皮肤僵硬综合征1例并文献复习

A Case of Stiff Skin Syndrome and Literature Review

患儿女,2岁8个月,左躯干、双下肢硬化性褐色斑1年余,毳毛轻度增多。皮损组织病理示:真皮内胶原增生,胶原束间黏蛋白沉积。诊断:皮肤僵硬综合征。本病尚无特效治疗,应尽早康复锻炼。

A 2-year-and-8-month old girl presented with rock-hard skin, mild overlying hypertrichosis and hyperpigmentation on her left trunk and bilateral thigh for more than one year.A skin biopsy specimen showed an increase in collagen fibers in the dermis with mucopolysaccharide deposition.Diagnosis: skin stiff syndrome.No effective therapy has yet been found.Rehabilitation exercises should start as early as possible.