title: Neurocutaneous melanocytosis.
abstract: Neurocutaneous melanosis or neurocutaneous melanocytosis is a rare sporadic congenital disorder characterized by the presence of giant and/or multiple satellite congenital melanocytic nevi in the skin and benign melanocytic pigmentation of the leptomeninges. These two defining features were recognized more than a century ago. A third characteristic feature is proliferative nodules arising from giant nevi. The etiology is unknown, but neurocutaneous melanosis is considered a developmental disorder of melanocyte precursors from neural crest. The distinctive unique distribution of the congenital giant nevi that gives a "garment" appearance is also an expression of the neural crest. The neurological manifestations often appear in infancy.The special association of neurocutaneous melanosis with Dandy-Walker malformation complex may be explained by a common pathogenesis. Mortality in infancy and childhood is high.
authors.affiliation: Departments of Clinical Neurosciences and Paediatrics, Division of Paediatric Neurology, University of Calgary, Alberta Children's Hospital, Calgary, Canada. Electronic address: laura.flores-sarnat@albertahealthservices.ca.
authors.forename: Laura
authors.initials: L
authors.lastname: Flores-Sarnat
mesh_terms: D001921:Brain / Q000473:pathology; D003616:Dandy-Walker Syndrome / Q000150:complications*; D049672:History, 19th Century; D006801:Humans; D008548:Melanosis / Q000150:complications / Q000175:diagnosis* / Q000266:history / Q000628:therapy*; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000175:diagnosis* / Q000266:history / Q000628:therapy*