Department of Dermatology, Keio University School of Medicine, Tokyo, Japan; Laboratory for Skin Homeostasis, RIKEN Center for Integrative Medical Science, Kanagawa, Japan.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
J Allergy Clin Immunol. 2020 Apr;145(4):1031-1047. doi: 10.1016/j.jaci.2020.02.013.
Autoimmune bullous skin diseases, such as pemphigus and pemphigoid, may enable clarification of the mechanisms of immune regulation in the skin. Pemphigus and pemphigoid are mediated by essentially IgG autoantibodies against structural proteins of the desmosomes at cell-cell junctions and hemidesmosomes at epidermal-dermal junctions, respectively, and are characterized by blisters and erosions in the skin and/or mucous membranes. Intensive investigation over the last 3 decades has identified their target antigens and developed serological diagnostic tools as well as mouse models to help us understand their pathophysiology. Based on these advances, several new therapeutic approaches have become available, and more effective and less toxic targeted approaches are under development.
自身免疫性大疱性皮肤病，如天疱疮和类天疱疮，可能有助于阐明皮肤中免疫调节的机制。天疱疮和类天疱疮分别由针对细胞间连接的桥粒和表皮-真皮连接的半桥粒的结构蛋白的本质上是 IgG 的自身抗体介导，其特征是皮肤和/或粘膜出现水疱和糜烂。在过去的 30 年中，经过深入研究已经确定了它们的靶抗原，并开发了血清学诊断工具以及小鼠模型，以帮助我们了解其病理生理学。基于这些进展，已经出现了几种新的治疗方法，并且正在开发更有效和毒性更小的靶向方法。
