Bullous pemphigoid (BP) is an autoimmune disease associated with subepidermal blistering due to autoantibodies directed against BP180 and BP230. BP180 is currently considered as the major pathogenic autoantigen. However, previous clinical findings suggested that anti‐BP230 autoantibodies alone can cause skin lesions in animal models and many BP patients. The characteristics of BP230 and the pathogenic roles of anti‐BP230 antibodies have been proposed. First, at the molecular level, BP230 mediates the attachment of keratin intermediate filaments to the hemidesmosomal plaque and interacts with other constituents of hemidesmosomes. Second, the presence of BP230 autoantibodies may correlate with specific clinical features of BP. The immunoglobulin (Ig)G autoantibodies from BP patients react mainly against the C‐terminus of BP230, while the IgE autoantibodies are still inconclusive. Third, in vivo, autoantibodies against BP230 involved in the disease may not only induce the inflammatory response but also impair the structural stability of hemidesmosomes. This article reviews recently published work about the role of BP230 and its antibodies, including IgG and IgE, aiming to find clues of its clinical association and lay the foundation for the research on the pathogenicity of antibodies against BP230.
Bullous pemphigoid is an autoimmune disease characterized by pruritic urticarial erythema and tense blisters, affecting mainly elderly people. Histopathology reveals subepidermal blisters with eosinophilic infiltration, and direct immunofluorescence (IF) demonstrates deposits of IgG and/or C3 to the basement membrane zone (BMZ). 1 Autoantibodies in BP sera were against hemidesmosomal proteins BP180 and BP230, which are involved in anchoring basal keratinocytes in the epidermis to the dermis. 2 In addition to IgG autoantibodies, the relevance of IgE autoantibodies in BP pathogenicity has been indicated. 3 Elevated levels of circulating total IgE have been demonstrated in more than 70% of BP sera. 4 In vivo, 18%-41% of BP patients presented with deposition of IgE in the BMZ. 5,6 There is emerging evidence showing that IgE autoantibodies against BP antigens were detected in 21%-67% of
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