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皮肤型皮肌炎的初始处理

Author
Ruth Ann Vleugels, MD, MPH
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD
Translators
黄怀球, 主任医师

引言

经典皮肌炎(dermatomyositis, DM)是一种特发性炎症性肌病，最常见的表现为进展性、对称性近端肌无力和一组特征性的皮肤表现。在没有可检测出的肌肉疾病的情况下，也可能出现皮肌炎皮肤表现，并且该表现可在成功治疗皮肌炎相关肌炎后仍持续存在。

皮肌炎患者常表现为头皮、面部、上躯干和上肢出现融合的紫红斑伴严重瘙痒。瘙痒病可导致患者失能。

皮肌炎的皮损往往对单独的光防护和局部治疗无反应，患者必须开始使用抗疟药物和/或甲氨蝶呤。这些干预措施均无效的患者可能需要更积极的免疫抑制或免疫调节治疗。

本文将讨论皮肌炎皮肤表现的初始治疗。难治性皮肌炎的治疗以及皮肌炎和幼年型皮肌炎的临床表现、诊断和皮肤外表现的治疗见其他专题。 (参见“难治性皮肤型皮肌炎的治疗”和“成人皮肌炎与多发性肌炎的临床表现”和“成人皮肌炎和多发性肌炎的诊断及鉴别诊断”和“成人皮肌炎和多发性肌炎的初始治疗”和“成人复发性及难治性皮肌炎和多发性肌炎的治疗”和“幼年型皮肌炎和多发性肌炎的发病机制和临床表现”和“幼年型皮肌炎和多发性肌炎的治疗、并发症和预后”)

临床特点概述

皮肌炎的相关皮肤改变包括一些具有诊断意义的表现，如Gottron丘疹(掌指和指间关节表面的紫罗兰色丘疹)和Gottron征[其他关节(如肘关节或膝关节)表面的紫罗兰色斑疹](图片 1A-D)。累及眶周皮肤(向阳性皮疹)、头皮、颈部的V区、肩部、上肢伸面、上胸部和上背部的紫红斑均是皮肌炎的其他特征性表现(图片 2A-D)。可能会存在鳞屑，但鳞屑通常在头皮上较为显著，并可能伴有弥漫性脱发(图片 2E)。

                                

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Literature review current through: 2017-06 . | This topic last updated: 2016-09-06.
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