Severe dermatitis-multiple allergies-metabolic wasting syndrome

Orpha code: 369992OMIM code: 615508

Definition

Severe dermatitis-multiple allergies-metabolic wasting syndrome is a rare, genetic, epidermal disorder characterized by 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, eosinophillia, nystagmus, growth impairment and cardiac defects.

Disease data
Classification

Disease

Synonyms
Congenital erythroderma-hypotrichosis-recurrent infections-multiple food allergies syndrome
Zespół SAM
SAM syndrome
ORPHA code
369992
OMIM code
615508
ICD10 code
Q82.8
ICD11 code
-

No additional description.

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