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[大疱性类天疱疮、原发性胆汁性肝硬化和白癜风:一种多发性自身免疫综合征?]

[Bullous pemphigoid, primary biliary cirrhosis and vitiligo: a multiple autoimmune syndrome?].

作者信息

Marcet Benjamin, Sibaud Vincent, Géniaux Marc, Taieb Alain

机构信息

Service de Dermatologie, Hôpital Saint-André, CHU de -Bordeaux, 33075 Bordeaux Cedex, France.

出版信息

Ann Med Interne (Paris). 2002 Sep;153(5):349-50.

Abstract

Bullous pemphigoid is the most frequent autoimmune blistering skin disease. There have been few reports of an association with primary biliary cirrhosis and vitiligo. We report the simultaneous occurrence of bullous pemphigoid and primary biliary cirrhosis in an 86-year-old patient who also suffered from vitiligo. Multiple autoimmune syndrome, proposed by Humbert and Dupond, can be divided into three groups based on preferential associations of autoimmune disorders. The association of bullous pemphigoid, cirrhosis biliary primary and vitiligo has been reported three times in the literature. This association is probably not fortuitous and suggests a pathogenic relationship. This association is not typical of the multiple autoimmune syndrome as defined by Humbert and Dupond but the collection of such observations may contribute to revise the classification of autoimmune disease and provide a better understanding of the pathophysiological mechanisms of autoimmunity.

摘要

大疱性类天疱疮是最常见的自身免疫性水疱性皮肤病。关于其与原发性胆汁性肝硬化和白癜风相关联的报道较少。我们报告了一名86岁同时患有大疱性类天疱疮、原发性胆汁性肝硬化和白癜风的患者。Humbert和Dupond提出的多发性自身免疫综合征可根据自身免疫性疾病的优先关联分为三组。大疱性类天疱疮、原发性胆汁性肝硬化和白癜风的关联在文献中已有三次报道。这种关联可能并非偶然,提示存在致病关系。这种关联并非Humbert和Dupond所定义的多发性自身免疫综合征的典型情况,但收集此类观察结果可能有助于修订自身免疫性疾病的分类,并更好地理解自身免疫的病理生理机制。

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