title: Epidermal nevus syndrome associated with polyostotic fibrous dysplasia, CNS lipoma, and aplasia cutis.
abstract: Epidermal nevus syndrome is a rare congenital sporadic neurocutaneous disorder characterized by an epidermal nevus and various developmental abnormalities of the skin, eyes, nervous, cardiovascular and urogenital systems. We describe a patient with an extensive epidermal nevus associated with various organ abnormalities, particularly polyostotic fibrous dysplasia, central nervous system lipoma, and aplasia cutis. Our patient demonstrates the polymorphic spectrum of involvement in epidermal nevus syndrome.
authors: Department of Dermatology, Complexo Hospitalario Arquitecto Marcide-Novoa Santos, Ferrol, Spain. miguel.cabanillas.gonzalez@sergas.es
authors: Miguel
authors: M
authors: Cabanillas
authors: Angel
authors: A
authors: Aneiros
authors: Benigno
authors: B
authors: Monteagudo
authors: Diego
authors: D
authors: Santos-García
authors: Oscar
authors: O
authors: Suárez-Amor
authors: Aquilina
authors: A
authors: Ramírez-Santos
mesh_terms: D000328:Adult; D004476:Ectodermal Dysplasia / Q000150:complications*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D006801:Humans; D008067:Lipoma / Q000150:complications*; D008297:Male; D009378:Neoplasms, Multiple Primary; D009506:Nevus / Q000150:complications*; D012878:Skin Neoplasms / Q000150:complications*; D013120:Spinal Cord Neoplasms / Q000150:complications*