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A Case of Phakomatosis Pigmentovascularis

Journal of the Korean Pediatric Society 1994;37(8):1156-1161.
Published online August 15, 1994.
A Case of Phakomatosis Pigmentovascularis
Seung Mo Park1, Kook In Park1, Hye Jung Joo1, Ran Namgung1, Chul Lee1, Dong Gwan Han1, Soo Il Chun2
1Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea
2Department of Dermatology, Yonsei University, College of Medicine, Seoul, Korea
색소혈관 모반증 1례
박승모1, 박국인1, 주혜정1, 남궁란1, 이철1, 한동관1, 전일수2
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 피부과학교실
Abstract
In 1947, Ota et al. First reported a case of phakomatosis pigmentovascularis and defined a subgroup of this disease having a congenital generalized hemangioma and pigmented lesions including anmongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovascularis type Iib in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyle between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.
Key Words: Phakomatosis pigmentovascularis, Nevus flammeus, Blue spot, Syndactyly, Dilated collecting system


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