- In middle-aged, elderly
- Deadly without treatment
- Pathomechanism
- Autoantibodies against desmosomes
- Acantholysis – loss of cell-cell adhesion
- Clinical features
- Painful, flaccid, intraepidermal (superficial) blisters
- -> lesions rupture
- -> erosions and crusts
- -> re-epithelialization with hyperpigmentation but no scarring
- No pruritus
- Lesions often begin in oral mucosa then to body parts exposed to pressure
- Diagnosis
- Positive Nikolsky – Skin comes off with scraping
- Histology and immunofluorescence
- Treatment
- High-dose prednisone (1-2 mg/bwkg)
- Azathioprine, other immunosuppressants