Abstract
The blistering skin disease bullous pemphigoid (BP) is an example of how autoantibodies from patients can be used as tools to improve our knowledge of the molecular structure of normal skin. BP autoantibodies bind to the basement membrane zone of normal human skin and usually react with two main epidermal antigens of approximately 230-kDa and 180-kDa. From recent studies, it is now established that the 180-kDa polypeptide is distinct from the 230-kDa BP antigen and is a major component of hemidesmosome. In this article, the recent data regarding the localization and structure of the epidermal 180-kDa BP antigen are reviewed in detail, together with its clinical significance, since it appears to be also a target antigen for pemphigoid (herpes) gestationis and, perhaps, cicatricial pemphigoid autoantibodies.
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