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: Miguel Cabanillas, Angel Aneiros, Benigno Monteagudo, Diego Santos-García, Oscar Suárez-Amor, Aquilina Ramírez-Santos
• Journal: Dermatology online journal (Dermatol Online J) Vol. 15 Issue 10 Pg. 7 (Oct 15 2009) ISSN: 1087-2108 [Electronic] United States
• PMID: 19951625 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Ectodermal Dysplasia (complications)
• Fibrous Dysplasia, Polyostotic (complications)
• Humans
• Lipoma (complications)
• Male
• Neoplasms, Multiple Primary
• Nevus (complications)
• Skin Neoplasms (complications)
• Spinal Cord Neoplasms (complications)