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Old lines tell new tales: Blaschko linear lupus erythematosis

机译:旧的故事讲述新的故事:Blaschko线性红斑狼疮

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摘要

Patients with lupus erythematosus (LE) specific cutaneous manifestations distributed along the lines of Blaschko are seldom encountered in clinic. In this paper, we reported 5 new cases and perform a systematic review of all the published cases in the English and Chinese literature. We provide a comprehensive summary of the clinical manifestations and explore potential etiology and pathogenesis. Sixty-eight published papers (53 English and 15 Chinese) and 93 cases (including 5 cases reported by us) were included in the analysis. In contrast to classical cutaneous LE, these cases have a series of distinctive clinical features and also have a potentially unique pathogenesis. The pathogenesis of this condition may involve the presence of mosaic abnormal skin cells, possessing crucial pathogenic gene(s)/epigenetic modification(s), located along the lines of Blaschko during embryogenesis. A comparison of the mosaic cells and surrounding "wild-type" cells and in-depth exploration of the interaction between susceptive gene(s)/epigenetic modification(s) and the immune system may potentially bring to light the secrets hidden in LE. Thus, we consider this condition to be a distinct subtype of LE and "Blaschko-linear lupus erythematosus" would be a more appropriate designation. The clues provided in the study of the pathogenesis of this form of LE may potentially unlock the mysteries of the various forms of LE as a whole. (C) 2015 Elsevier B.V. All rights reserved.
机译:在临床上很少遇到沿Blaschko线分布的红斑狼疮(LE)特定皮肤表现的患者。在本文中,我们报告了5个新病例,并对中英文文献中所有已发表的病例进行了系统的综述。我们提供了临床表现的综合摘要,并探讨了潜在的病因和发病机理。分析包括68篇发表的论文(53篇英文和15篇中文)和93例(包括我们报告的5例)。与经典的皮肤LE相比,这些病例具有一系列独特的临床特征,并且还具有潜在的独特发病机理。此病的发病机理可能涉及镶嵌异常的皮肤细胞的存在,其具有重要的致病基因/表观遗传修饰,在胚胎发生过程中沿着布拉什科谱系定位。镶嵌细胞与周围的“野生型”细胞的比较以及敏感性基因/表观遗传修饰与免疫系统之间相互作用的深入探索可能会揭示LE中隐藏的秘密。因此,我们认为这种情况是LE的独特亚型,“ Blaschko-线性红斑狼疮”将是一个更合适的名称。在研究这种形式的LE的发病机理中提供的线索可能从整体上揭示各种形式的LE的奥秘。 (C)2015 Elsevier B.V.保留所有权利。

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