A Case of Netherton's Syndrome in a Newborn.
- Author:
Eun Hee LEE
1
;
Ellen Ai Rhan KIM
;
Ki Soo KIM
;
Beom Jin CHO
;
Jai Kyoung KOH
;
Soo Young PI
Author Information
1. Department of Pediatrics, Asan Medical Center, College of Medicine, Ulsan University, Seoul, Korea. arkim@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Netherton's syndrome;
Ichthyosiform dermatosis;
Bamboo hair;
Newborn
- MeSH:
Diagnosis;
Disease Susceptibility;
Hair;
Humans;
Ichthyosiform Erythroderma, Congenital;
Ichthyosis;
Infant, Newborn*;
Skin Diseases
- From:Journal of the Korean Pediatric Society
2003;46(4):389-392
- CountryRepublic of Korea
- Language:Korean
-
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.
