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Resolution in bullous pemphigoid.
Seminars in Immunopathology ( IF 9 ) Pub Date : 2019-11-15 , DOI: 10.1007/s00281-019-00759-y
Christian D Sadik 1 , Enno Schmidt 1, 2
Affiliation  

Pemphigoid diseases are a group of autoimmune blistering skin diseases defined by an immune response against certain components of the dermal-epidermal adhesion complex. They are prototypical, autoantibody-driven, organ-specific diseases with the emergence of inflammatory skin lesions dependent on the recruitment of immune cells, particularly granulocytes, into the skin. During an acute flare of disease, inflammatory skin lesions typically progressing from erythema through urticarial plaques to subepidermal blisters erosions erupt and, finally, completely resolve, thus illustrating that resolution of inflammation is continuously executed in pemphigoid disease patients and can be directly monitored on the skin. Despite these superb conditions for examining resolution in pemphigoid diseases as paradigm diseases for antibody-induced tissue inflammation, the mechanisms of resolution in pemphigoid are underinvestigated and still largely elusive. In the last decade, mouse models for pemphigoid diseases were developed, which have been instrumental to identify several key pathways for the initiation of inflammation in these diseases. More recently, also protective pathways, specifically IL-10 and C5aR2 signalling on the molecular level and Tregs on the cellular level, counteracting skin inflammation have been highlighted and may contribute to the continuous execution of resolution in pemphigoid diseases. The upstream orchestrators of this process are currently under investigation. Pemphigoid disease patients, particularly bullous pemphigoid patients, who are predominantly above 75 years of age, often succumb to the side effects of the immunosuppressive therapeutics nowadays still required to suppress the disease. Pemphigoid disease patients may therefore represent a group of patients benefiting most substantially from the introduction of non-immunosuppressive, proresolving therapeutics into the treatment regimens for their disease.

中文翻译:

解决大疱性天疱疮。

天疱疮性疾病是一组自身免疫性水疱性皮肤疾病,其定义为对皮肤-表皮粘附复合物某些成分的免疫反应。它们是典型的,自身抗体驱动的,器官特异性疾病,并伴有炎症性皮肤病变的出现,这取决于免疫细胞(尤其是粒细胞)在皮肤中的募集。在疾病的急性发作期间,炎症性皮肤病变通常从红斑通过荨麻疹斑块发展到表皮下水疱侵蚀,并最终完全消退,从而说明在类天疱疮患者中炎症的解决是连续进行的,并且可以在皮肤上直接监测。尽管在检查作为抗体诱导的组织炎症的范式疾病的类天疱疮疾病中的分辨率方面有这些极好的条件,但对类天疱疮中分辨率的机制仍未进行充分的研究,并且在很大程度上尚不清楚。在过去的十年中,开发出了针对类天疱疮疾病的小鼠模型,这些模型已被用于识别引发这些疾病中炎症的几种关键途径。最近,还有保护途径,特别是分子水平和T上的IL-10和C5aR2信号传导在细胞水平上的调节,抵抗皮肤炎症已经被强调,并且可能有助于在类天疱疮疾病中持续执行解决方案。目前正在研究此过程的上游协调器。天疱疮样疾病的患者,尤其是主要在75岁以上的大疱天疱疮样的患者,经常屈服于当今仍需要抑制该疾病的免疫抑制疗法的副作用。因此,类天疱疮疾病患者可以代表一组患者,这些患者将从将非免疫抑制的,能解决疾病的治疗方法引入其疾病的治疗方案中而受益最大。
更新日期:2019-11-15
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