Overview
Definition
Severe autoimmune blistering disease of skin and mucous membranes.
Epidemiology
Varies globally depending on genetics, environment, or exposure.
Etiology & Risk Factors
- IgG autoantibodies against desmoglein 1 and 3 (desmosomes).
Clinical Symptoms
- Flaccid blisters that rupture easily
- painful oral ulcers
- positive Nikolsky sign.
Clinical Approach
Diagnosis
- Skin biopsy with direct immunofluorescence (net-like intraepidermal IgG).
Management
- High-dose Corticosteroids
- Rituximab
- Azathioprine.
Complications
- Fatal if untreated due to massive fluid loss and sepsis.