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表皮下自身免疫性大疱性皮肤病:概述、流行病学和相关性。

Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.

机构信息

Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.

出版信息

Immunol Res. 2018 Feb;66(1):6-17. doi: 10.1007/s12026-017-8975-2.

Abstract

Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Observational studies investigating comorbidities and associations among patients with these diseases are inconsistent and sometimes inconclusive. This review provides a brief overview regarding each one of the subepidermal autoimmune bullous diseases. In addition, it summarizes the most recent understanding of the epidemiological features and associations of this group of organ-specific autoimmune diseases.

摘要

皮肤和黏膜的皮下自身免疫性大疱性疾病包括一大组慢性疾病,包括大疱性类天疱疮、妊娠疱疹、黏膜类天疱疮、线状 IgA 大疱性皮病、获得性大疱性表皮松解症和抗 p200 天疱疮。这些疾病的特征是针对基底膜带结构成分的抗体反应,导致皮下水疱形成。这些疾病在世界不同地区的流行病学特征有很大差异。观察性研究调查这些疾病患者的合并症和关联结果不一致,有时甚至没有结论。本文简要概述了每一种皮下自身免疫性大疱性疾病,并总结了这组器官特异性自身免疫性疾病的最新流行病学特征和关联的理解。

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