Epidermal nevus syndrome associated with polyostotic fibrous dysplasia, CNS lipoma, and aplasia cutis.
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.
Cabanillas
Miguel
M
Department of Dermatology, Complexo Hospitalario Arquitecto Marcide-Novoa Santos, Ferrol, Spain. miguel.cabanillas.gonzalez@sergas.es
Aneiros
Angel
A
Monteagudo
Benigno
B
Santos-García
Diego
D
Suárez-Amor
Oscar
O
Ramírez-Santos
Aquilina
A
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*