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天疱疮:一种复杂的依赖T细胞的自身免疫性疾病,可导致棘层松解。

Pemphigus: a complex T cell-dependent autoimmune disorder leading to acantholysis.

作者信息

Veldman Christian, Feliciani Claudio

机构信息

Department of Dermatology and Allergology, Philipps University, Marburg, Germany.

出版信息

Clin Rev Allergy Immunol. 2008 Jun;34(3):313-20. doi: 10.1007/s12016-007-8045-y.

Abstract

Pemphigus is a relatively rare autoimmune bullous disorder involving the skin and mucous epithelia. Clinically characterized by blisters and erosions, its histological hallmark is acantholysis induced by IgG antibodies (ab) against desmoglein 3 and/or desmoglein 1. The role of ab alone in inducing acantholysis is still a matter of debate as several mechanisms could be involved. Another intriguing area of research is the trigger factor inducing autoimmunity in pemphigus patients and the role of T and B cells in this process. This paper will review the data related to the mechanisms of acantholysis between keratinocytes and the role of T cell in this phenomenon.

摘要

天疱疮是一种相对罕见的自身免疫性大疱性疾病,累及皮肤和黏膜上皮。临床上以水疱和糜烂为特征,其组织学特征是由针对桥粒芯糖蛋白3和/或桥粒芯糖蛋白1的IgG抗体诱导的棘层松解。仅抗体在诱导棘层松解中的作用仍存在争议,因为可能涉及多种机制。另一个有趣的研究领域是天疱疮患者自身免疫的触发因素以及T细胞和B细胞在此过程中的作用。本文将综述与角质形成细胞间棘层松解机制以及T细胞在此现象中的作用相关的数据。

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