色素血管性斑痣性错构瘤病Ⅱb型并发Klippel-Trenaunay综合征及其皮肤镜表现  

Phakomatosis pigmentova scularis type Ⅱb with Klippel-Trenaunay syndrome: a case report with dermoscopic features

作　　者：丁帆 金轶 李谢伦 许阳[1] 苏婷[1] 陈斌[1] DING Fan;JIN Yi;LI Xie-lun;XU Yang;SU Ting;CHEN Bin(Department of Dermatology,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院皮肤科,江苏南京210029

出　　处：《临床皮肤科杂志》2020年第11期669-672,共4页Journal of Clinical Dermatology

摘　　要：报告1例儿童色素血管性斑痣性错构瘤病。患儿男,4岁,因皮肤红色及青灰色斑片4年就诊。体格检查:左侧腰背部、左侧臀部、左下肢、外阴及左足暗红色斑片;左侧额部、颊部散在分布青灰色斑片,左眼巩膜青灰色斑;躯干大范围青灰色斑片,左侧腰背部色素斑与红斑融合;双侧臀部不对称,双下肢不等长、不等粗。皮肤镜检查:红斑区见红色点、球状小血管及扩张屈曲的线状血管;青灰色斑片区见蜂窝样灰褐色网状色素沉着;局部见球状血管与网状色素沉着的交织融合。超声心动图:左心室稍大;轻度三尖瓣关闭不全;轻度主动脉瓣关闭不全。诊断:色素血管性斑痣性错构瘤病Ⅱb型并发Klippel-Trenaunay综合征。通过文献复习,探讨总结色素血管性斑痣性错构瘤病并发Klippel-Trenaunay综合征的病因、诊断及治疗,并探讨该病皮肤镜表现的特点。A case of phakomatosis pigmentovascularis in children is reported. The patient was a 4-year-old boy, visited for the red and cyan-grey patches presented at birth. Physical examination showed dark-red patches on left back, left buttock, left lower limb, genitalia and left foot, cyan-grey patches scattered on left forehead, left cheek and left sclera, extensive cyan-grey patches on bilateral thoracoabdominal, bilateral back. Pigment patches and erythema fused on left back. Bilateral buttocks were asymmet rical and lower limbs were unequal in length and thickness. Dermoscopy showed red punctate, globular vessels and dilated, flexed linear vessels in the erythema,honeycomb-like greyish-brown reticular pigmentations in the cyan-grey patches. A mixture of globular vessels and reticular pigmentations is visible. Echocardiography showed slightly larger left ventricle, mild tricuspid insufficiency and mild aortic insufficiency. The diagnosis of phakomatosis pigmentovascularis type Ⅱb with Klippel-Trenaunay syndrome was made. We reviewed the current literature and summarized the etiology, diagnosis and treatment of phacomatosis pigmentovascularis associated with Klippel-Trenaunay syndrome. Especially, dermoscopic features of this case were also discussed.

关 键 词：色素血管性斑痣性错构瘤病 KLIPPEL-TRENAUNAY综合征 皮肤镜表现 

分 类 号：R739.5[医药卫生—肿瘤] R725.5[医药卫生—临床医学]