Bullous Pemphigus
Print Friendly, PDF & Email

almostadoctor app banner for android and iOSalmostadoctor iPhone, iPad and android appsalmostadoctor iOS appalmostadoctor android app

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)
Pemphigus Vulgaris
Pemphigus Vulgaris. Image from Dermnet. Used in accordance with Creative Commons Attribution-NonCommercial-NoDerivs 3.0 (New Zealand) license.

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

 

Related Articles

Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) currently works as a GP Registrar and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

Leave a Reply