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[黏膜大疱性自身免疫性皮肤病]

[Bullous autoimmune dermatoses of the mucous membranes].

作者信息

Didona Dario, Hinterseher Julia, Eming Rüdiger

机构信息

Klinik für Dermatologie und Allergologie, Philipps-Universität Marburg, Marburg, Deutschland.

Klinik III Dermatologie, Venerologie und Allergologie, Bundeswehrzentralkrankenhaus Koblenz, Koblenz, Deutschland.

出版信息

Dermatologie (Heidelb). 2022 Sep;73(9):692-700. doi: 10.1007/s00105-022-05036-y. Epub 2022 Aug 25.

Abstract

Autoimmune bullous diseases (AIBD) comprise a group of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against adhesion molecules and structural proteins of skin and mucosae. Depending on the target protein, AIBD are classified into intraepidermal (pemphigus group) and subepidermal (pemphigoid group, epidermolysis bullosa acquisita, dermatitis herpetiformis) blistering disorders. Depending on the clinical entity, patients can develop blisters, pustules, erosions, and erythema on the skin and mucosae.

摘要

自身免疫性大疱性疾病(AIBD)是一组器官特异性自身免疫性疾病,其特征是产生针对皮肤和黏膜黏附分子及结构蛋白的自身抗体。根据靶蛋白不同,AIBD可分为表皮内(天疱疮组)和表皮下(类天疱疮组、获得性大疱性表皮松解症、疱疹样皮炎)水疱性疾病。根据临床症状,患者的皮肤和黏膜可出现水疱、脓疱、糜烂和红斑。

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