术语“皮肤硬化性疾病”包括特定的皮肤病学实体,有共同的皮肤纤维化变化。根据皮肤和皮外受累,这些疾病大多表现为不同的临床亚型,有时很难区分。本指南侧重于特征性的临床和组织病理学特征,诊断评分和血清自身抗体对鉴别诊断最有用。此外,详细介绍了硬化性皮肤病一线和高级治疗的当前策略。本指南的第1部分为临床医生提供了局限性硬皮病(硬皮病)的诊断和治疗概述。和系统性硬化症,包括系统性硬化症与风湿病谱疾病的重叠综合征。
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 1 of this
guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.