Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
4170324 | Journal de Pédiatrie et de Puériculture | 2008 | 5 Pages |
Abstract
Netherton syndrome is a rare genodermatosis characterized by ichthyosiform scaling, hair shaft abnormalities and atopic features. We report two cases of Netherton syndrome in an 18-month-old girl and a 24-month-old boy. The girl presented pruriginous erythroderma, frequent otitis, growth retardation and Trichorrhexis invaginata. Immunohistochemistry shows LEKTI deficiency. The boy presented non-bullous congenital erythroderma, signs of atopy, several episodes of bacterial infection and growth retardation. The hair was normal; immunohistochemistry showed LEKTI deficiency. Immunohistochemistry of LEKTI protein allows a fast diagnosis of Netherton syndrome.
Related Topics
Health Sciences
Medicine and Dentistry
Perinatology, Pediatrics and Child Health
Authors
N. El Fékih, M. Kharfi, M. Khmiri, N. Mattoussi, S. Fraitag, C. Bodemer, M. Ridha Kamoun,