[Epidermal nevus syndrome (of Solomon, Fretzin and Dewald)].

Epidermal naevus syndrome is an entity which has been well established by Solomon, Fretzin and Dewald since 1968, presenting cutaneous, visceral, ocular, osseous and neurological malformations. We report a case with multiple osseous manifestations affecting the entire skeleton, but mainly the skull (osteolysis with absence of frontal bone), the vertebral column (scoliosis with triple deviation), the upper right limb (demineralisation and multiple pathological fractures) and lower members (fractures of the right and left femur) with enlargement of the entire ventricular system and well marked scissures, signs of an cortico-subcortical atrophy shown by scanogram.