cutaneous vascular and pigmentary lesions. The pregnancy of his mother and family history were unremarkable. Physical examination showed bilateral nevus flammeus involving the scalp, face, neck, trunk, upper part of the back, scrotum and the flexor sides of arms and thighs. Large, bluish-grey pigmentation was observed on the abdominal area, back, buttocks, legs and the extensor sides of the arm...

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 ...