进行性肢端黑变病

Acromelanosis progressiva:a case report

报告1例进行性肢端黑变病。患者男,15岁。双手(足)、指(趾)甲、面颊、耳郭出现褐色斑疹9年。皮肤科检查示双手(足)背侧及掌(跖)侧缘、面颊、耳郭、指(趾)甲弥漫分布暗褐色斑疹及斑片,双手(足)背关节处及耳郭皮肤轻度萎缩、变硬,手指间关节屈侧可见黑褐色色素性条纹,指(趾)甲有纵行的黑褐色色素线。4个月后手(足)背、掌(跖)侧缘、面颊及耳郭弥漫性暗褐色斑疹稍减退,耳郭呈现蛛网状线状褐色斑。组织病理检查示基底层黑素细胞增多,透明细胞呈簇状,黑素增加,真皮浅层见大量噬黑素细胞及黑素颗粒。

A 15-year-old boy with a 9-year history of brown macules located on the acral areas of the fingers and toes, nails, cheeks and external ears. Physical examination found dark brownish macules and patches on both palmoplantar side and back side of his hands and feet, cheeks, external ears, and nails. Slightly atrophic and indurated skin were seen in the dorsal side of interphalangeal joints of both hands and feet, and external ears. Black-brownish hyperpigmentation stripes were found at the flexor aspect of interphalangeal joints of the hands. There were brownish black longitudinal lines on finger and toe nails, Four months later, the dark brownish macules on his hands, feet, cheeks and external ears subsided slightly, leaving a brownish reticular patches on his external ears. Histopathology revealed proliferation of melanocytes in the basal layer of epidermis, cluster of clear ceils and increased melanin in the epidermis, and many melanophages and melanin granules in the dermis.