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Skin disease: a cardinal feature of systemic sclerosis

机译：皮肤疾病：全身性硬化症的主要特征

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Despite the heterogeneity of SSc, almost all patients have skin involvement. As such, skin manifestations are critical in the initial diagnosis of SSc and in the subsequent sub-classification into the different subsets of disease. The two principal subsets are lcSSc and dcSSc. The main difference between these two subsets is the speed of disease progression and the extent and severity of skin and visceral involvement; lcSSc has an insidious onset with skin involvement confined largely to the face and extremities. Whilst vascular manifestations of SSc such as pulmonary arterial hypertension are typically more common in lcSSc, patients in both subsets can develop ischaemic digital ulcers. In dcSSc, disease progression is very rapid, with skin thickening extending beyond the extremities and earlier, more widespread internal organ involvement. DcSSc is generally considered to be the more severe subset of the disease. Skin scores in SSc correlate inversely with survival and are considered a valuable marker of disease severity. Skin involvement is easily detectable and, using the modified Rodnan skin score, the degree of skin fibrosis can be quantified. As well as general management measures, a number of targeted therapies are commonly used for treatment of cutaneous manifestations of SSc. These include the intravenous prostanoid iloprost and the dual endothelin receptor antagonist bosentan, which is approved in Europe for the prevention of new digital ulcers.
机译：尽管SSc的异质性，几乎所有患者都涉及皮肤。因此，皮肤表现对于SSc的初始诊断和随后的亚分类为疾病的不同亚群至关重要。两个主要子集是lcSSc和dcSSc。这两个子集之间的主要区别是疾病进展的速度以及皮肤和内脏受累的程度和严重程度； lcSSc起病隐匿，主要受累于面部和四肢。虽然lcSSc中SSc的血管表现（例如肺动脉高压）通常更为常见，但是这两个亚组的患者都可以发展出缺血性数字溃疡。在dcSSc中，疾病进展非常迅速，皮肤增厚超出了四肢，更早地出现了更广泛的内部器官受累。 DcSSc通常被认为是疾病的更严重亚型。 SSc中的皮肤评分与生存率成反比，被认为是疾病严重程度的重要标志。皮肤受累很容易检测到，使用改良的Rodnan皮肤评分，可以量化皮肤纤维化程度。除一般管理措施外，许多靶向疗法通常用于治疗SSc的皮肤表现。这些药物包括静脉注射类前列腺素伊洛前列素和双重内皮素受体拮抗剂波生坦（bosentan），在欧洲已获批准用于预防新的数字溃疡。

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  • 来源
    《Rheumatology》 |2009年第3期|p.14-18|共5页
  • 作者

    T. Krieg1 and K. Takehara2;

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    1Department of Dermatology, University of Cologne, Cologne, Germany and 2Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.;

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