硬皮病样表现的皮肤病  

Skin diseases with scleroderma.like cutaneous manifestations

作　　者：耿怡[1] 徐秀莲[1] Geng Yi Xu Xiulian(Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China)

机构地区：[1]中国医学科学院北京协和医学院皮肤病研究所病理科,南京210042

出　　处：《国际皮肤性病学杂志》2017年第2期88-91,共4页International Journal of Dermatology and Venereology

摘　　要：系统性硬皮病被认为是造成皮肤硬化的主要病因，但临床上，仍有许多疾病可表现为皮肤硬化及组织纤维化，易与系统性硬皮病相混淆。这类疾病包括一组广谱的异质性疾病，从仅累及局部皮肤的良性病变至广泛系统受累甚至危及生命的严重病变。回顾这类皮肤纤维化疾病的主要类型，包括：肾源性纤维性皮病、硬化性黏液性水肿、POEMS综合征、骨髓瘤、Buschke硬肿症、嗜酸性筋膜炎、嗜酸性肌痛综合征、类癌综合征、迟发性皮肤卟啉病、慢性移植物抗宿主病、环境因素诱发的硬皮病样皮肤病等。通过区分这组疾病的发病机制、临床及病理表现、治疗方法，提高对其认识，对正确诊断该类疾病提供适当的治疗具有重要的临床意义。Systemic sclerosis is usually considered to be the main cause of cutaneous sclerosis, but many other diseases can also manifest as cutaneous sclerosis and tissue fibrosis in clinic, which are easy to be confused with systemic sclerosis. This kind of diseases include a wide spectrum of heterogeneous diseases, ranging from a benign disease with a localized cutaneous involvement, to a widespread systemic, life-threatening disease. This review summarizes several main kinds of cutaneous fibrosing disorders, such as nephrogenic fibrosing dermopathy, seleromyxedema, POEMS syndrome, myeloma, Buschke scleroderma, eosinophilic fasciitis, eosinophilia-myalgia syndrome, carcinoid syndrome, porphyria cutanea tarda, chronic graft- versus host disease, environmentally triggered scleroderma- llke disorders. Distinguishing the pathogenesis, clinical and pathological manifestations and therapeutic methods between this group of diseases will improve the understanding of these diseases, and has great clinical significance for their correct diagnosis and proper treatment.

关 键 词：硬皮病 系统性 病人模拟 病理学 诊断 治疗应用 

分 类 号：R593.25[医药卫生—内科学]