CHRONIC VESICO BULLOUS

BY : Dr. Endang Sutedja, dr, SpKK(K)

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
-

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

Pemphigus vulgaris

Flaccid bullae

At mucous membrane

Flaccid bullae easily ruptur

Pemphigus foliaceus

Pemphigus erythematous

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

DERMATITIS HERPETIFORMIS
(Duhrings 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