A Case Report Of Epidermal Nevi Syndrome And Review Of The Literature

The epidermal nevus syndromes (ENS) are a group of congenital neurocutaneous disorders characterized by epidermal nevi in association with cerebral, ocular, skeletal, isometmes as well as blood vessel , cardiac and renal abnormalities. It is seldom encountered in clinics. We describe a patient with extensive epidermal nevi in combination with neurologic abnormalities, demonstrating the multiple-organ spectrum of involvement in epidermal nevus syndromes (ENS). Advances in molecular biology have revealed that the manifestations of epidermal nevus syndromes(ENS) are due to genomic mosaicism. Solomon proposed the term"epidermal nevus syndrome"(ENS) to describe the association of epidermal amartomas and extra-cutaneous abnormalities at the first time.This report is about a 21-year femal patient who was diagnosed epidermal nevus syndromes (ENS), but she has not any primary central nervous system(CNS)lesions .This girl has an extensive epidermal nevus all over her body for 21 years. When she was 3 months old many keratotic papules appeared on her neck, back, chest and limbs,which were dirty gray or brown and horny excrescences, soon they spread over her whole body. Erythra became more thickened, verrucous, and the color gradually hyperpigmented. The nails and mucosae are normal. In the body folds, lesions are softer and less verrucous. Sweating and friction Can mitigate the condition. The year of onset of epilepsy was 3 years ago. There are no other skin lesions and skeletal deformities .her nails and teeth are normal. There was familial background of onsanguinity. In her family, her mother and little brother have the same dermatogic disease but without epilepsy. The girl and her mother ,brother were all born at term after an eventful pregnancy. Adnexal structure is hyperplasia, hyperkeratesis, parakeratosis, and nevi cell do not occur on biopsy. The diagnosis of epidermal nevi syndrome is generally straightforward in her presenting with epidermal nevi in association with other organ abnormalities. This article discusses various facets of the ENS, including clinical presentations, histopathology, genetic basis, pathogenesis, and management. Some article report that nevi were located on the head and/or neck in 91% of the patients and 55% of the patients had seizures.