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Ichthyosiform Erythroderma, Congenital

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Definition: Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type.  an ichthyosis rather than an erythroderma; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES   
Examples Hyperkeratosis, Epidermolytic; Ichthyosis, Lamellar; Netherton Syndrome 		Other names Congenital Ichthyosiform Erythroderma; Ichthyosiform Erythrodermas, Congenital; Erythrodermas, Congenital Ichthyosiform; Congenital Ichthyosiform Erythrodermas; Erythroderma, Congenital Ichthyosiform; Congenital Ichthyosiform Erythroderma, Wet Type; Congenital Ichthyosiform Erythroderma, Dry Type
 
SubstanceCAS Registry & nameCategoriesSource
Triglyceride storage disease with impaired long-chain fatty acid oxidation  0   *Lipid Metabolism, Inborn Errors *Muscular Diseases *Ichthyosiform Erythroderma, Congenital.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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