节段性皮肤僵硬综合征一例

Segmental stiff skin syndrome:a case report

患儿,男,8岁。因右侧股部、臀部及腰部皮肤触之僵硬8年就诊。患者皮损呈单侧分布,伴有轻度多毛和色素沉着。病理活检示表皮过度角化,基底层色素增加,真皮中层胶原纤维增粗、致密,呈均质化,无炎症细胞浸润,附属器未见异常。阿新蓝染色呈阳性。综上诊断为节段性皮肤僵硬综合征。

A 8-year-old boy was seen for indurated skin of right thigh,buttock and waist for 8 years.The lesions were unilateral with mild hypertrichosis and pigmentation.Skin histology showed hyperkeratosis of the epidermis,increased pigment in the basal layer,and thickened dense collagen fibers in the middle dermis with no obvious inflammatory infiltrates and no obvious atrophy of appendages.Alcian blue stainingwas positive.The diagnosis of segmental stiff skin syndrome was made.