皮肤僵硬综合征21例分析

Clinicopathological analysis of 21 patients with stiff skin syndrome

目的 探讨皮肤僵硬综合征患者的临床及组织病理特征.方法 回顾分析21例皮肤僵硬综合征患者临床及组织病理特点.结果 21例中男7例,女14例,平均发病年龄（5.2±4.2）岁.发病部位包括臀股部、腰部、肩背部,表现为皮肤硬化、局部多毛、色素沉着,部分患者出现关节活动受限.组织病理表现为真皮内胶原纤维增多增粗,无炎症细胞浸润.结论 皮肤僵硬综合征多发生于出生时或者儿童早期,以皮肤硬化、毛发增多、关节活动受限为最常见的临床表现.

Objective To assess the clinicopathological characteristics of stiff skin syndrome.Methods The clinical and histopathological manifestations of 21 patients with stiff skin syndrome were retrospectively studied.Results There were 7 males and 14 females among the 21 patients.The average age at onset was 5.2 ± 4.2 years.Buttocks,thighs,waist,shoulders and back were the most frequently involved sites in these patients.Clinical manifestations mainly included skin stiffness,localized hypertrichosis and pigmentation.Limited joint mobility was also observed in some patients.Histological examination showed an increase in the number and diameter of collagen fibers in the dermis without infiltration of inflammatory cells.Conclusions Stiff skin syndrome often occurs at birth or in early childhood with skin stiffness,limited joint mobility,and hypertrichosis as the most common clinical manifestations.