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syndroom van ernstige dermatitis, meerdere allergieën en metabole aftakeling (aandoening)
syndroom van ernstige dermatitis, meerdere allergieën en metabole aftakeling
'severe dermatitis-multiple allergies-metabolic wasting'-syndroom
SAM-syndroom
Severe dermatitis, multiple allergies, metabolic wasting syndrome
Congenital erythroderma, hypotrichosis, recurrent infections, multiple food allergies syndrome
SAM syndrome
A rare genetic epidermal disorder with characteristics of congenital erythroderma with severe psoriasiform dermatitis, ichthyosis, severe palmoplantar keratoderma, yellow keratosis on the hands and feet, elevated immunoglobulin E, multiple food allergies, and metabolic wasting. Other variable features may include hypotrichosis, nail dystrophy, recurrent infections, mild global developmental delay, eosinophilia, nystagmus, growth impairment and cardiac defects.
Id774211005
StatusPrimitive
Associated morphologydiffuse ontsteking
Finding sitestructuur van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Pathological processafwijkend immuunproces
Associated morphologyerytheem
Finding sitestructuur van huid
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetQ82.8
TermOverige gespecificeerde congenitale misvormingen van huid
SNOMED CT to Orphanet simple map369992
SNOMED CT to ICD-10 extended map
TargetQ82.8
RuleTRUE
AdviceALWAYS Q82.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified