神经纤维瘤病  

A case of neurofibromatosis with congenital melanocytic nevi and pigmented hairy epidermal nevus

作　　者：罗颖[1] 陈柏嘉[2] 黄海艳[2] 杜娟[2] 张建中[2] 

机构地区：[1]广州军区武汉总医院皮肤性病科,武汉430070 [2]北京大学人民医院皮肤性病科,北京100044

出　　处：《临床皮肤科杂志》2013年第6期359-360,共2页Journal of Clinical Dermatology

摘　　要：报告1例伴有先天性色素痣和色素性毛表皮痣的神经纤维瘤病。患儿女,9岁。出生后躯干及臀部即有大片淡褐色斑疹,随年龄增长面积逐渐扩大,呈裤衩样分布。左臀部一鸽蛋大黑色斑块,腰背部深褐色皮损上可见粗密毛发。阴蒂及左侧大阴唇明显肥大柔软。左侧肛周一胡桃大肤色柔软包块,表面形成褶皱,其上可见小的颗粒状突起,基底部境界不清。患儿生长发育正常,智力无明显异常。腰背部皮肤组织病理示:符合神经纤维瘤病。临床诊断:伴有先天性色素痣和色素性毛表皮痣的神经纤维瘤病。A 9 year-old female patient presented with large areas of light brown macules on her trunk and buttocks since birth. The lesions enlarged with age and appeared in a short pants shape. There was a pigeon＇s egg-sized black plaque on her fight buttock. There was thick hair on the surface of dark brown lesion on her dorsal trunk. Her clitoris and right labium majus were soft hypertrophy. On the right side of anus there was a walnut-sized, soft, skin-colored mass with granular surface and unclear border. The patient＇s growth and intelligent development were normal. The pathologic results confirmed neurofibro- matosis. The final diagnosis was neurofibromatosis with congenital melanoeytic nevi and pigmented hairy epidermal nevi.

关 键 词：神经纤维瘤病 色素痣 先天性 毛表皮痣 色紊眭 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]