Skip to main content
SpringerLink
Log in

Congenital Melanocytic Nevi

  • Chapter
  • First Online:
Pediatric Skin of Color

  • 1244 Accesses

Abstract

Pigmentary nevi are the most common skin neoplasm found in humans with an incidence of 3 % in whites and 16 % in black infants at birth. The incidence of melanocytic nevi increases throughout infancy and adulthood, as well as at puberty, during pregnancy and with hormonal therapy. Congenital melanocytic nevi (CMN) are defined as neural crest derived benign nevomelanocytic proliferations that are present at birth or in the few weeks of life. They are genetically determined and persist throughout life. Prevalence of CMN is 1–2.4 % of newborns; African and Japanese decent have higher incidence than Hispanics or whites and the incidence is 1:500,000. Prevalence of CMN is 1–2.4 % of newborns; African and Japanese decent have a higher incidence than Hispanics or whites and the incidence is 1:500,000. A new classification for determining risks of adverse events in CMN includes the following criteria: satellite nevus count, anatomic localization, color heterogeneity, surface, rugosity, hypertrichosis, and dermal or subcutaneous nodules. They are more frequent on the trunk and extremities, brown-black in color, with small nodules and coarse hair, with a regular smooth and well demarcated border. Satellite melanocytic nevi are common. Proliferative nodules may appear within the nevus, which usually represent benign neurotization of the lesion.

By dermatoscopy, the main pattern seen in congenital nevi is the so-called cobblestone pattern, constituted of large, angulated globules, resembling cobblestones. Excision of CMN before the development of malignant melanoma (MM) should be considered to reduce the chances of malignant melanoma development and cosmetic reasons, although it is technically difficult, and complete removal is often impossible.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 149.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Happle R. What is a nevus? A proposed definition of a common medical term. Dermatology. 1995;191:1–5.

    Article  CAS  PubMed  Google Scholar 

  2. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics. 1976;58:218–22.

    CAS  PubMed  Google Scholar 

  3. Walton RG, Jacobs AH, Cox AJ. Pigmented lesions in newborn infants. Br J Dermatol. 1976;95:389–96.

    Article  CAS  PubMed  Google Scholar 

  4. Alikhan A, Ibrahimi OA, Eisen DB. Congenital melanocytic nevi. Where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 2012;67:496 e1–17.

    Article  Google Scholar 

  5. Tannous ZS, Mihm MC, Sober AJ, Duncan LM. Congenital melanocytic nevi: clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol. 2005;52:197–203.

    Article  PubMed  Google Scholar 

  6. Krengel S. Nevogenesis—new thoughts regarding a classical problem. Am J Dermatopathol. 2005;27:456–65.

    Article  PubMed  Google Scholar 

  7. Kinsler VA, Thomas AC, Ishida M, et al. Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol. 2013;133:2229–36.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Happle R. Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin. J Am Acad Dermatol. 1987;16:899–906.

    Article  CAS  PubMed  Google Scholar 

  9. Hamm H, Höger PH. Skin tumors in childhood. Dtsch Arztebl Int. 2011;108:347–53.

    PubMed Central  PubMed  Google Scholar 

  10. Marghoob AA, Schoenbach SP, Kopf AW, Orlow SJ, Nossa R, Bart RS. Large congenital melanocytic nevi and the risk for the development of malignant melanoma: a prospective study. Arch Dermatol. 1996;132:170–5.

    Article  CAS  PubMed  Google Scholar 

  11. Marghoob AA. Congenital melanocytic nevi. Evaluation and management. Dermatol Clin. 2002;20:607–16.

    Article  PubMed  Google Scholar 

  12. Krengel S, Scope A, Dusza SW, Vonthein R, Marghoob AA. New recommendations for the categorization of cutaneous features of congenital melanocytic nevi. J Am Acad Dermatol. 2013;68:441–51.

    Article  PubMed  Google Scholar 

  13. Ruiz-Maldonado R. Measuring congenital melanocytic nevi. Pediatr Dermatol. 2004;2:178–9.

    Article  Google Scholar 

  14. Moscarella E, Piccolo V, Argenziano G, et al. Problematic lesions in children. Dermatol Clin. 2013;31:535–47.

    Article  CAS  PubMed  Google Scholar 

  15. Kroon S, Clemmensen OJ, Hastrup N. Incidence of congenital melanocytic nevi in newborn babies in Denmark. J Am Acad Dermatol. 1987;17:422–6.

