Department of Dermatology, Venereology, and Allergology, University of Lübeck, Lübeck, Germany; Lubeck Institute of Experimental Dermatology (LIED), University of Lubeck, Lubeck, Germany.
Dtsch Arztebl Int. 2021 Jun 18;118(24):413-420. doi: 10.3238/arztebl.m2021.0136.
Bullous autoimmune dermatoses are a clinically and immunopatho - logically heterogeneous group of diseases, characterized clinically by blisters or erosions of the skin and/or mucous membranes. In Germany, their prevalence is approximately 40 000 cases nationwide, and their incidence approximately 20 new cases per million people per year.
This review is based on publications that were retrieved by a selective search of the literature focusing on the current German and European guidelines.
Recent years have seen the publication of guidelines, controlled prospective clinical trials, and multicenter diagnostic studies improving both diagnosis and therapy. Specific monovalent and multivariate serological test systems and pattern analysis of tissue-bound autoantibodies allow identification of the target antigens in 80-90% of patients. This enables the precise classification of disease entities, with implications for treatment selection and disease outcome. In 2019, the anti-CD20 antibody rituximab was approved by the European Medicines Agency for the treatment of moderate and severe pemphigus vulgaris, with an ensuing marked improvement in the care of the affected patients. To treat mild and moderate bullous pemphigoid, topical clobetasol proprionate is recommended, in severe disease, combined with systemic treatment, i.e. usually (a) prednisolone p.o. at an initial dose of 0.5mg/kg/d , (b) an immunomodulant, e.g. dapsone or doxycycline, or (c) prednisolone plus an immunomodulant.
The early recognition and precise diagnostic evaluation of bullous autoimmune dermatoses now enables improved, often interdisciplinary treatment, in accordance with the available guidelines. Current research projects are focused on new treatment approaches, an improved understanding of the underlying pathophysiology, and further refinements of diagnostic techniques.
大疱性自身免疫性皮肤病是一组具有临床和免疫病理学异质性的疾病,其临床特征为皮肤和/或黏膜水疱或糜烂。在德国,全国范围内的患病率约为 40000 例,发病率约为每百万人每年 20 例新病例。
本综述基于通过选择性搜索文献检索到的出版物,重点关注当前德国和欧洲的指南。
近年来,发布了指南、对照前瞻性临床试验和多中心诊断研究,改善了诊断和治疗。特定的单价和多变量血清学检测系统以及组织结合自身抗体的模式分析可在 80-90%的患者中识别靶抗原。这使得疾病实体的精确分类成为可能,这对治疗选择和疾病结局具有重要意义。2019 年,抗 CD20 抗体利妥昔单抗被欧洲药品管理局批准用于治疗中重度寻常型天疱疮,随后显著改善了患者的治疗效果。治疗轻度和中度大疱性类天疱疮推荐使用局部卤倍他索丙酸酯,重度疾病则推荐联合全身治疗,即通常(a)口服泼尼松龙,初始剂量为 0.5mg/kg/d,(b)免疫调节剂,如氨苯砜或多西环素,或(c)泼尼松龙加免疫调节剂。
大疱性自身免疫性皮肤病的早期识别和精确诊断评估现在可以根据现有指南进行改善,通常是跨学科的治疗。目前的研究项目集中在新的治疗方法、对潜在病理生理学的更深入理解以及诊断技术的进一步改进上。