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

Manisha Reddy, Keya Rani Lahiri, Anand Sude

Abstract: 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.

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