Pemphigus is an autoimmune disorder, known to be caused by autoantibodies directed against critical adhesion molecules of squamous epithelial cells, the desmogleins. These autoantibodies induce blistering of skin and mucosal surfaces and lead to severe morbidity and, potentially, death. Key factors include associated major histocompatibility complex class II genes, the structure of the desmoglein antigens, and the role of autoantibody in impairing cellular adhesion. This article discusses the precise structure of the major histocompatibility complex class II gene-peptide-T-cell receptor complex involved and of the environmental and genetic factors that induce autoimmunity against desmoglein 1. Discovery of antigen-specific immunotherapy and insight into environmental factors that initiate autoimmunity in genetically susceptible individuals are needed.
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