色素血管性斑痣性错构瘤病23例临床分析

目的：探讨我科2001年来23例色素血管性斑痣性错构瘤病的临床表现及分型。方法：收集、研究和评价我院2001～2008年间23例色素血管性宽痣性错构瘤的资料，根据传统和Happle分类方法进行分型，总结其临床特征。结果：23例患者中以中年女性多见（15例），平均年龄21．4岁。根据传统分类，最常见的为Ⅱ型，占78％，其次为IⅡ型，占13%根据Happle新分类方法，18例为phakomatosis ccsioflammca,3例为phakomatosis Spilorosca，2例为未分类型，18例有太田痣。结论：色素血管性斑痣性错构瘤病临床上较少见，往往因认识不够容易造成误诊及漏诊，详细询问病史和全面查体有助于准确的诊断。

Clinical analysis of 23 cases with phakomatosis pigmentovascularis

Objective To describe the clinical features and classification of the Phakomatosis Pigmentovascularis. Methods The clinical features of 23 cases of Phakomatosis Pigmentovascularis admitted from 2001 to 2008 were respectively analyzed according to both the classical and Happle＇s classification. Results Most were middle aged female （n=15） with a mean age of 21.4 years. According to the classical classification, eighteen belong to the type II （78%）, three belong to type III （13%）. We reclassified them according to Happle＇s new classification, found 18 had phakomatosis cesioflammea, 3 phakomatosis Spilorosea, and 2 an unclassifiable form. Of the 23 patients, 18 had nevus of Ota. Conclusions PPV are rare syndromes with a wide variability in their clinical expression, and are easy to be misdiagnosed. Detailed case history and systematical physical examination are helpful for diaganosis.