婴儿弥漫性皮肤肥大细胞增生症  

A case of infantile diffuse cutaneous mastocytosis

作　　者：喻光莲 吴菁华 张婷婷[1] 刘彤云[1] YU Guanglian;WU Jinghua;ZHANG Tingting;LIU Tongyun(Department of Dermatology,the First Affiliated Hospital of Kunming Medical University,Kunming 650032,China)

机构地区：[1]昆明医科大学第一附属医院皮肤科,云南昆明650032

出　　处：《临床皮肤科杂志》2023年第11期671-673,共3页Journal of Clinical Dermatology

基　　金：云南省皮肤与免疫性疾病临床医学中心(ZX2019-03-02)。

摘　　要：报告1例弥漫性皮肤肥大细胞增生症。患儿女,10个月。躯干水疱及大疱5个月。皮肤科检查:全身皮肤呈橘黄色、纹理粗糙、颜色较暗,背部见水疱簇集性分布,尼氏征阴性。背部散在分布色素减退斑,色素减退区域摩擦后可见皮肤发红。额部、右侧颞部可见风团。背部皮损组织病理检查:表皮内陈旧性水疱,真皮内肥大细胞弥漫性浸润,肥大细胞呈圆形或梭形,细胞质丰富,呈嗜碱性。免疫组化:CD117(+),CD2及CD25为(-)。诊断:弥漫性皮肤肥大细胞增生症。A case of infantile diffuse cutaneous mastocytosis is reported.A 10-month-old female child presented with blisters and bullae on the trunk for five months.Physical examination showed that the whole body skin was orange and the texture was rough,the blister cluster distribution was seen on the back,and the Nikolsky’s sign was negative.Hypopigmented spots scattered on the back,and the hypopigmented areas showed redness on rubbing.The frontal and right temporal regions were covered with a windswept elevated skin lesion.Histopathological examination of the back showed old blisters in the epi dermis,diffuse infiltration of mast cells in the dermis,with a round or spindle shape and abundant basophilic cytoplasm.Immunohistochemical examination was positive for CD117 and negative for CD2 and CD25.A diagnosis of diffuse cutaneous mastocytosis was made.

关 键 词：肥大细胞增生症 弥漫性 婴儿 

分 类 号：R751[医药卫生—皮肤病学与性病学]