儿童色素血管性斑痣性错构瘤病18例临床分析

Clinical analysis of 18 cases of children with phakomatosis pigmentovascularis

目的:探讨儿童色素血管性斑痣性错构瘤病(phakomatosis pigmentovascularis,PPV)的临床特征。方法:对临床确诊的18例儿童PPV的资料进行回顾性总结,分析其临床特点、分型及治疗随访情况。结果:男女均可发病,以女性患儿多见,平均年龄2.8岁。鲜红斑痣是最常见的血管性改变,太田痣并发蒙古斑为最常见的色素性改变,血管和色素的嵌合为棋盘样模式。根据最新的Happle分型方法分类,Cesioflanmea型17例,Melanorosea型1例。8例患儿有系统受累。3例患儿的面部皮损行激光治疗,疗效显著。结论:儿童PPV应全面体检和长期随访,血管和色素性皮损可尽早行激光治疗。

Objective： To elucidate the clinical features of phakomatosis pigmentovascularis（PPV）. Methods： The clinical data were collected from 18 children with PPV, and the clinical features and classification were summarized. Results： Most of cases were females（n=13） with a mean age of 2.8 years. Naevus flammeus was the most common vascular abnormality, while nevus of Ota with Mongolian spots was the most common pigmentary disorder. These pigmentary and vascular naevi were intermingled with each other, forming a chequer-board pattern. According to Happle＇s new classification, seventeen patients （94.4%） belonged to phakomatosis cesioflammea, and one was Melanorosea type. 8 patients were with systemic involvement. Lesions responded to laser therapy well in 3 patients. Conclusions： Comprehensive physical examination and long time follow-up should be given to PPV patients. Cutaneous vascular and pigmentary abnormalities should be treated with lasers as early as possible.