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Chapter 31 – Pemphigoid Group

Luca Borradori,
Philippe Bernard

BULLOUS PEMPHIGOID

Synonym:
      Pemphigoid

Key features

      Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease, and it predominantly affects the elderly
      It is usually a chronic disease, with spontaneous exacerbations and remissions, which may be accompanied by significant morbidity
      BP is associated with tissue-bound and circulating autoantibodies directed against the BP antigen 180 (BP180, BPAG2 or type XVII collagen) and the BP antigen 230 (BP230 or BPAG1), components of junctional adhesion complexes called hemidesmosomes that promote dermo-epidermal cohesion
      The spectrum of clinical presentations is extremely broad. Characteristically, BP is an intensely pruritic eruption with widespread blister formation. In early stages, or in atypical variants of the disease, only excoriated, eczematous or urticarial lesions (either localized or generalized) are present
      Diagnosis relies on immunopathologic examinations, …