LAUSR

bullous pemphigoid (BP) is an autoimmune blistering skin disease that predominantly occurs in the elderly, often accompanied by neurological disorders and increased mortality. Diagnosis of BP is established by clinical presentation, typical histology with subepidermal blisters and an inflammatory infiltrate containing eosinophils, positive direct immunofluorescence (DIF) microscopy of perilesional skin and serological assays. Almost all patients show linear basement membrane zone (BMZ) deposits of complement component 3 (C3) and IgG in DIF microscopy, implying a critical role of complement for disease. In this regard, formation of the terminal membrane attack complex of complement can be observed in BP patients' epidermal basal cells, potentially resulting in direct cytotoxic effects, and the complement-activating capacity of BP autoantibodies ex vivo has been shown to be correlated to disease activity. This article is protected by copyright. All rights reserved.