Camacho Martinez F, Moreno Gimenez J C
Ann Dermatol Venereol. 1985;112(2):143-7.
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.
自1968年以来，所罗门、弗雷茨金和德瓦尔德已明确表皮痣综合征这一病症，其表现为皮肤、内脏、眼部、骨骼和神经畸形。我们报告一例具有多种骨骼表现的病例，累及整个骨骼系统，但主要是颅骨（额骨缺失伴骨质溶解）、脊柱（伴有三重弯曲的脊柱侧弯）、右上肢（骨质脱矿质和多处病理性骨折）及下肢（左右股骨骨折），同时整个脑室系统扩大且脑沟明显，扫描图显示有皮质 - 皮质下萎缩迹象。
