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A Case of Netherton's Syndrome in a Newborn

Journal of the Korean Pediatric Society 2003;46(4):389-392.
Published online April 15, 2003.
A Case of Netherton's Syndrome in a Newborn
Eun-Hee Lee1, Ellen Ai-Rhan Kim1, Ki-Soo Kim1, Beom-Jin Cho2, Jai-Kyoung Koh3, Soo-Young Pi1
1Department of Radiology, College of Medicine, Ulsan University, Asan Medical Center, Seoul, Korea
2Department of Ophthalmology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea
3Department of Dermatology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea
신생아기에 진단된 Netherton 증후군 1례
이은희1, 김애란1, 김기수1, 조범진2, 고재경3, 피수영1
1울산대학교 의과대학 서울아산병원 소아과
2울산대학교 의과대학 서울아산병원 안과
3울산대학교 의과대학 서울아산병원 피부과
Correspondence: 
Ellen Ai-Rhan Kim, Email: arkim@amc.seoul.kr
Abstract
Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.
Key Words: Netherton's syndrome, Ichthyosiform dermatosis, Bamboo hair, Newborn


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