Phakomatosis pigmentovascularis

Phakomatosis pigmentovascularis
Other namesAssociation of cutaneous vascular malformations and different pigmentary disorders
SpecialtyDermatology

Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.

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