Bullous Pemphigus

Question 1

Pathophysiology and aetiology of Bullous Pemphigus?

Answer 1

Autoimmune, epidermal blistering due to IgG autoantibodies for keratinocyte surfaces (desmoglein)

• often MUCOSAL involvement (>90%)
• potentially life threatening (rare) if it is paraneoplastic
• middle aged (40-60yrs)
• high prevalence in Jewish regions
• lifelong condition

aka Pemphigus Vulgaris

Question 2

Sign and symptoms of Bullous Pemphigus

Answer 2

• <50% present with oral lesions
• think roofed and flaccid blisters (superficial) that are easily ruptured
• usually no prodromal symptoms
• affected skin painful but not pruritic
• NIKOLSKY sign, slight rubbing exfoliates the outermost layer of skin (not present in pemphigoid)

Acantholysis seen on biopsy (loss of cohesion between keratinocytes)

Question 3

Management of Bullous Pemphigus?

Answer 3

• oral steroids (pred gradually reduced over 6 months)
• betamethasone mouth wash if oral disease
• plasmapheresis in severe cases (IVIG)
• steroid sparing: mycophenylate mofetil, azathioprine, IVIG, rituximab