Port-wine stain is a vascular malformation characterized pathologically by ectasia of superficial dermal capillaris and clinically by persistent macular erythema. The association of a port-wine stain on a limb with soft tissue swelling, with or without bony overgrowth, is termed klippel-Trenaunay syndrome. Phakomatosis pigmento-vascularis is a combination of port-wine stain and cutaneous pigmentary abnormality.
The patient who is described here is a 25-year-old man with Klippel-Trenaunay syndrome in association with bilateral Ota nevus. He can be classified as phakomatosis pigmento vascularis type IIa which is a relatively rare finding with this presentation.