Autosomal recessive hereditary disorder	Combined immunodeficiency disease	Congenital anomaly of skin	Congenital immunodeficiency disease	Developmental hereditary disorder	Diffuse dermatitis	Erythroderma	Hereditary disorder of immune system	Hereditary disorder of the integument	Increased immunoglobulin
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Severe dermatitis, multiple allergies, metabolic wasting syndrome (disorder)
	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.
	syndroom van ernstige dermatitis, meerdere allergieën en metabole aftakeling
	'severe dermatitis-multiple allergies-metabolic wasting'-syndroom SAM-syndroom

Id	774211005
Status	Primitive

Associated morphology	Diffuse inflammation
Finding site	Skin structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Above reference range
Interprets	Immunoglobulin E measurement

Pathological process

Abnormal immune process

Associated morphology	Erythema
Finding site	Skin structure

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q82.8
Rule	TRUE
Advice	ALWAYS Q82.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified