Department of Dermatology, Medical University of Vienna, Vienna, Austria.
Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.
J Eur Acad Dermatol Venereol. 2017 Oct;31(10):1581-1594. doi: 10.1111/jdv.14466. Epub 2017 Aug 8.
The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy).
“皮肤硬化病”一词包括具有共同皮肤纤维化改变的特定皮肤科实体。这些疾病根据皮肤和皮肤外受累的不同，大多表现为不同的临床亚型，有时彼此之间难以区分。本指南重点介绍了特征性的临床和组织病理学特征、诊断评分以及对鉴别诊断最有用的血清自身抗体。此外，还详细介绍了硬化性皮肤病一线和晚期治疗的当前策略。本指南的第二部分为临床医生提供了硬肿病、硬皮病（Buschke）和肾源性系统性硬化症（肾源性纤维化皮肤病）的诊断和治疗概述。
