Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany.
Department of Dermatology, University of Marburg, Marburg, Germany.
J Invest Dermatol. 2018 Jan;138(1):32-37. doi: 10.1016/j.jid.2017.06.022. Epub 2017 Oct 14.
The autoimmune blistering skin disease pemphigus is caused by IgG autoantibodies against desmosomal cadherins, but the precise mechanisms are in part a matter of controversial discussions. This review focuses on the currently existing models of the disease and highlights the relevance of desmoglein-specific versus nondesmoglein autoantibodies, the contribution of nonautoantibody factors, and the mechanisms leading to cell dissociation and blister formation in response to autoantibody binding. As the review brings together the majority of laboratories currently working on pemphigus pathogenesis, it aims to serve as a solid basis for further investigations for the entire field.
自身免疫性水疱性皮肤病天疱疮是由针对桥粒钙黏蛋白的 IgG 自身抗体引起的,但确切的发病机制在一定程度上存在争议。本综述重点介绍了目前存在的疾病模型,并强调了桥粒芯糖蛋白特异性与非桥粒芯糖蛋白自身抗体、非抗体因素的贡献,以及针对自身抗体结合导致细胞解离和水疱形成的机制的相关性。由于该综述汇集了目前从事天疱疮发病机制研究的大多数实验室的研究成果,因此旨在为整个领域的进一步研究提供坚实的基础。