A Case of Incontinentia Pigmenti Associated with Ocular Complications.

Lee, Nam Kyung; Kim, Hyun Kyung; Han, Young Bock

Case Report

Journal of the Korean Ophthalmological Society 1993;34(7):692-695.
Published online July 1, 1993.

A Case of Incontinentia Pigmenti Associated with Ocular Complications.

Nam Kyung Lee, Hyun Kyung Kim, Young Bock Han

Department of Ophthalmology, College of Medicine, Ewha Womans University, Seoul, Korea.

안증상을 동반한 색소실조증 1 예

이남경(Nam Kyung Lee),김현경(Hyun Kyung Kim),한영복(Young Bock Han)

Abstract

A Case of lncontinentia pigmenti. Incontinentia pigmenti is a hereditary disorder of abnormal skin pigmentation with associated ocular, skeletal, and central nervous system abnormalities. Ocular findings are present in up to 35% of cases, but in Korea only two cases have been reported. Recently, the authors have experienced one case of incontinentia pigmenti in a five-day old female patient who manifested both the typical dermatologic findings and ocular abnormalities in the right eye. The fundus showed markedly dilated and tortuous vessels with flat retinal neovascularization, scattered retinal hemorrhage of right macular area. Retinal cryopexy and posterior vitrectomy were performed.

Key Words: lncontinentia pigmenti;Skin pigmentation;Ocular abnormalities