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J Korean Pediatr Soc. 2003 Apr;46(4):389-392. Korean. Case Report.
Lee EH , Kim EA , Kim KS , Cho BJ , Koh JK , Pi SY .
Department of Pediatrics, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea. arkim@amc.seoul.kr
Department of Ophthalmology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea.
Department of Dermatology, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea.
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.

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