[Neurocutaneous melanosis]

Neurocutaneous melanosis is a sporadic neurocutaneous syndrome characterized by the presence of giant or multiple melanocytic nevi and excessive proliferation of melanotic cells in the central nervous system. Patients usually consult dermatologists because the congenital cutaneous nevi. Most neurologic symptoms appear in the first two years of life and the prognosis in such cases is poor, especially if they are caused by hydrocephalus, which is the main complication. All patients with giant or multiple melanocytic nevi should be seen frequently to detect promptly the eventual development of neurologic involvement.