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The Dry Baby - (Non-Bullous Congenital Ichthyosiform Erythroderma) - A Case Report

Journal: International Journal of Science and Research (IJSR) (Vol.9, No. 5)

Publication Date:

Authors : ; ; ;

Page : 551-552

Keywords : icthyosis; parakeratosis; pseudoepitheliomatous hyperplasia; telangectasia; hydration; lubrication; keratolytics; retinoids; calcipotriol;

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The ichthyosis are typically inherited conditions exhibiting disordered keratinization secondary to excessive transepidermal water loss.1 Non-bullous congenital ichthyosiform (NBCIE) erythroderma is a rare chronic autosomal recessive form of inherited ichthyosis. Clinically NBCIE appears as generalised erythroderma with fine white scales that gradually replace the collodion membrane. NBCIE is estimated to occur in 1: 300, 000 newborns of all races, more frequently seen in consanguinity.2, 3 The genes involved in mutation are TGM1, ALOX12B, ALOXE3 and ichthyin. Here we report one such rare case of NBCIE in a 10 month old child with consistent clinical and skin biopsy findings.

Last modified: 2021-06-28 17:06:43