congenitale letale erytrodermie (aandoening)
congenitale letale erytrodermie
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.
Associated morphologyinflammatoire morfologie
Finding sitestructuur van huid
Associated morphologyexfoliatief erytheem
Finding sitestructuur van huid
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TermOverige gespecificeerde congenitale misvormingen van huid
SNOMED CT to Orphanet simple map1954
SNOMED CT to ICD-10 extended map
AdviceALWAYS Q82.8
CorrelationSNOMED CT source code to target map code correlation not specified