Pemphigus vulgaris

From IDWiki

Background

  • Blistering skin condition of unknown etiology
  • Associated with autoantibodies targeting cadherins

Drug-Induced

  • Penicillamine and captopril
  • NSAIDs, penicillin, and cephalosporins
  • Possibly certain foods, though mostly anecdotal

Clinical Manifestations

  • Initially presents in oral mucosa in 80% of cases
  • Followed by cutaneous lesions
  • Lesions are bullae that rupture leaving painful erosions
  • Nikolsky sign may be present, where blisters form at sites of minor pressure or minor trauma

Diagnosis

  • Based on clinical, histopathological, and laboratory evidence
  • Skin biopsy is helpful
    • Direct immunofluorescence can show net-like or chicken-wire pattern of IgG on the epidermis
    • Tzanck smear shows acantholysis

Management

  • Systemic corticosteroids
  • Rituximab and other anti-CD20 monoclonal antibodies