Abstract

Anti-p200 pemphigoid, initially described in 1996, is a subepidermal autoimmune blistering disease. It is manifested as tense blisters, mostly in an acral distribution, and is accompanied by mucosal involvement in more than half the cases. The disease is produced by circulating autoantibodies directed against the dermal antigen of 200-kDa, the exact identity of which remains unknown. This review focuses on the clinical features, immunopathogenesis, and diagnosis of anti-p200 pemphigoid.

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