幼儿色素血管性斑痣性错构瘤病合并Klippel-Trenaunay综合征1例及其皮肤影像学表现  

A case of phakomatosis pigmentovascularis complicated with Klippel-Trenaunay syndrome and its skin imaging findings in a child

作　　者：冯小燕[1] 刘欣欣[1] 李钦峰[1] Feng Xiaoyan;Liu Xinxin;Li Qinfeng(Department of Dermatology,Tianjin Children’s Hospital,Tianjin 300134,China)

机构地区：[1]天津市儿童医院皮肤科,天津300134

出　　处：《中华整形外科杂志》2023年第11期1209-1212,共4页Chinese Journal of Plastic Surgery

摘　　要：该文报道1例2021年2月天津市儿童医院皮肤科收治的2岁女性幼儿色素血管性斑痣性错构瘤病(PPV)合并Klippel-Trenaunay(KT)综合征及其皮肤影像学表现。患儿出生时面部、躯干、臀部及四肢泛发红色及青褐色斑片,后逐渐出现四肢不对称,其临床表现、MR扫描等相关检查及病理活检结果符合PPV合并KT综合征的诊断。该病例罕见,治疗棘手。作者通过文献复习,总结PPV合并KT综合征的病因、诊断及治疗,并探讨其在反射式共聚焦扫描显微镜及皮肤镜下的表现特点。To report a case of phakomatosis pigmentovascularis(PPV)complicated with Klippel-Trenaunay(KT)syndrome and its skin imaging findings in a 2-year-old child.The child was born with red and cyan brown patches on the face,torso,buttocks and limbs,and then gradually developed limb asymmetry.The clinical manifestations,MR and related examinations,pathological biopsy results and skin imaging examination of the child were consistent with the diagnosis of PPV combined with KT syndrome.The case was rare and difficult to treat.Through literature review,the etiology,diagnosis and treatment of PPV complicated with KT syndrome was summarized,and the features of reflection confocal scanning microscope(RCM)and dermoscopy examinations were discussed.

关 键 词：错构瘤 儿童 皮肤 诊断 色素血管性斑痣性错构瘤病 

分 类 号：R751[医药卫生—皮肤病学与性病学]