硬皮病鉴别诊断.pdfVIP

硬皮病鉴别诊断* 梁敏锐 1,2 姜智星 1,2 邹和建 1,2 黄琼 3** 1. 2. （ 复旦大学附属华山医院风湿免疫科 ； 复旦大学风湿、免疫、过敏性疾病研究中心 ； 3. 复旦大学附属华山医院皮肤科 上海 200040 ） 摘 要 硬皮病（系统性硬化症）是一种少见的多脏器受累的系统性自身免疫性疾病，以皮肤纤维化为特征表现。 许多疾病临床上也可表现为皮肤变硬和组织纤维化，易与硬皮病混淆。本文强调皮肤病理对硬皮病鉴别诊断的重要性， 综述了成人硬肿病、硬化性黏液水肿、嗜酸性筋膜炎、慢性移植物抗宿主病、肾源性系统性纤维化、硬化萎缩性苔藓和 僵硬皮肤综合征等硬皮病类似疾病的临床表现、病因、病理形态和治疗方法。 关键词 硬皮病 系统性硬化症 鉴别诊断 R593.25 A 1006-1533(2017)S1-0012-05 中图分类号 ： 文献标识码 ： 文章编号 ： * Differential Diagnosis of Scleroderma LIANG Minrui 1,2 , JIANG Zhixing 1,2, ZOU Hejian 1,2, HUANG Qiong3** (1. Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai 200040, China; 2. Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China; 3. Department of Dermatology, Huashan Hospital, Fudan University, Shanghai 200040, China) ABSTRACT Scleroderma (systemic sclerosis, SSc) is a rare systemic autoimmune disease with multiple organ manifestations, characterizing of skin fibrosis. Many conditions presenting with clinical hardening skin and tissue fibrosis can be confused with scleroderma. We emphasize the distinct morphological difference between scleroderma and other conditions. This article reviews the clinical presentation, etiology, morphology, and treatment options available for scleroderma mimics, including scleredema adultorum, scleromyxedema, eosinophilic fasciitis (EF), chronic graft-versus-host disease (cGVHD), nephrogenic systemic fibrosis (NSF), liche