Bullous pemphigoid

Bullous pemphigoid 

Updated: 02/09/2021

© Jun Wang, MD, PhD

General features
  • Subepidermal, nonacantholytic, autoimmune disease
  • More common in elder population
  • Most patients need long term treatment
Pathogenesis
  • IgG antibodies to hemidesmosomal proteins BP230  (BPAG1) and BP180 (BPAG 2)
Clinical features
  • Large tense pruritic skin blisters
  • Rarely involves oral or ocular mucosa
  • Bullae don't rupture easily, and heal without scarring unless infected
  • High level IgG1 and IgG4 against BP180 (BPAG 2) 16A domain indicate worse prognosis
Pathological features
Immunofluorescence feature
Management
  • Corticosteroid
  • Immunosuppressant
  • Treatment of infection


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