SKIN PATHOLOGY

Pathology Anatomic Department 2012

Normal Skin

Skin -Normal

Stratified Squamous epithelial cells (keratinocytes) = production of keratin protein, Melanocytes =production of a brown pigment (melanin) Langerhans cells = take up and process antigens Neural end-organs and axonal processes Merkel cells =
within the basal cell layer mechanoreceptors or neuroendocrine function

Skin -Normal
Sweat glands - temperature control Specialized dermal cells (dendrocytes) - for antigen presentation as well as for production of molecules (e.g., factor XIIIa) Hair follicle- harbor protected repositories of epithelial stem cells

 DEFINITIONS OF MACROSCOPIC TERMS Macule Circumscribed lesion of up to 5 mm in diameter characterized by flatness and usually distinguished from surrounding skin by its coloration. Patch Circumscribed lesion of > 5 mm in diameter characterized by flatness and usually distinguished from surrounding skin by its coloration. Papule Elevated dome-shaped or flat-topped lesion 5 mm or less across. Nodule Elevated lesion with spherical contour > 5 mm across.

Skin - Pathology

Skin - Pathology
Plaque
Elevated flat-topped lesion, usually > 5 mm across (may be caused by coalescent papules).

Vesicle
Fluid-filled raised lesion 5 mm or less across.

Bulla
Fluid-filled raised lesion > 5 mm across.

Blister
Common term used for vesicle or bulla.

Pustule
Discrete, pus-filled, raised lesion.

Wheal
Itchy, transient, elevated lesion with variable blanching and erythema formed as the result of dermal edema.

Skin - Pathology
Scale Dry, horny, plaque like excrescence; usually the result of imperfect cornification. Lichenification Thickened and rough skin characterized by prominent skin markings; usually the result of repeated rubbing in susceptible persons. Excoriation Traumatic lesion characterized by breakage of the epidermis, causing a raw linear area (i.e., a deep scratch); often self-induced. Onycholysis Separation of nail plate from nail bed.

Skin - Pathology
Hyperkeratosis Thickening of the stratum corneum, often qualitative abnormality of the keratin. Parakeratosis Modes of keratinization characterized by the retention of the nuclei in the stratum corneum. Hypergranulosis Hyperplasia of the stratum granulosum, often due to intense rubbing. Acanthosis Diffuse epidermal hyperplasia. Papillomatosis Surface elevation caused by hyperplasia and enlargement of contiguous dermal papillae.

 Dyskeratosis Abnormal keratinization Acantholysis Loss of intercellular connections resulting in loss of cohesion between keratinocytes. Spongiosis Intercellular edema of the epidermis. Hydropic swelling (ballooning) Intracellular edema of keratinocytes, often seen in viral infections. Exocytosis Infiltration of the epidermis by inflammatory or circulating blood cells. Erosion Discontinuity of the skin exhibiting incomplete loss of the epidermis

Skin - Pathology

Skin - Pathology
Ulceration
Discontinuity of the skin exhibiting complete loss of the epidermis and often of portions of the dermis and even subcutaneous fat.

Vacuolization
Formation of vacuoles within or adjacent to cells; often refers to basal cell-basement membrane zone area.

Lentiginous
Referring to a linear pattern of melanocyte proliferation within the epidermal basal cell layer. occur as a reactive change or as part of a neoplasm of melanocytes.

SKIN PATHOLOGY
Pigmentation Epidermal tumors, benign Epidermal tumors premalignant Epidermal tumors, malignant Dermal tumors Immigrant tumors Maturation disorders Dermatoses, acute Dermatoses, chronic Blisters (Bullae) Appendage (adnexal) disorders Panniculitis Infection/Infestation

Tumors of the Dermis

XANTHOMAS collections of foamy histiocytes within the dermis lipid filled Familial or acquired resulting in hyperlipidemia five types. Eruptive wax and wane according to variations in plasma triglyceride and lipid contents Tuberous and tendinous Achilles tendon & extensor tendons of the fingers Plane with primary biliary cirrhosis Xanthelasma (without lipid abnormality) eyelids.

Tumors of the Dermis

DERMAL VASCULAR TUMORS Benign (capillary and cavernous hemangiomas), malformations (nevus flammeus or port-wine stain), gone with age 14 to15 malignant vascular tumors (Angiosarcomas) bad prognosis seen in liver patients exposed to plastics angioproliferative lesions (Kaposi sarcoma HIV (tumor like- bacillary angiomatosis)

 Increased number of normal or abnormal vessels Localized : hemangioma (vasc.mal) Involved large segment of the body : angiomatosis Extremely common Present from birth in children

Hemangioma

Capillary hemangioma (1)

Composed of blood vessels that resemble capillaries Skin, subcutaneous tissue, mucous membrane, internal viscera Juvenile hemangioma (strawberry nevus)
Common in the newborn Grow rapidly in the first few months Fade when child is 1-3 yrs regress by age 7 in 7590%

Capillary hemangioma (2)

Gross Few mm. to several cm. Bright red to blue Level with skin surface or slightly elevated or pedunculated

Juvenile hemangioma

Capillary hemangioma (3)

Histology Unencapsulated, lobulated aggregates of closely packed, thin-walled capillaries, usually blood filled Scant CNT stroma Fibrin thrombi

Capillary hemangioma

Cavernous hemangioma (1)

Less common than capillary type Composed of large, dilated vascular channels Larger & involve deep structure than capillary type Gross : red-blue, soft, sponge mass, 1-2 cm.

