Abstract
Phacomatosis pigmentovascularis is a rare disorder which occurs sporadically, with just about 200 cases reported worldwide.1 The syndrome is characterized by a capillary nevus occurring in conjunction with a pigmented nevus of various types.2 The condition may occur with systemic/syndromic involvement.3 The first reported case dates back to 1920, but it was only in 1947 that the condition was described in detail by Ota et al. Happle simplified and reclassified the disorder in 2005.4 We report a case of phacomatosis pigmentovascularis with Raynaud's phenomena in a young adult male.
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