filiform
of verruca vulgaris, 396 cutaneous anthrax, 251 warts; Genital warts; Plane
Verrucous stage of incontinentia diabetic bulla/bullous warts; Viral warts
pigmenti, 175 diabeticorum, 240 Warty papules, 159, 191
Verruga peruana, 577 ecthyma gangrenosum, 250–251 Warty plaques, 159
Vertigo, 122 epidermolysis bullosa (EB), 235 Washing eczema, 498
Vesicles of lymphangioma erysipelas, 249–250 Wegener’s granulomatosis, 220, 322, 603
circumscriptum, 584 erysipeloid, 251 Well-demarcated depigmented patch, 407
Vesicobullous lesions, generalized, 254 erythema multiforme, 235 Wells syndrome (Eosinophilic
acute phototoxic drug reactions, friction blister, 243 cellulitis), 213
271–272 hand, foot, and mouth disease, 237 Werner syndrome, 70, 74, 621
bullous lichen planus (BLP), 263 herpes simplex, 246 Whipple disease, 434
bullous pemphigoid, 259 herpes zoster (HZ), 246–247 White blanching ring around the
Bullous SLE, 272 hidrocystoma, 252–253 lesion, 143
congenital syphilis, 273–274 incontinentia pigmenti, 247–248 White lesions, 365–366
dermatitis herpetiformis, 263 lymphangioma circumscriptum, 248 candidiasis, 373–374
diffuse cutaneous mastocytosis, 265 orf, 251–252 chemical burn, 375
epidermolysis bullosa acquisita paederus dermatitis, 248–249 dyskeratosis congenita, 375
(EBA), 261 pompholyx/dyshidrosiform fissured tongue, 374
epidermolytic ichthyosis (bullous eczema, 235 geographic tongue, 370–372
congenital ichthyosiform sucking blister, 240 hairy leukoplakia, 368
erythroderma), 272 thermal burns, 243–244 hairy tongue, 372–373
Grover’s disease, 254 vesicobullous tinea pedis, 239 leukoedema, 368
Hailey-Hailey disease, 269 vibrio vulnificus infection, 251 leukoplakia, 366–368
IgA pemphigus, 258–259 Vesicobullous tinea pedis, 239 lichen planus, 368–370
inherited epidermolysis bullosa, 273 Vesicular PLE, 625 of mucosa, 366
kaposi varicelliform eruption, 267 Vibrio vulnificus infection, 251 nicotinic stomatitis, 375
langerhans cell histiocytosis, 269 Violaceous macules, 213 pachyonychia congenita, 375
lichen planus pemphigoides (LPP), Violaceous nodules, 586 white sponge nevus, 375
263–264 Violaceous papules White plaque on buccal mucosa lip, 367
linear IgA dermatosis/chronic bullous of lichen planus, 414 White sponge nevus, 368, 375
disease of childhood, 261–262 plaque of lichen planus, 414 Whitish confluent papules, 397
miliaria crystallina, 254 Viral exanthem, 599 Whitish polygonal papules, 164
paraneoplastic pemphigus (PNP), Viral warts, 132–133, 583 Wickham striae on buccal mucosa, 368
257–258 Visceral leishmaniasis (Kala azar), 12 Winkler’s disease, see Chondrodermatitis
pemphigoid gestationis, 259–261 Vitamin A deficiency, 476–478 nodularis helicis
pemphigus foliaceus, 269 Vitamin B12 deficiency, 51–52, 478 Wohlfahrtia opaca, 339
pemphigus vulgaris, 254–257 Vitamin B2 (riboflavin) deficiency, 482 Wohlfahrtia vigil, 339
porphyria cutanea tarda (PCT), 269–271 Vitamin C deficiency, 317 Wood’s lamp examination, 7, 30
pseudoporphyria, 271 Vitamin K deficiency, 483–484 Woolsorter’s disease, 347
staphylococcal scalded skin syndrom Vitamin K deficiency bleeding (VKDB) Woringer–Kolopp pagetoid reticulosis,
(SSSS), 264–265 disorder, 320 153, 187
Stevens-Johnson syndrome/toxic Vitiligo, 7, 9, 10, 14, 69, 407, 423, 589 Woronoff’s ring, 9, 143
epidermal necrolysis, 267–269 Vogt-Koyanagi-Harada syndrome, 9 Wound myiasis, 339
varicella, 265–267 Voight’s lines, 47 Wyburn–Mason syndrome, 589
660   Index

X X-linked reticulate pigmentary Z

disorder, 58
Xanthelasma palpebrum, 154, 155 Zebra-like pattern, 193
X-ray therapy, 603
Xanthogranulomas, 223 Zinc deficiency, 476, 480, 482
Xanthomas, 91, 152, 154–155, 170, 176, Zinsser-Engman-Cole syndrome, 63
187, 220–221, 228, 434 Zoon’s balanitis, 409–410
Y
Xanthomata, 223 Zoster, 376
Xeroderma pigmentosum (XP), 58, Yaws, 75 Zosteriform, 109
69–70, 71, 72, 619–621 Yeast Cryptococcus neoformans, 122 hyperpigmentation, 28
Xerostomia, 373 Yellow nail syndrome, 466 nodules of leiomyoma, 208
X-linked dominant genodermatosis, 64 Yellow-orange solitary papule, 87 Zygomycetes, 304, 324
X-linked recessive, 63 Yersinia pestis, 347 Zygomycoses, 343