Netherton syndrome was first described by Dr. E. W. Nethertonin 1958 as a disorder characterized by ichthyosis (Ichthyosis lineariscircumflexa and/or congenital lamellar ichthyosis), bamboo hair(also known as trichorrhexis invaginata), and atopic manifestations.1 Netherton syndrome accounts for 18% of all congenitalerythroderma cases.2 Other clinical characteristics include mentalretardation, enteropathy, aminoaciduria, hypoalbuminemia,growth retardation, and immunologic abnormalities.3 Serum IgElevels are markedly elevated in Netherton syndrome patients,which suggests an imbalance of the immune system.
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