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Autoimmune bullous skin diseases, pemphigus and pemphigoid
Journal of Allergy and Clinical Immunology ( IF 11.4 ) Pub Date : 2020-04-06 , DOI: 10.1016/j.jaci.2020.02.013
Shohei Egami , Jun Yamagami , Masayuki Amagai

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.



中文翻译:

自身免疫性大疱性皮肤病,天疱疮和天疱疮

自身免疫性大疱性皮肤病,例如天疱疮和天疱疮,可能有助于弄清皮肤中免疫调节的机制。天疱疮和天疱疮分别由基本上针对细胞-细胞连接处的桥粒结构蛋白和表皮-真皮连接处的半桥粒结构蛋白的IgG自身抗体介导,其特征在于皮肤和/或粘膜的水泡和糜烂。在过去的30年中,经过深入的研究,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来帮助我们了解其病理生理学。基于这些进展,已经有了几种新的治疗方法,并且正在开发更有效和毒性更低的靶向方法。

更新日期:2020-04-21
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