Aetiology

  •  Middle aged (40-60yrs)
  •  High prevalence in Jewish regions
  •  Lifelong condition
 

Pathophysiology

  • Autoimmune, epidermal blistering due to IgG  autoantibodies for keratinocyte surfaces (desmoglein)
  • Often mucosal involvement (>90%)
  • Potentially life threatening (rare) if it is paraneoplastic
 

Signs & Symptoms

  • <50% present with oral lesions
  • Thin roofed & flaccid blisters (superficial) that are easily ruptured
  • Uusally No Prodromal Symptoms
  • Affected skin painful put not pruritic
  • Nikolsky Sign – slight rubbing exfoliates the outermost layer of skin (not present in pemphigoid)
 

Management

Acantholysis seen on biopsy (loss of cohesion between keratinocytes)
  • Oral steroids
  • Plasmapharesis in severe cases 

 

References:

 

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