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A case of Incontinentia Pigmenti.

Journal of the Korean Pediatric Society 1985;28(9):940-944.
Published online September 30, 1985.
A case of Incontinentia Pigmenti.
Wan Seob kim1, Yong Woo Choi1, Myung Ho Lee1, Chul Wan Lim2
1Department of Pediatrics, Presbyterian Medical Center, Jeon-ju
2Department of Dermatology Jeon-bug National University Medical School
중추신경 증상을 동반한 색조실조증 1례
김완섭1, 최용우1, 이명호1, 임철완2
1전주예수병원 소아과
2전북의대 피부과
A case of incontinentia pigmenti with CNS involvement was presented. And literatures have been reviewed. The patient was a 7 month-old female had been affected with numerous dark brownishinear streaks, irregular macules, and splashes of brown to slate gray pigmentation on the entire body, especially both trunk, axillary, and extremity. The chief complaint was severe vomiting and comatous mental state for 1 day. Neurologic examination showed left upward deviation of both eyeball, miosis, and Barbinski reflex:was positive. The chest and skull X-ray showed no gross abnormality, the Lab showed no specific findings, brain CT and EEG showed marked abnormality. The skin pigmentation was also noted in her grandmother, mother, and one of her sisters. Diagnosis was confirmed by the characteristic clinical pictures and histopathologic findings.
Key Words: Incontinentia pigmenti, CNS involvement, Histopathologic findings

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