EMed

Pemphigoid

Background

  • Commonest autoimmune sub-epidermal blistering disorder
  • Auto-antibodies vs “adhesion complexes” in skin’s basement membrane = blister
  • High relapse rate

Clinical

  • Itchy +, tense fluid filled blisters skin ± mucous memb
  • Usually limbs, groin, and abdomen
  • Older patients
  • Beware septicaemia (esp. if immunocompromised)
Bullous Pemphigoid - From BMJ 2013

Differential Dx

  • Bullous pemphigoid
  • Linear IgA disease
  • Epidermolysis bullosa acquisita
  • Bullous drug reaction

Investigations

  • Clinical diagnosis
  • Confirmatory biopsy ± immunofluorescence
  • ↑CRP & ↑ESR

Management

  • Potent topical steroids (clobetasol propionate)
  • Oral steroids or Dapsone (esp. for mucous membrane disease)
  • Tetracyclines ± nicotinamide in milder cases
  • Immunosuppressants (MTX / Azathioprine) in severe cases
  • Regular skin antiseptic soaks. and protective non-adherent dressings

Content by Dr Íomhar O' Sullivan. Last review Dr ÍOS7/01/23.