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Dermatology Online Journal

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Red-white and blue baby: a case of phacomatosis pigmentovascularis type V


Phacomatosis pigmentovascularis is a rare genodermatosis characterized by the combination of an extensive pigmentary nevus with a widespread vascular nevus. The coexistence of aberrant dermal melanocytosis and cutis marmorata telangiectatica congenita has been termed phacomatosis pigmentovascularis type V or phacomatosis cesiomarmorata. Phacomatosis pigmentovascularis type V was first described in a 3-month-old boy in 2000. Since then, there have been a further seven cases published in the literature.

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