[Epidermal nevus syndrome with multiple vascular hamartomas and malformations]

The authors described a 39 year old woman affected by epidermal nevus syndrome, with cutaneous (verrucous epidermal nevus), skeletal (thoracolumbar levoscoliosis and frontal bossing) and ocular (papillar coloboma and coroideal nevus) defects. Moreover the patient presented vascular malformations and hamartomas: lymphangioma circumscriptum of the mammary area, left peroneal Gorham's disease, artero-venous acral tumour of the left foot and multiple artero-venous shunts of the lower limbs. Since puberty, hemodynamic modifications have caused pseudo-Kaposi of Bluefarb-Stewart of legs and feet and malleolar painful ulcers. Solomon's epidermal nevus syndrome is an heterogeneous entity. In our opinion, this is the first case report with a severe vascular involvement.