Systemic sclerosis (SSc) is a severe disease whose pathophysiology remains partly unknown, combining autoimmune, vascular, and fibrotic features. Recently, we evidenced a link between vasculopathy and pigmentary changes in SSc. CCN3 (NOV) is a matricellular protein implicated in both angiogenesis and pigmentation regulation, in particular melanocyte adhesion to the basal layer. We decided to study CCN3 expression in SSc epidermis. We show that in SSc patients with pigmentary changes compared to patients with normal pigmentation, CCN3 is specifically downregulated in situ in melanocytes and upregulated in keratinocytes. Moreover, the number of melanocytes is significantly decreased in SSc patients with a disease duration of more than 5 years compared to the other patients. Altogether, our findings could provide new insights on the mechanisms of pigmentary changes in SSc patients, as well as treatment adaptation in a personalized manner.

中文翻译：

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具有色素变化的系统性硬化症患者表皮中 CCN3 (NOV) 表达的失调。

系统性硬化症 (SSc) 是一种严重的疾病，其病理生理学仍然部分未知，结合了自身免疫、血管和纤维化特征。最近，我们证明了 SSc 中血管病变和色素变化之间的联系。CCN3 (NOV) 是一种基质细胞蛋白，参与血管生成和色素沉着调节，特别是黑色素细胞与基底层的粘附。我们决定研究 SSc 表皮中的 CCN3 表达。我们表明，与正常色素沉着的患者相比，在有色素变化的 SSc 患者中，CCN3 在黑素细胞中原位特异性下调，在角质形成细胞中上调。此外，与其他患者相比，病程超过 5 年的 SSc 患者的黑色素细胞数量显着减少。共，