皮肤僵硬综合征1例并文献复习

A Case of Stiff Skin Syndrome and Literature Review

患儿男,13岁。2岁时发现右股内侧的皮肤僵硬伴凹陷,5岁时发现右腰部的皮肤僵硬伴凹陷,僵硬部位的皮肤毳毛增多、增粗。皮损组织病理示:真皮层未见明显炎症细胞浸润,深层胶原纤维组织增生增粗,胶原纤维组织间可见脂肪组织。诊断:皮肤僵硬综合征(SSS)。

A 13-year-old boy was first noted with stony hard skin on the medial site of the right thigh at two years old. At the age of five, the skin of the right waist was stiff and depressed with excess and thick vellus hairs on the stiff skin. Histological examination showed no inflammatory infiltration in the dermis. Thickening and proliferation of collagen fibers were observed in the lower dermis. Adipose tissue was present between collagen fibers. The diagnosis of stiff skin syndrome (SSS) was made.