Ferro-cerebro-cutaneous syndrome

Orpha code: 397922OMIM code: 301072

Definition

Ferro-cerebro-cutaneous syndrome is a rare, genetic, metabolic liver disease characterized by progressive neurodegeneration, cutaneous abnormalities, including varying degrees of ichthyosis or seborrheic dermatitis, and systemic iron overload. Patients manifest with infantile-onset seizures, encephalopathy, abnormal eye movements, axial hypotonia with peripheral hypertonia, brisk reflexes, cortical blindness and deafness, myoclonus and hepato/splenomegaly, as well as oral manifestations, including microdontia, widely spaced and pointed teeth with delayed eruption, and gingival overgrowth.

Disease data
Classification

Disease

Synonyms
Cerebro-cutaneous syndrome with iron overload
Zespół mózgowo-skórny z nadmiarem żelaza
ORPHA code
397922
OMIM code
301072
ICD10 code
G23.0
ICD11 code
-

No additional description.

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