Cutaneous Vascular Anomalies – Phakomatosis Pigmentovascularis

Phakomatosis Pigmentovascularis is a group of skin disorders in which patients have a combination of a vascular skin malformation(s) and a melanocytic skin lesion(s) that I encounter in both my The Woodlands dermatology and Conroe dermatology offices.  The skin lesions that may be involved in this disorder include nevus flammeus, nevus spillus, Mongolian spots, epidermal nevus, cutis marmorata to name a few.  In some patients with Phakomatosis Pigmentovascularis, there may be underlying systemic abnormalities such as CNS vascular malformations, ocular abnormalities, choroidal melanoma, hemihypertrophy of the limbs and internal organ vascular malformations.  Most patients with Phamkomatosis Pigmentovascularis are Asian.