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Neurocutaneous melanocytosis

ORPHA:2481· ICD-10 D22.4

Definition

Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterized by abnormal aggregations of nevomelanocytes within the central nervous system (leptomeningeal melanocytosis) associated with large or giant congenital melanocytic nevi (CMN). NCM can be asymptomatic or present as variably severe and progressive neurological impairment, sometimes resulting in death.

Prevalence
1-9 / 100 000
Inheritance
Not applicable
Age of onset
Childhood