J Korean Acad Rehabil Med Search


Journal of the Korean Academy of Rehabilitation Medicine 1995;19(1):28.
Epidermal Nevus Syndrome Accompanied by Neurologic Manifestation(A case report)
Jeong Lim Moon, M.D., Sae Yoon Kang, M.D., Keun Young Park, M.D., , Jong Hyun Kim, M.D.,
Department of Rehabilitation Medicine, Catholic University Medical College
신경학적 이상을 동반한 표피 모반 증후군(증례보고)
문정림, 강세윤, 박근영, 김종현
가톨릭대학교 의과대학 재활의학교실

The epidermal nevi are congenital skin lesions that consist of slightly raised ovoid or linear plagues. Smaller lesions occur most frequently on the face, scalp and neck, while larger lesions may affect any areas, There is often a predilection for the midline.

The epidermal nevus syndrome (ENS) is a rare neurocutaneous disorder that consists of epidermal nevi and a wide variety of congenital anomalies of the brain, eye, skeleton, and other systems.

Since 1964, about 60 cases of ENS have been reported in other countries and there was only one case report on ENS accompanied by multiple skeletal abnormalities and hydrosalpinx in Korea.

For the first time in Korea, We identified a patient with a neurologic syndrome consisting of epidermal nevi. left ptosis, left cryptorchidism, right ectopic testis, direct lateral abdominal wall hernias on both sides, right hemiparesis and mental retardation.

For this report, we describe data on the patient with ENS based on clinical description and pathologic studies.

Key Words: Epidermal nevus syndrome, Neurologic syndrome, Hemiparesis, Mental retardation


Browse all articles >

Terms of Use   |   Privacy Polity
Editorial Office
Department of Rehabilitation Medicine, Seoul National University Hospital
101 Daehak-ro, Jongno-gu, Seoul, Korea
Tel: +82-10-8678-2671    Fax: +82-2-6072-5244    E-mail: edit@e-arm.org; edit.karm@gmail.com
Business Registration: 110-82-07460                

Copyright © 2024 by Korean Academy of Rehabilitation Medicine.

Developed in M2PI

Close layer