Epidermal Nevus Syndrome and Dysplatic Kidney Disease
Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this association, it is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders.
Alsohim F, Abou-Jaoude P, Ninet J, et al. Bilateral renal artery stenosis and epidermal nevus syndrome in a child. Pediatr Nephrol 2011;26(11):2081-4.
Kaur T, Kumar B, Chawwla YK. Epidermal nevi massociated with extrahepatic portal venous obstruction, hypoplastic kidney and lymphangiectasia: a new syndromic variant? BR J Dermatol 2004;150(3):618-9.
Kishida ES, Muniz Silva MA, da Costa Pereira F, et al. Epidermal nevus syndrome associated with adnexal tumors, spitz nevus, and hypophosphatemic vitamin Dresistant rickets. Pediatr Dermatol 2005;22(1):48-54.
Kim HS, Shin YK, Lee KH, et al. A Case of Epidermal Nevus Syndrome with Infantile Spasm. J Korean Child Neurol Soc 1998;5:346-50.
Aizawa K, Nakamura T, Ohyama Y, et al. Renal artery stenosis associated with epidermal nevus synd rome. Nephron 2000;84(1):67-70.
Oranje AP, Przyrembel H, Meradji M, et al. Solomon's epidermal nevus syndrome (type: linear nevus sebaceus) and hypophosphatemic vitamin Dresistant rickets. Arch Dermatol 1994;130(9):1167-71.
Herman TE, Siegel MJ. McCune Albright Syndrome: Epidermal Nevus Syndrome. Clin Pediatr 2010;49(12):1164-6.
Stosiek N, Hornstein OP, Hiller D, et al. Extensive linear epidermal nevus associated with hemangiomas of bones and vitamin-D-resistant rickets. Dermatology 1994;189(3):278-82.
Moreira AI, Ferreira G, Santos M, et al. Epidermal nevus syndrome associated with hypophosphatemic rickets. Dermatol Online J 2010;16(9):14.
Bourdeaut F, Hérault A, Gentien D, et al. Mosaicism for oncogenic G12D KRAS mutation associated with epidermal nevus, polycystic kidneys and rhabdomyosarcoma. J Med Genet 2010;47(12):859-62.
Cambio AJ, Evans CP, Kurzrock EA. Non-surgical management of multicystic dysplastic kidney. BJU Int 2008;101(7):804-8.
Hains DS, Bates CM, Ingraham S, et al. Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 2009;24(2):233-41.
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