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1. The document discusses three types of chronic vesico bullous diseases: pemphigus vulgaris (epidermal blister), bullous pemphygoid (sub-epidermal blister), and dermatitis herpetiformis (infra-basal blister).
2. Pemphigus vulgaris is an autoimmune disease characterized by chronic intraepidermal blisters on normal to erythematous skin. It predominantly affects oral mucosa and causes blisters and erosions. Treatment involves topical and systemic corticosteroids and immunosuppressants.
3. Bullous pemphygoid also involves sub-epidermal blistering and is
1. The document discusses three types of chronic vesico bullous diseases: pemphigus vulgaris (epidermal blister), bullous pemphygoid (sub-epidermal blister), and dermatitis herpetiformis (infra-basal blister).
2. Pemphigus vulgaris is an autoimmune disease characterized by chronic intraepidermal blisters on normal to erythematous skin. It predominantly affects oral mucosa and causes blisters and erosions. Treatment involves topical and systemic corticosteroids and immunosuppressants.
3. Bullous pemphygoid also involves sub-epidermal blistering and is
1. The document discusses three types of chronic vesico bullous diseases: pemphigus vulgaris (epidermal blister), bullous pemphygoid (sub-epidermal blister), and dermatitis herpetiformis (infra-basal blister).
2. Pemphigus vulgaris is an autoimmune disease characterized by chronic intraepidermal blisters on normal to erythematous skin. It predominantly affects oral mucosa and causes blisters and erosions. Treatment involves topical and systemic corticosteroids and immunosuppressants.
3. Bullous pemphygoid also involves sub-epidermal blistering and is
CLASSIFICATION OF CVB 1. EPIDERMAL BLISTER Pemphigus vulgaris 2. SUB-EPIDERMAL BLISTER Bullous pemphygoid 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 Nikolskys sign Asboe-Hansens sign (spread phenomen) Histopathology : - acantholytic cells (Tzancks 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 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 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)