All issues > Volume 28(9); 1985

Case Report

J Korean Pediatr Soc. 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

Abstract

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.

Keywords :Incontinentia pigmenti; CNS involvement; Histopathologic findings