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病例报告:大疱性类天疱疮的多种表现。

Case Report: The many faces of bullous pemphigoid.

机构信息

Department of Dermatology, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany.

Deutsches Zentrum Immuntherapie (DZI), Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany.

出版信息

Front Immunol. 2023 Oct 12;14:1272742. doi: 10.3389/fimmu.2023.1272742. eCollection 2023.

Abstract

The pemphigoid group comprises a number of bullous skin diseases with autoantibodies against different constituents of the basement membrane zone that result in subepidermal detachment and clinically characteristic tense blisters, erosions, urticarial erythema, and itching. Apart from the most frequent type of bullous pemphigoid with antibodies against BP180, which is found predominantly in elderly patients, the disease may present at other ages and different pathogenic conditions. Here, four cases are presented of young age (3 months and 25, 34, and 46 years) and in association with vaccination, pregnancy, or metastatic cancer. Though anti-BP180 was found in all cases, a different pathogenic background may be found in any of them, resulting in characteristic clinical manifestation, yet demanding specifically adapted therapeutic approaches.

摘要

疱疹样类天疱疮组包含了多种大疱性皮肤病,这些疾病的自身抗体针对基底膜带的不同成分,导致表皮下水疱分离,临床上表现为紧张性水疱、糜烂、荨麻疹样红斑和瘙痒。除了最常见的、以抗 BP180 抗体为特征的大疱性类天疱疮(主要发生于老年患者),该疾病还可能发生在其他年龄段和不同的发病条件下。在此,我们报告了 4 例年轻患者(3 个月和 25、34、46 岁)的病例,他们与疫苗接种、妊娠或转移性癌症有关。尽管所有病例均发现了抗 BP180 抗体,但在任何一个病例中都可能存在不同的发病机制,导致特征性的临床表现,但需要采用特定的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a78/10601641/e1097728b3dc/fimmu-14-1272742-g001.jpg

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