Not to be confused with Bullous Pemphigoid. Pemphigus is also often referred to as Pemphigus Vulgaris although technically this only refers to one particular type of pemphigus.
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:
- British Association of Dermatologists: Dermatology, a handbook for medical students & junior doctors – bad.org.uk
- dermnetnz.org