Skip to main content Accessibility help
  • Get access
    Check if you have access via personal or institutional login
  • Cited by 2
  • Print publication year: 2004
  • Online publication date: July 2009

8 - Neurocutaneous melanosis


Neurocutaneous melanosis (NCM) is a neurodevelopmental disorder of melanotic cell development and migration (Rokitansky, 1861). The disorder has been described by a confusing array of names (giant melanocytic nevus, melanose neurocutanee, dark hairy nevus syndrome, congenital melanocytic hairy nevi, giant hairy nevus, garment hairy pigmented nevus, nevus pigmentosus et pillosus, nevomelanocytic nevus, melanotic nevus, Becker hairy nevus, and heredofamilial melanosis or Van Bogaert syndrome). The term NCM is used when there is abnormal melanocytic proliferation in the central nervous system, especially along the leptomeninges, in association with cutaneous dark nevi. Congenital melanocytic nevi may occur without central nervous system (CNS) involvement and conversely, melanin is found normally in the central nervous system in the absence of congenital nevi.

Neurocutaneous melanosis apparently occurs sporadically and affects males and females with equal frequency (DeDavid et al., 1996). The incidence of NCM is unknown but it is uncommon. Ascertainment of NCM is probably fairly complete because the characteristic and often dramatic skin lesions rarely escape notice.

Clinical features

Skin findings

The characteristic lesions are very dark to light brown hairy nevi (Fig. 8.1) that are present at birth. Multiple small nevi (‘satellite nevi’) usually occur around one giant nevus that most commonly appears on the lower trunk and perineal area (‘swimming trunk nevus’) or over the shoulders, upper arms and lower neck (‘cape nevus’). About a third of patients have a cape nevus. A giant nevus is absent in a substantial number of patients with NCM.

Betancourt, Y., Trocello, O., Sierra, L. & Jimenez, J. C. (1996). Neurocutaneous melanosis and hydrocephalus. Report of a case. Acta Neuropediatrica, 2: 264–269
Byrd, S. E., Darling, C. F., Tomita, T., Chou, P., Leon, G. A. & Radkowski, M. A. (1997). MR imaging of symptomatic neurocutaneous melanosis in children. Pediatric Radiology, 27: 39–44
Chaloupka, J. C., Wolf, R. J. & Varma, P. K. (1996). Neurocutaneous melanosis with the Dandy–Walker malformation: a possible rare pathoetiologic association. Neuroradiology, 38: 486–489
Craver, R. D., Golladay, S. E., Warrier, R. P., Gates, A. J. & Nelson, J. S. (1996). Neurocutaneous melanosis with Dandy–Walker malformation complicated by primary spinal leptomeningeal melanoma. Journal of Child Neurology, 11: 410–414
DeDavid, M., Orlow, S. J., Provost, N. et al. (1996). Neurocutaneous melanosis: clinical features of large congenital melanocytic nevi in patients with manifest central nervous system melanosis. Journal of the American Academy of Dermatology, 35: 529–538
Fox, H. (1972). Neurocutaneous melanosis. In Handbook of Clinical Neurology, vol. 14, ed. P. J. Vinken & G. W. Bruyn, Neurocutaneous Disorders, pp. 414–428. Amsterdam: North-Holland
Frieden, I. J., Williams, M. L. & Barkovich, A. J. (1994). Giant congenital melanocytic nevi: brain magnetic resonance findings in neurologically asymptomatic children. Journal of the American Academy of Dermatology, 31: 423–429
Green, L. J., Nanda, V. S., Roth, G. M. & Barr, R. J. (1997). Neurocutaneous melanosis and Dandy–Walker syndrome in an infant. International Journal of Dermatology, 36: 356–359
Hendrickson, M. R. & Ross, J. C. (1981). Neoplasms arising in congenital nevi. American Journal of Surgical Pathology, 5: 109–135
Jerden, M. M., Smith, R. R. L., Cohen, B. A. & Hood, A. F. (1985). Neuroectodermal neoplasms arising in congenital nevi. American Journal of Dermatopathology, 7 (Suppl.): 41–48
Kadonaga, J. N., Barkovich, A. J., Edwards, M. S. & Frieden, I. J. (1992). Neurocutaneous melanosis in association with the Dandy–Walker complex. Pediatric Dermatology, 9: 37–43
Kadonaga, J. N. & Frieden, I. J. (1991). Neurocutaneous melanosis: definition and review of the literature. Journal of the American Academy of Dermatology, 24: 747–755
Ko, S. F., Wang, H. S., Lui, T. N., Ng, S. H., Ho, Y. S. & Tsai, C. C. (1993). Neurocutaneous melanosis associated with inferior vermian hypoplasia: MR findings. Journal of Computed and Assisted Tomography, 17: 691–695
Leaney, B. J., Rowe, P. W. & Klug, G. L. (1985). Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report. Journal of Neurosurgery, 62: 148–152
Miller, V. S. & Roach, E. S. (2000). Neurocutaneous disorders. In Neurology in Clinical Practice, ed. W. G. Bradley, R. B. Daroff, G. M. Fenichel & C. D. Marsden. Butterworth Heinemann
Novotny, E. J. J. & Urich, H. (1986). The coincidence of neurocutaneous melanosis and encephalofacial angiomatosis. Clinical Neuropathology, 5: 246–251
Pascual-Castroviejo, I. (1987). Neurocutaneous melanosis. In Neurocutaneous Diseases, ed. M. Gomez, pp. 329–334. Stoneham, MA: Butterworth
Reyes-Mugica, M., Chou, P., Byrd, S., Ray, V., Castelli, M. & Gattuso, P. G.-C. F. (1993). Nevomelanocytic proliferations in the central nervous system of children. Cancer, 72: 2277–2285
Rokitansky, J. (1861). Ein ausgezeichneter Fall von Pigment-Mal mit ausgebreiteter Pigmentierung der inneren Hirn-und Rückenmarkshäute. Allg.Wien.Med.Ztg., 6: 113–116
Roth, M. J., Medeiros, J., Kapur, S. et al. (1993). Malignant schwannoma with melanocytic and neuroepithelial differentiation in an infant with congenital giant melanocytic nevus: a complex neurocristopathy. Human Pathology, 24: 1371–1375
Sasaki, Y., Kobayashi, S., Shimizu, H. & Nishikawa, T. (1996). Multiple nodular lesions seen in a patient with neurocutaneous melanosis. Journal of Dermatology, 23: 828–831
Heuzen, E. P., Kaiser, M. C. & Slegte, R. G. (1989). Neurocutaneous melanosis associated with intraspinal lipoma. Neuroradiology, 31: 349–351
Zuniga, S., Las, H. J. & Benveniste, S. (1987). Rhabdomyosarcoma arising in a congenital giant nevus associated with neurocutaneous melanosis in a neonate. Journal of Pediatric Surgery, 22: 1036–1038