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A Case of Incontinentia Pigmenti

Journal of the Korean Pediatric Society 1993;36(3):428-433.
Published online March 15, 1993.
A Case of Incontinentia Pigmenti
Soo Am Chung1, Won Rae Kim1, Hyung Kun Nam1, Jin Sam No1, Jung Hee Park2
1Department of Pediatrics, St. Francisco General Hospital, Seoul, Korea
2Department of Anatomical Pathology, St. Francisco General Hospital, Seoul, Korea
색소실조증 1례
정수암1, 김원래1, 남형근1, 노진삼1, 박정희2
1방지거병원 소아과
2방지거병원 해부병리과
Abstract
Incontinentia pigmenti (Bloch-Sulzberger syndrome)is a genetic disease of the skin with generalized ectodermal and mesodermal dysplasia. Skin lesions are uually present at birth or shortly after in the form of eythermatous eruptions with linear vesiculations. The final stage is characterised by irregular macular streaks of brown to slate-grey pigmentation. We experienced five cases of incontinentia pigmenti maternal female relative of a three generation.
Key Words: Incontinentia pigmenti, Bloch-Sulzberger syndrome


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