Background

Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker.

Objectives

To assess the association between pigmentation disorders and systemic involvement in patients with SSc.

Methods

A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation.

Results

A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89).

Limitations

This was a single-center retrospective study of a cohort of patients with SSc.

Conclusion

Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.

中文翻译：

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皮肤色素沉着异常与系统性硬化症中数字溃疡的关联：239名患者的分析

背景

文献中很少描述系统性硬化症（SSc）中的皮肤色素沉着症。但是，它们可能是诊断和/或严重性标记。

目标

评估色素沉着症和SSc患者全身受累之间的关联。

方法

总共定义了5种皮肤色素沉着症的模式：弥漫性色素沉着过度；皮肤色素沉着。暴露在阳光下的地方色素沉着; 头部，颈部和/或胸部上部色素沉着不足; 肢端色素沉着; 和弥漫性色素减退。

结果

总共包括239名患者。88名患者（36.8％）患有皮肤色素沉着症，如下：分别在38.6％（n = 34）和27.3％（n = 24）的患者中出现弥漫性色素沉着过度和阳光照射区域色素沉着过多；10.2％的患者面部，颈部和/或胸部色素沉着不足（n = 9）；弥漫性色素减退的比例为12.5％（n = 11）；以及肢端色素沉着的比例为17％（n = 15）。 在单变量分析中，弥漫性色素沉着与弥漫性SSc（P  = .001），改良的Rodnan皮肤评分增加（P  = .001）和较短的Raynaud现象持续时间（P = .002）有关，而在多变量分析中则没有。此外，弥漫性色素沉着与数字溃疡相关（P = 0.005），通过多变量分析证实（赔率为2.96； 95％置信区间为1.28-6.89）。

局限性

这是对一群SSc患者的单中心回顾性研究。

结论

皮肤色素沉着症的筛查可能对管理SSc的患者可能有用，以识别那些具有发展为数字溃疡的高风险的人，这是SSc中血管受累的症状。