Scribd
Upload
58 views34 pages

Chronic Vesico-Bullous Disorders Guide

This document summarizes various chronic vesicobullous diseases. It describes three main classifications: epidermal blisters including pemphigus vulgaris, subepidermal blisters including bullous pemphigoid, and infrabasal blisters including dermatitis herpetiformis. Pemphigus vulgaris is characterized by chronic intraepidermal blisters on normal to erythematous skin and commonly involves the oral mucosa. Bullous pemphigoid features tense subepidermal blisters that often arise on erythematous skin of the trunk and limbs. Dermatitis herpetiformis, associated with gluten intolerance, is defined by clusters of tense vesicles with severe

Uploaded by

Muthia Dewi
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd
58 views34 pages

Chronic Vesico-Bullous Disorders Guide

This document summarizes various chronic vesicobullous diseases. It describes three main classifications: epidermal blisters including pemphigus vulgaris, subepidermal blisters including bullous pemphigoid, and infrabasal blisters including dermatitis herpetiformis. Pemphigus vulgaris is characterized by chronic intraepidermal blisters on normal to erythematous skin and commonly involves the oral mucosa. Bullous pemphigoid features tense subepidermal blisters that often arise on erythematous skin of the trunk and limbs. Dermatitis herpetiformis, associated with gluten intolerance, is defined by clusters of tense vesicles with severe

Uploaded by

Muthia Dewi
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPT, PDF, TXT or read online on Scribd
You are on page 1/ 34

CHRONIC VESICO – BULLOUS

CLASSIFICATION OF CVB
1. EPIDERMAL BLISTER
- Pemphigus vulgaris
- Pemphigus foliaceus
- Pemphigus erythematous
2. SUB-EPIDERMAL BLISTER
- Bullous pemphygoid
- Dermatitis herpetiformis
3. INFRA-BASAL BLISTER
- Dermatitis herpetiformis
PEMPHIGUS VULGARIS

Definition :
A disease of skin and mucous
membrane that characterized by
chronic intraepidermal blister on
normal to erythematous skin
EPIDEMIOLOGY
• Male = female, age : 50-60 years
• Children are rare

ETIOLOGY
• Certainty?
• Drugs : penicillamine, sulfhidryl,
captopril,penicillin,rifampicin,etc.
CLINICAL FEATURES

 flaccid vesicles or blister, fragile, erosion


hyperpigmentation and crusts
 oral mucous membrane, esofagus with
swallowing disorder
 Predilection : oral mucous membrane,
scalp, trunk, buttock, face, neck, armpit,
genital
PATHOGENESIS

auto-immune mechanism, acantholysis, intra epidermal

antibody binding surface cell glycoprotein

plasmin and enzyme activity

Intercellular cement damage


SUPPORTING EXAMINATION
 Nikolsky’s sign
 Asboe-Hansen’s sign (“spread phenomen”)
 Histopathology :
- acantholytic cells (Tzanck’s method)
- intra epidermal blister
- IgG dermoepidermal junction
(immunofluorescence)
TREATMENT
 Severe, hospitalized
 Topical and systemic therapy,

corticosteroid, immunosupressive agent,


cyclophosphamide
PROGNOSIS
• Quo ad vitam, high mortality rate
• Quo ad functionam, dubia
• Quo ad sanationam, dubia
Pemphigus vulgaris
Pemphigus vulgaris

Flaccid bullae easily ruptur


Pemphigus vulgaris

Flaccid bullae

At mucous membrane
Pemphigus vulgaris
Pemphigus vulgaris
Pemphigus foliaceus
Pemphigus foliaceus
Pemphigus erythematous
Paraneoplastic pemphigus
Paraneoplastic pemphigus
PEMPHYGOID BULLOSA
DEFINITION :
tense vesicles/blister,chronic,erythematous base
ETIOLOGY :
 Certainty ?

 Auto-immune (concomittant with other

autoimmune diseases,e.g. rheumatoid,


myastenia gravis)
EPIDEMIOLOGY :
 All ages, especially elderly

 Male = female
PATHOGENESIS

Antigen Complex + Antibodies (IgG,C3,IgM)

Lamina lucida (BMZ)

Inflammation, tissues damage


CLINICAL FEATURES
 Good general state, mucous membrane (20%)
 Predilection : armpit, flexor forearm, groin
 Vesicles,blister,erosion
Supporting Examination
 Histopathology, sub epidermal blister
 IgG,C3 sedimentation  dermoepidermal junction
Treatment
 Topical dermatotherapy
 Systemic :
Corticosteroid
Dapsone
Sulphapyrimidine
Bullous Pemphigoid

Tense bullae
Bullous Pemphigoid

Tense bullae
Cicatricial pemphigoid
 Synonims : mucouse membrane pemphigoid,
ocular pemphigoid, scarring pemphigoid, oral
pemphigoid.
 chronic blistering of mucosa
 may involve the skin
 usually result in permanent scarring of
affected area
Cicatricial pemphigoid
Cicatricial pemphigoid
Cicatricial pemphigoid
DERMATITIS HERPETIFORMIS
(Duhring’s disease)

 DEFINITION
Clustered vesico-bullous, erythematous base,
severe itching
 EPIDEMIOLOGY

- Male = female
- Age of 20-40 years
- Children : rare
• ETIOLOGY ?
• CLINICAL FEATURES
Vesicle-blister,tense, content clear fluid
turbid, subjective severe itching

predilection: scalp, posterior neck, posterior armpit,


sacral,extensor forearm,knee,and elbow

• PATHOGENESIS
Disorder of jejunum mucosa
Gluten + IgA inflammatory response
skin damage
SUPPORTING EXAMINATION
- KI test
-Histopathology:

subepidermal blister, eosinophilic infiltration


-Immunofluorescence, IgA & C3 deposits in dermal papillary

TREATMENT :
- Gluten-free diet
-Topical dermato therapy

-Systemic dermatotherapy: DDS, sulfapyridine

PROGNOSIS :
- Quo ad vitam and functionam ad bonam
-Quo ad sanationam dubia (recidive chronic)
Dermatitis herpetiformis
Dermatitis herpetiformis
Dermatitis herpetiformis

You might also like