Pemphigoid diseases: Pathogenesis, diagnosis, and treatment

Pemphigoid diseases: Pathogenesis, diagnosis, and treatment
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DOI:
10.3109/08916934.2011.606447
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发表时间:
2012-02-01
期刊:
影响因子:
3.5
通讯作者:
Schmidt, Enno
Schmidt, Enno
中科院分区:
医学4区
文献类型:
--
作者:
Kasperkiewicz, Michael;Zillikens, Detlef;Schmidt, Enno

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类天疱疮疾病(包括大疱性类天疱疮、粘膜类天疱疮、妊娠类天疱疮、线性伊加皮肤病、扁平苔藓类天疱疮和抗p200类天疱疮)是自身免疫性大疱性皮肤病的一个亚组,其特征是针对半桥粒结构成分的自身抗体反应,导致表皮下起泡。通过使用不同的体外系统和实验动物模型,这些自身抗体的致病相关性已被证明。对自身抗体反应的理解的最新进展已经导致了针对这些疾病的新的诊断工具和更差异化的治疗方法。这篇综述涵盖了这组自身免疫性疾病的病理生理学,诊断和治疗的最新认识。
Pemphigoid diseases (including bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear IgA dermatosis, lichen planus pemphigoides, and anti-p200 pemphigoid) are a subgroup of autoimmune bullous skin diseases characterized by an autoantibody response toward structural components of the hemidesmosome resulting in subepidermal blistering. By the use of different in vitro systems and experimental animal models, the pathogenic relevance of these autoantibodies has been demonstrated. Recent advances in the understanding of autoantibody responses have led to novel diagnostic tools and a more differentiated therapeutic approach for these disorders. This review covers the most recent understanding of the pathophysiology, diagnosis, and treatment of this group of autoimmune diseases.