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Epidermal Nevus (epidermal + nevus)
Terms modified by Epidermal Nevus Selected AbstractsLinear Epidermolytic Verrucous Epidermal Nevus of the Male GenitaliaPEDIATRIC DERMATOLOGY, Issue 4 2007EVREN SARIFAKIOGLU M.D. No abstract is available for this article. [source] Mosaic Chromosome 6 Trisomy in an Epidermal NevusPEDIATRIC DERMATOLOGY, Issue 2 2007Glenda J. Sobey F.C.Derm. Mosaicism for chromosome 6 in skin fibroblasts of affected skin was discovered. Trisomy 6 has not been previously implicated as an isolated finding in epidermal nevi or cutaneous mosaicism. [source] Epidermal Nevus Syndromes: Clinical Findings in 35 PatientsPEDIATRIC DERMATOLOGY, Issue 4 2004Helena Vidaurri-de la Cruz M.D. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms. [source] Five Fanconi anemia patients with unusual organ pathologiesAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2004Selma Unal Abstract Fanconi anemia (FA) is a rare autosomal recessive disorder that presents with variable organ abnormalities, progressive cytopenia, and susceptibility to the development of several malignancies. Although some of the organ pathologies such as microcephaly, microphthalmia, skin dyspigmentation, urogenital system involvement, and radial ray skeletal abnormalities are relatively common, there are some other abnormalities that are rarely associated with the disease [Alter BP. In: Nathan DG, Oski FA, editors. Hematology of infancy and childhood. Philadelphia: Saunders; 2003. p 259,273]. In this paper, five cases of unrelated FA patients with unusual organ pathologies, including chronic obstructive lung disease, lipodystrophy, Sprengel's deformity, diaphragmatic hernia, and inflammatory linear verrucous epidermal nevus (ILVEN) are presented. Recognition of unusual pathologies associated with FA is important in order to improve our understanding of the relationship between the disease and presenting organ pathologies. Am. J. Hematol. 77:50,54, 2004. © 2004 Wiley-Liss, Inc. [source] |