    Article  CAS  PubMed  Google Scholar 

  16. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. 2005;52:793–7.

    Article  PubMed  Google Scholar 

  17. Castilla EE, da Graca DM, Orioli-Parreiras IM. Epidemiology of congenital pigmented naevi: II. Risk factors. Br J Dermatol. 1981;104:421–7.

    Article  CAS  PubMed  Google Scholar 

  18. Warner PM, Yakuboff KP, Kagan RJ, Boyce S, Warden GD. An 18-year experience in the management of congenital nevomelanocytic nevi. Ann Plast Surg. 2008;60:283–7.

    Article  CAS  PubMed  Google Scholar 

  19. Kanada KN, Merin MR, Munden A, Friendlander SF. A prospective study of cutaneous findings in newborns in the United States: correlation with race, ethnicity, and gestational status using updated classification and nomenclature. J Pediatr. 2012;161:240–5.

    Article  PubMed  Google Scholar 

  20. Alper JC, Holmes LB. The incidence and significance of birthmarks in a cohort of 4641 newborns. Pediatr Dermatol. 1983;1:58–68.

    Article  CAS  PubMed  Google Scholar 

  21. Dickson J, Yue P. A study of the incidence of neonatal dermatoses. Australas J Dermatol. 1979;20:78–81.

    Article  CAS  PubMed  Google Scholar 

  22. Hidano A, Purwoko R, Jitsukawa K. Statistical survey of skin changes in Japanese neonates. Pediatr Dermatol. 1986;3:140–4.

    Article  CAS  PubMed  Google Scholar 

  23. Kahana M, Feldman M, Abudi Z, Yurman S. The incidence of birthmarks in Israeli neonates. Int J Dermatol. 1995;34:704–6.

    Article  CAS  PubMed  Google Scholar 

  24. Karvonen SL, Vaajalahti P, Marenk M, Janas M, Kuokkanen K. Birthmarks in 4346 Finnish newborns. Acta Derm Venereol. 1992;72:55–7.

    CAS  PubMed  Google Scholar 

  25. Magana-Garcia M, Gonzalez-Campos N. Skin lesions in 1000 newborns: a case-control study. Gac Med Mex. 1997;133:407–11.

    CAS  PubMed  Google Scholar 

  26. Nanda A, Kaur S, Bhakoo ON, Dhall K. Survey of cutaneous lesions in Indian newborns. Pediatr Dermatol. 1989;6:39–42.

    Article  CAS  PubMed  Google Scholar 

  27. Shih IH, Lin JY, Chen CH, Hong HS. A birthmark survey in 500 newborns: clinical observation in two northern Taiwan medical center nurseries. Chang Gung Med J. 2007;30:220–5.

    PubMed  Google Scholar 

  28. Tsai FJ, Tsai CH. Birthmarks and congenital skin lesions in Chinese newborns. J Formos Med Assoc. 1993;92:838–41.

    CAS  PubMed  Google Scholar 

  29. Hurwitz S. Cutaneous tumors in childhood. In: Hurwitz S, editor. Clinical pediatric dermatology. 2nd ed. Philadelphia: WB Saunders Co; 1993. p. 203–6.

    Google Scholar 

  30. Zitelli JA. Congenital nevocytic nevi. In: Demis DJ, editor. Clinical dermatology. 26th revision, Vol 2; Section 11–48. Philadelphia, PA: Lippincott-Raven; 1999. pp. 1–9.

    Google Scholar 

  31. Mark GJ, Mihm MC, Liteplo MG, Reed RJ, Clark WH. Congenital melanocytic nevi of the small and garment type. Clinical, histologic, and ultrastructural studies. Hum Pathol. 1973;4:395–418.

    Article  CAS  PubMed  Google Scholar 

  32. Mizushima J, Nogita T, Higaki Y, Horikoshi T, Kawashima M. Dormant melanocytes in the dermis: do dermal melanocytes of acquired dermal melanocytosis exist from birth? Br J Dermatol. 1998;139:349–50.

    Article  CAS  PubMed  Google Scholar 

  33. Drakensjö IT, Hedblad MA, Wahlgren CF. Rapidly involuting congenital melanocytic naevi in two children. Acta Derm Venereol. 2012;92:585–663.

    Article  Google Scholar 

  34. Strauss RM, Newton Bishop JA. Spontaneous involution of congenital melanocytic nevi of the scalp. J Am Acad Dermatol. 2008;58:508–11.

