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Abstract：
Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud\'s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.
摘要：
硬皮病是指自身免疫性结缔组织纤维化疾病，包括三个不同的子集：局部硬皮病，局限性皮肤系统性硬化症，和弥漫性皮肤系统性硬化症，具有不同的器官受累模式，自身抗体谱，管理,和预后影响。虽然系统性硬化症被认为是导致皮肤硬化症的疾病原型，还有许多其他条件可以模仿和与SSc混淆。它们可以分为免疫介导的/炎症，免疫介导的/炎症异常沉积（粘液病），遗传,药物诱导和毒性，新陈代谢，脂膜炎/血管性,根据临床病理和病理相关性和(para)肿瘤性疾病。本文回顾了以皮肤病为重点的临床表现，病因，诊断,以及可用于不同形式的硬皮病和硬皮病样疾病的治疗选择，包括巩膜水肿,scleedema,肾源性系统性纤维化，嗜酸细胞性筋膜炎,慢性移植物抗宿主病，皮肤卟啉病,糖尿病僵硬手综合征（糖尿病性滑膜病），和其他次要形式。后一组条件，也被称为硬皮病模仿者，硬皮病,或者假硬皮病,分享皮肤增厚的共同点，但表现出明显的皮肤表现，皮肤组织学,以及系统性影响或疾病关联，区分每个实体与其他实体和硬皮病。缺乏雷诺现象，毛细血管镜异常,或硬皮病特异性自身抗体也是重要的诊断线索。由于皮肤受累是最早的，硬皮病和硬皮病的最常见和特征性表现，皮肤科医生通常是一线医生，他们必须能够及时识别皮肤症状，为受影响的患者提供正确的诊断和适当的管理。
