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Melanosis Neurocutanea

Rare condition characterized by extensive congenital hairy nevocytic nevi, often in the bathing trunk region, a dense scattering of smaller, pigmented nevocytic nevi, especially on the palms and soles, and similar aggregations of pigment-forming naevus cells in the meninges, brain, and spinal cord. Complications arise from the development of an obstructive internal hydrocephalus. A metastasising melanoma may also arise in the skin or CNS, accounting for the poor prognosis of the disorder.

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