线状和回旋状色素沉着症并发炎性线状表皮痣及寻常性鱼鳞病

报告1例线状和回旋状色素沉着症并发炎性线状表皮痣、寻常性鱼鳞病。患者女,17岁。2岁起胸背部出现线状和回旋状色素沉着,双下肢皮损覆鱼鳞状鳞屑,双手背关节和左侧膝部、踝部、足背关节处可见线状鳞屑性红斑。系统及实验室检查均正常。结合组织病理改变诊断为线状和回旋状色素沉着症并发炎性线状表皮痣、寻常性鱼鳞病。

Linear and whorled nevoid hypermelanosis complicated with inflammatory linear epidermal nevus and ichthyosis vulgaris

A 17-year-old girl presented with linear and whorled melanosis following Blaschko's lines mainly on her trunk is reported. The skin lesions began when she was 2 years old. Ichthyosiform lesions and linear scaly erythema were observed respectively on her lower limbs and the dorsa of her hands, left knee, ankle and foot. No abnormality was found in systemic examination and blood tests. There was increased pigmentation within the basal keratinocytes, with focal incontinentia pigmenti. Notable lymphangiectasis could also be seen in the mid part of the dermis, which had never been described before. The diagnosis of linear and whorled nevoid hypermelanosis complicated with inflammatory linear epidermal nevus and ichthyosis vulgaris was made in accordance with clinical and pathological manifestations. It ought to be differentiated from incontientia pigmenti and hypomelanosis of Ito.