    Article  PubMed  Google Scholar 

  35. Sotiriou E, Apalla Z, Panagiotidou D, Ioannides D. Partial pigmentary regression of congenital nevocytic naevus without halo phenomenon followed by generalized vitiligo. J Eur Acad Dermatol Venereol. 2009;23:600–1.

    Article  CAS  PubMed  Google Scholar 

  36. Cusack C, Warde D, Lynch W, McDermott M, Watson R. Dramatic spontaneous regression of a medium-sized congenital melanocytic naevus. Clin Exp Dermatol. 2009;34:34–6.

    Article  Google Scholar 

  37. Nath AK, Thappa DM, Rajesh NG. Spontaneous regression of a congenital melanocytic nevus. Indian J Dermatol Venereol Leprol. 2011;77:507–10.

    Article  PubMed  Google Scholar 

  38. Itin PH, Lautenschlager S. Acquired leukoderma in congenital pigmented nevus associated with vitiligo-like depigmentation. Pediatr Dermatol. 2002;19:73–5.

    Article  PubMed  Google Scholar 

  39. Vilarrasa E, Baselga E, Rincon C, Alomar A. Histologic persistence of a congenital melanocytic nevus of the scalp despite clinical involution. J Am Acad Dermatol. 2008;59:1091–2.

    Article  PubMed  Google Scholar 

  40. Tokura Y, Yamanaka K, Wakita H, et al. Halo congenital nevus undergoing spontaneous regression: involvement of T-cell immunity in the involution and presence of circulating anti-nevus cell IgM antibodies. Arch Dermatol. 1994;130:1036–41.

    Article  CAS  PubMed  Google Scholar 

  41. Zack LD, Stegmeier O, Solomon LM. Pigmentary regression in a giant nevocellular nevus: a case report and a review of the subject. Pediatr Dermatol. 1988;5:178–83.

    Article  CAS  PubMed  Google Scholar 

  42. Shin JH, Kim MJ, Cho S, Whang KK, Hahm JH. A case of giant congenital nevocytic nevus with neurotization and onset of vitiligo. J Eur Acad Dermatol Venereol. 2002;16:384–6.

    Article  CAS  PubMed  Google Scholar 

  43. Hogan DJ, Murphy F, Bremner RM. Spontaneous resolution of a giant congenital melanocytic nevus. Pediatr Dermatol. 1988;5:170–2.

    Article  CAS  PubMed  Google Scholar 

  44. Ruiz-Maldonado R. Desmoplastic hairless hypopigmented naevus: a variant of giant congenital melanocytic naevus. Br J Dermatol. 2003;148:1253–7.

    Article  CAS  PubMed  Google Scholar 

  45. Boente M, Asial R. Desmoplastic hairless hypopigmented nevus (DHHN). A distinct variant of giant melanocytic nevus. Eur J Dermatol. 2005;15(6):451–3.

    Google Scholar 

  46. Gass JK, Grant JW, Hall PN, Atherton DJ, Burrows NP. Clinical resolution of a neonatally eroded giant congenital melanocytic nevus. Pediatr Dermatol. 2006;23:567–70.

    Article  PubMed  Google Scholar 

  47. Hamming N. Anatomy and embryology of the eyelids: a review with special reference to the development of divided nevi. Pediatr Dermatol. 1983;1:51–8.

    Article  CAS  PubMed  Google Scholar 

  48. Barnhill RL, Fleischli M. Histologic features of congenital melanocytic nevi in infants 1 year of age or younger. J Am Acad Dermatol. 1995;33:780–5.

    Article  CAS  PubMed  Google Scholar 

  49. Clemmensen OJ, Kroon S. The histology of “congenital features” in early-acquired melanocytic nevi. J Am Acad Dermatol. 1988;19:742–6.

    Article  CAS  PubMed  Google Scholar 

  50. Haliasos EC, Kermer M, Jaimes N, et al. Dermoscopy for the pediatric dermatologist part III: Dermoscopy of melanocytic lesions. Pediatr Dermatol. 2013;30:281–93.

    Article  PubMed  Google Scholar 

  51. Ibrahimi OA, Alikhan A, Eisen DB. Congenital melanocytic nevi: Where are we now? Part II. Treatment options and approach to treatment. J Am Acad Dermatol. 2012;67:515e1–13.

    Article  Google Scholar 

  52. Pavlidou E, Hagel C, Papavasilliou A, Giouroukos S, Panteliadis C. Neurocutaneous melanosis: report of three cases and up-to date review. J Child Neurol. 2008;23:1382–91.

