Pemphigus Vulgaris
Pemphigus Vulgaris is a blistering skin disorder that is common in middle age (40-60 years). It has a high prevalence in the Jewish population. It is a lifelong condition. It often has mucosal involvement (>90%) and can be a potentially life-threatening condition (rare, especially if it is paraneoplastic)
- Pathophysiology
- IgG autoantibodies against desmoglein on keratinocytes → intra-epidermal split in the skin
- Signs and symptoms
- Thin-roofed, and flaccid (superficial) blisters that easily rupture to form erosions and crusts
- Affects mucosal areas (may precede skin involvement)
- Lesions are painful (but not pruritic)
- Nikolsky sign (slight rubbing exfoliates the outermost layer of the skin; this is not present in Bullous Pemphigoid)
- No prodromal symptoms
- Investigations
- Skin biopsy: Acantholysis (loss of cohesion between keratinocytes)
- Treatment
- Wound dressing when required
- Monitor for signs of infection
- Good oral care if the oral mucosa is involved
- Oral therapy: high-dose oral steroids, immunosuppressive agents (mycophenolate mofetil, azathioprine, methotrexate, cyclophosphamide, etc.)
- Plasmapheresis in severe cases
