By Gary M. White, MD

Phacomatosis pigmentovascularis (PPV) (also phakomatosis pigmentovascularis) is a very rare disorder in which various combinations of congenital melanocytic and vascular lesions occur together. Multiple subtypes have been described.


From Arch Dermatol. 2005 Mar;141(3):385-8.


Phacomatosis cesioflammea. Bilateral Nevus flameus and aberrant Mongolian spots on the back. J Dermatol Venereol Leprol. 2010 May-Jun;76(3):307

Extensive dermal melanocytosis and capillary malformation. Dermatology Online Journal 21(6)

A solitary lesion with features of dermal melanocytosis and nevus spilus. Dermatology Online Journal 17(8)


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