Pemphigus vulgaris

From greek.doctor
  • 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