局部有疝囊样表现的皮肤僵硬综合征

Stiff skin syndrome with hernial sac-like lesion:a case report

报告1例皮肤僵硬综合征。患儿女,14岁。因双侧臀部皮肤变硬13年,腰背部皮肤变硬伴局部膨隆1年就诊。皮损呈淡褐色,表面毛发轻度增多,局部质硬如石,边界不清。腰背部皮损表面凸凹不平,局部呈疝囊样,质地较软,与正常皮肤一致。脊柱侧弯,弯腰受限。皮损组织病理检查示真皮增厚,胶原纤维增粗,胶原束间阿新蓝染色有阳性物质沉积。

A case of stiff skin syndrome is reported. A 14-year-old female presented with large brown induration on two sides of her buttocks for 13 years, which developed from the buttock to the back for 1 year. The lesions were rock-hard with mild hypertriehosis and no clear edge. The surface of the lesions on the back were uneven ,some lesions were hernial sac-like with a soft texture of normal skin.She had scoliosis,and was difficult in bending her body. Histologic examination showed that the thiekness of the dermis increased with collagen fibers thickened. Alcian blue staining was positive between the collagen fibers.