Abstract

Bullous pemphigoid (BP) is the most common autoimmune blistering disease characterized by chronic subepidermal blistering, autoantibodies directed against hemidesmosomal components, and a predominant eosinophil infiltrate. BP autoantibodies recognize two hemidesmosomal proteins of basal keratinocytes: BP230 (also termed BPAG1) and BP180 (also termed BPAG2 or collagen XVII) with the NC16A domain of the human BP180 antigen containing the major autoantibody-reactive epitopes. Anti-BP180 autoantibodies belong to IgG and IgE isotypes.

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