    PubMed  Google Scholar 

  53. Faillace WJ, Okawara SH, McDonald JV. Neurocutaneous melanosis with extensive intracerebral and spinal cord involvement. Report of two cases. J Neurosurg. 1984;61:782–5.

    Article  CAS  PubMed  Google Scholar 

  54. DeDavid M, Orlow SJ, Provost N, Marghoob AA, Rao BK, Wasti Q, et al. Neurocutaneous melanosis: clinical features of large congenital melanocytic nevi in patients with manifest central nervous system melanosis. J Am Acad Dermatol. 1996;35:529–38.

    Article  CAS  PubMed  Google Scholar 

  55. Fox H, Emery JL, Goodbody RA, Yates PO. Neuro-cutaneous melanosis. Arch Dis Child. 1964;39:508–16.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  56. Miranda P, Esparza J, Hinojosa J, Munoz A. Neurocutaneous melanosis and congenital melanocytic nevus in the head. Pediatr Neurosurg. 2005;41:109–11.

    Article  CAS  PubMed  Google Scholar 

  57. Castellano E, Downward J. RAS interaction with PI3K: more than just another effector pathway. Genes Cancer. 2011;2:261–74.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  58. Pylayeva-Gupta Y, Grabocka E, Bar-Sagi D. RAS oncogenes: weaving a tumorigenic web. Nat Rev Cancer. 2011;11:761–74.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  59. Tidyman WE, Rauen KA. The RASopathies: developmental syndromes of Ras/MAPK pathway dysregulation. Curr Opin Genet Dev. 2009;19:230–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  60. Kinsler VA, Abu-Amero S, Budd P, et al. Germline melanocortin-1 receptor genotype is associated with severity of cutaneous phenotype in congenital melanocytic nevi: a role for MC1R in human fetal development. J Invest Dermatol. 2012;132:2026–32.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  61. Groesser L, Herschberger E, Ruetten A, et al. Postzygotic HRAS and KRAS mutations cause nevus sebaceous and Schimmelpenning syndrome. Nat Genet. 2012;44:783–7.

    Article  CAS  PubMed  Google Scholar 

  62. Kinsler V, Shaw AC, Merks JH, et al. The face in congenital melanocytic nevus syndrome. Am J Med Genet A. 2012;158A:1014–9.

    Article  PubMed  Google Scholar 

  63. Zenker M. Clinical manifestations of mutations in RAS and related intracellular signal transduction factors. Curr Opin Pediatr. 2011;23:443–51.

    Article  CAS  PubMed  Google Scholar 

  64. Marghoob AA, Charles CA, Busam KJ, et al. In vivo confocal scanning laser microscopy of a series of congenital melanocytic nevi suggesting of having developed malignant melanoma. Arch Dermatol. 2005;141:1401–12.

    PubMed  Google Scholar 

  65. Seidenari S, Pellacani G. Surface microscopy features of congenital nevi. Clin Dermatol. 2002;20:263–7.

    Article  PubMed  Google Scholar 

  66. Illig L, Weidner F, Hundeiker M, et al. Congenital nevi less than or equal to 10 cm as precursors to melanoma: 52 cases, a review, and a new conception. Arch Dermatol. 1985;121:1274–81.

    Article  CAS  PubMed  Google Scholar 

  67. Marghoob AA, Orlow SJ, Kopf AW. Syndromes associated with melanocytic nevi. J Am Acad Dermatol. 1993;29:373–88.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Hospital del Niño Jesús, Tucumán, Argentina

    María del Carmen Boente M.D.

Authors
  1. María del Carmen Boente M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to María del Carmen Boente M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Mt. Sinai St. Luke’s-Roosevelt Hospital and Beth Israel Medical centers, New York, New York, USA

    Nanette B. Silverberg

  2. Department of Pediatric Dermatology, National Institute of Pediatrics, Mexico City, Mexico

    Carola Durán-McKinster

  3. Department of Dermatology, Changi General Hospital, Singapore, Singapore

    Yong-Kwang Tay

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media New York

About this chapter

Cite this chapter

del Carmen Boente, M. (2015). Congenital Melanocytic Nevi. In: Silverberg, N., Durán-McKinster, C., Tay, YK. (eds) Pediatric Skin of Color. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6654-3_27

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-6654-3_27

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6653-6

  • Online ISBN: 978-1-4614-6654-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation