Autosomal recessive hereditary disorder
Congenital disease
Erythroderma
Hereditary disorder of the integument
Inflammatory dermatosis
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Congenital lethal erythroderma (disorder)
Congenital lethal erythroderma
A rare skin disorder characterized by erythrodermic peeling skin from birth with no obvious nail or hair-shaft abnormalities and other associated anomalies including diarrhea, failure to thrive and severe hypoalbuminemia resistant to correction by enteral or intravenous supplementation. An autosomal recessive mode of inheritance is highly probable. The prognosis is poor and infants die in the first months of life. There have been no further descriptions in the literature since 1992.
congenitale letale erytrodermie
Id722391005
StatusPrimitive
Associated morphologyInflammatory morphology
Finding siteSkin structure
OccurrenceCongenital
Associated morphologyExfoliative erythema
Finding siteSkin structure
OccurrenceCongenital
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetQ82.8
RuleTRUE
AdviceALWAYS Q82.8
CorrelationSNOMED CT source code to target map code correlation not specified