Ichthyosiform erythroderma and cardiomyopathy: report of two cases and review of the literature
Authors: Hoeger1; Adwani2; Whitehead3; Finlay4; Harper1
Source: British Journal of Dermatology, Volume 139, Number 6, December 1998 , pp. 1055-1059(5)
Publisher: Blackwell Publishing
Abstract:
We report two children with ichthyosiform erythroderma who at the ages of 9 weeks and 8 years, respectively, developed dilated cardiomyopathy, which was fatal in one and required heart transplantation in the other. A link between these conditions is considered likely, either as a primary genetic syndrome or secondary to micronutrient deficiency and/or infection. Owing to its insidious onset, cardiomyopathy may be overlooked, or symptoms attributed to the other conditions such as severe infections and failure to thrive that are common in these patients. We therefore recommend that children with congenital erythroderma are monitored closely, clinically and with echocardiography, for cardiomyopathy.Document Type: Original article
DOI: 10.1046/j.1365-2133.1998.02565.x
Affiliations: 1: Department of Paediatric Dermatology, 2: Departments of Congenital Heart Disease, 3: Transplantation Centre, Great Ormond Street Hospital for Children, London WC1 3JH, 4: Dermatology, University Hospital of Wales, Cardiff CF4 4XW

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