A Case of Congenital CMV Infection associated with Incontinentia Pigmenti.

An,  Joo Ran; Jeong,  Myung Sook; Kim,  Kyung Ah; Ko,  Sun Young; Lee,  Yeon Kyung; Shin,  Son Moon

Case Report

Journal of the Korean Society of Neonatology 2004;11(2):236-240.
Published online November 1, 2004.

A Case of Congenital CMV Infection associated with Incontinentia Pigmenti.

Joo Ran An, Myung Sook Jeong, Kyung Ah Kim, Sun Young Ko, Yeon Kyung Lee, Son Moon Shin

Department of Pediatrics, Sungkyunkwan University School of Medicine, Samsung Chehil Hospital, Seoul, Korea. ykleeped@hanmail.net

색소실조증을 동반한 선천성 거대세포바이러스 감염증 1례

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Abstract

Congenital cytomegalovirus (CMV) infection is one of the most frequent congenital infections in neonates. It can manifest as asymptomatic infection in 90% or symptoms may appear in 10% of the patients. Asymptomatic congenital CMV infection is likely to be a leading cause of sensorineural hearing loss, mental retardation and microcephaly. Incontinentia pigmenti is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eyes, CNS, teeth, hair, and nail. It is regarded as an X-linked dominent genetic disorder. We are reporting a case of neonate who presented with delayed development and bilateral hearing loss due congenital CMV infection which was incidentally assoicated with incontinentia pigmenti.

Key Words: Congenital cytomegalovirus infection; Hearing loss; Incontinetia pigmenti