Epidermal nevus syndromes: clinical findings in 35 patients.

摘要: Of the patients with epidermal nevi, 10-18% may have disorders of eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal mesodermal structures also be found. There are six different nevus syndromes described so far: Proteus, congenital hemidysplasia ichthyosiform limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker comedonicus. Thirty-five syndrome seen at National Institute Pediatrics Mexico City during a 31-year period described. This represented 7.9% 443 nevi; its relative frequency was 1 case per 11,928 pediatric 1080 dermatologic patients. Nine (26%) had Proteus syndrome. Sebaceous found (17%), while comedonicus three (8%). Two were diagnosed pigmentokeratotica one patient is first report Mexican One an inflammatory linear verrucous systemic involvement. Thirteen (37%) keratinocytic nevi We propose defined as association neuronal migration and/or addition higher risk for neoplasms.