Cavernous hemangioma (2)

Histology Well demarcated but not encapsulated Large, cavernous vascular spaces filled with blood Scant CNT stroma Thombosis with dystrophic calcification (phlebolith)

Cavernous hemangioma

Venous hemangioma
Present in adult Involve deep structure : retroperitoneum, mesentery, muscle of extremity Thick-walled with less well organized smooth muscle and often blend with surrounding soft tissue Phleboliths

Venous hemangioma

Granulation tissue-type hemangioma (1)

Pyogenic granuloma or lobular hemangioma Polypoid form of capillary hemangioma Rapidly growing red nodule of skin, gingiva, oral mucosa Easily bleeds and ulcerated 1/3 develop after trauma

Granulation tissue-type hemangioma (2)

Extensive edema, acute & chronic inflammatory infiltrate like exuberant granulation tissue Granuloma gravidarum
Pyogenic granuloma of gingiva of 1% of pregnant woman Regress after delivery

Pyogenic granuloma

Intramuscular hemangioma (1)

Most common hemangioma of deep soft tissue 80-90% manifested before age 30 but may be congenital and slow growing Most affects muscles of thigh, head & neck

Intramuscular hemangioma (2)

Clinical
Enlarged soft tissue mass May cause pain and deformity Diagnosis by radiograph or arteriography

Histology
Blood v.(capillary or cavernous) extend between individual muscle fibers Presence of adipose tissue within the tumor

Local recurrence ~ 18-50%

Intramuscular hemangioma

Angiosarcoma (1)
Malignant endothelial tumor Older adults Skin, soft tissue, breast and liver Etiology
Chronic lymphedema (post-radical mastectomy) Radiation Foreign body Carcinogens (hepatic angiosarcoma) : arsenical pesticides, thorothrast, PVC

Angiosarcoma (2)
Classification
Cutaneous angiosarcoma unassociated with lymphedema most common* Cutaneous angiosarcoma associated with lymphedema (lymphangiosarcoma) Angiosarcoma of the breast Radiation-induced angiosarcoma Angiosarcoma of deep soft tissue

Cutaneous angiosarcoma unassociated with lymphedema (1)

Most common Elderly person Head & neck region : scalp, forehead Clinical
Ill-defined bruise-like area with indurated border Advanced lesion : elevated, nodular, ulcerated

Cutaneous angiosarcoma unassociated with lymphedema (2)

Gross
Hemorrhagic area with microcystic or spongelike appearance

Histology
Well or moderately differentiated
Distinct vascular channels (hemangioma-like) anastomosing with each other and lined by atypical endothelial cells

Cutaneous angiosarcoma unassociated with lymphedema (3)

Poorly differentiated
Pleomorphic spindle cell sarcoma with vascular channels Proved by immunohistochem. CD31, CD34, vWF

Prognosis : poor
Metastasis to cervical lymph node, lung, liver and spleen

Angiosarcoma

Lymphangioma (1)
Developmental malformation Classification
Capillary lymphangioma Cavernous lymphangioma Cystic lymphangioma (cystic hygroma)

Relatively rare compared with hemangioma

Lymphangioma (2)
Location
Head & neck more common Retroperitoneum

Clinical
Soft fluctuated mass enlarge, static or waxes & wanes Cystic hygroma detect in utero by U/S, hydrops fetalis

Lymphangioma (3)
Gross Vary from well-circumscribed lesion to illdefined spongelike compressible lesion Histology Lymphatic spaces lined by attenuated endothelium Large lymphatic spaces have fascicles of poorly developed smooth muscle The spaces contain proteinaceous fluid and lymphocytes (occasionally erythrocytes) Fibrous stroma with small lymphoid aggregates

lymphangioma

Cystic hygroma

Tumors of Cellular Immigrants to the Skin

MYCOSIS FUNGOIDES
(CUTANEOUS T-CELL LYMPHOMA) ( seen in celiac sprue and HTLV)

lymphoproliferative disorders affecting the skin Two different clinical types Mycosis fungoides, a chronic proliferative process Nodular eruptive variant, mycosis fungoides d'emble Mycosis fungoides primarily in the skin and that may evolve into generalized lymphoma most commonly persons older than age 40.

Tumors of Cellular Immigrants to the Skin

MYCOSIS FUNGOIDES
(CUTANEOUS T-CELL LYMPHOMA)

Clinically
scaly, red-brown patches; raised, scaling plaques that may even be confused with psoriasis Fungating nodules- Development of multiple red-brown nodules correlates with systemic spreading seeding of the blood by malignant T cells is accompanied by diffuse erythema and scaling of the entire body surface (erythroderma), a condition known as Szary syndrome

Tumors of Cellular Immigrants to the Skin

MYCOSIS FUNGOIDES (CUTANEOUS T-CELL LYMPHOMA) Morphology
histological hallmark -Szary-Lutzner cells they have cerebriform nuclei ie looks like brain (T-helper cells (CD4 positive) that characteristically form band like aggregates within the superficial dermis infolded nuclear membranes -hyperconvoluted or cerebriform contour

(epidermotropism) and form clusters Pautrier microabscesses.

Definitions
Extranodal Non-Hodgkins lymphoma of Tcell origin, with primary involvement of the skin Sezary Syndrome: Generalized erythroderma Lymphadenopathy Atypical T- cells (Sezary cells) in the peripheral blood

Epidemiology MF

3 cases/ 1,000,000/ year<1000/year US Peak age 55-60 Male: female 2:1

Clinical
Indolent cutaneous eruption with erythematous scaly patches or plaques, typically bathing trunk distribution Heterogeneity in presentation

Extracutaneous Disease
Progression to Extracutaneous disease correlates with extent of skin disease Limited patch or plaque very rare Generalized plaque 8 % Tumorous or generalized erythroderma30-40%
Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

Histology

Sezary Cell

Hoppe RT, Kim YH,Clinical features, staging, and prognosis of mycosis fungoides and Sezary syndrome, Uptodate.com, 11/06

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