Pathophysiology in Pemphigus and Pemphigoid

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  • 天疱瘡と類天疱瘡
  • テン ホウソウ ト ルイテン ホウソウ

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Abstract

Recent rapid advances in basic reseach for various autoimmune bullous dermatoses have provided us with many insights into their pathomechanisms. In particular, a recently developed enzyme-linked immunosorbent assay (ELISA) for desmogleins 1 and 3 (Dsg1 and Dsg3), the antigens for pemphigus foliaceus (PF) and pemphigus vulgaris (PV), respectively, has provided great progress in the diagnosis and classification of pemphigus, as well as in the understanding of its pathomechanisms. The study of the anti-Dsg1 and Dsg3 antibodies indicated that there are two different types of PV; i.e., the mucosal dominant type and the mucocutaneous type. In addition, the desmoglein compensation theory, which has been proposed by Dr. Stanley and Dr. Amagai, can reasonably explain the different depths of skin lesions and the different occurrences of skin and oral mucosal lesions between PV and PF. In bullous pemphigoid (BP), the NC16a domain of BP180 has been shown to play an important role in blister formation, while the role of the other BP antigen, BP230, is not clear. Mucous membrane pemphigoid (MMP) is heterogeneous, and several distinct proteins have been shown as candidates. Most MMP cases react with BP180, particularly with its C-terminal domain. A lesser number of MMP cases react with laminin 5. Because the patients react with BP180 and laminin 5 can not be distinguished clinically, the epitopes in BP180 and laminin 5 are considered to be present at similar sites. Various autoimmune bullous diseases, including several types of pemphigus and pemphigoid, are the only diseases, in which the pathogenic roles of circulating autoantibodies have been confirmed by newborn mouse animal models. Therefore, such studies on the pathophysiology of pemphigus and pemphigoid should be extremely important as paradigms for the research into various types of autoimmune diseases in other fields.

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