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Pemphigus vulgaris

Revision as of 16:51, 17 July 2024 by Nikolas (talk | contribs) (Created page with "<section begin="dermatology" />* 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 * Dia...")
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  • 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