A Case of Incontinentia Pigmenti Associated with Ocular Complications

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.