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Epidermal Nevous in Association with Some Uncommon Manifestations

Nima Rezaei


Puni tekst: engleski PDF 139 Kb

preuzimanja: 617

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Sažetak

Epidermal nevus syndrome (ENS) is a rare disorder, characterized with congenital epidermal nevi in association with several abnormalities in different organs.

Herein, an infant with EN is presented who suffered from various manifestations, including aplasia cutis congenital, congenital hemangioma, ambiguous genitalia, mislocated anus, and growth retardation. Skin biopsy showed dysmorphic folliculosebaceous apparatus in the dermis and hypertrichosis, compatible with organoid nevus. No mutations were found in FGFR3, PIK3CA, NRAS, HRAS and KRAS genes.

Although the diagnosis of ENS is made for the patient, association of some rare manifestations could suggest either presence of mutation in an unknown gene or an undefined syndrome with manifestations resembling ENS.

Ključne riječi

Hrčak ID:

116003

URI

https://hrcak.srce.hr/116003

Datum izdavanja:

13.1.2014.

Posjeta: 1.292 *