%0 Journal Article %T Report of a case of Klippel-Trenaunay syndrome associated with bilateral nevus of Ota %J Iranian Journal of Dermatology %I Iranian Society of Dermatology %Z 2717-0721 %A Ehsani, Amir Hooshang %A Tork, Ali Naser %A Yazdanian, Shideh %D 2006 %\ 12/01/2006 %V 9 %N suppl.1 %P 12-12 %! Report of a case of Klippel-Trenaunay syndrome associated with bilateral nevus of Ota %K phakomatosis %K Ota nevus %K vascular malformation %R %X 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. %U