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Congenital melanocytic nevi

Authors
Julie V Schaffer, MD
Jean L Bolognia, MD
Section Editors
Moise L Levy, MD
Hensin Tsao, MD, PhD
Deputy Editor
Rosamaria Corona, MD, DSc

INTRODUCTION

Melanocytic nevi (moles) represent benign proliferations of a type of melanocyte known as a "nevus cell." The two major differences between ordinary melanocytes that reside in the basal layer of the epidermis and nevus cells are:

Nevus cells cluster as nests within the lower epidermis and/or dermis, whereas epidermal melanocytes are evenly dispersed as single units

Nevus cells do not have dendritic processes (with the exception of those within blue nevi)

Both melanocytes and nevus cells are capable of producing the pigment melanin. Melanocytic nevi can be congenital or acquired. Congenital melanocytic nevi (CMN) are classically defined as melanocytic nevi present at birth or within the first few months of life.

CMN and speckled lentiginous nevi (a subtype of CMN) will be discussed below. Acquired melanocytic nevi and other benign pigmented skin lesions are discussed separately. (See "Acquired melanocytic nevi (moles)" and "Benign pigmented skin lesions other than melanocytic nevi (moles)".)

                  

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Literature review current through: Nov 2016. | This topic last updated: Tue Oct 14 00:00:00 GMT+00:00 2014.
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References
Top
  1. Kanada KN, Merin MR, Munden A, Friedlander 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.
  2. Castilla EE, da Graça Dutra M, Orioli-Parreiras IM. Epidemiology of congenital pigmented naevi: I. Incidence rates and relative frequencies. Br J Dermatol 1981; 104:307.
  3. Tannous ZS, Mihm MC Jr, Sober AJ, Duncan LM. Congenital melanocytic nevi: clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol 2005; 52:197.
  4. Price HN, Schaffer JV. Congenital melanocytic nevi-when to worry and how to treat: Facts and controversies. Clin Dermatol 2010; 28:293.
  5. Ichii-Nakato N, Takata M, Takayanagi S, et al. High frequency of BRAFV600E mutation in acquired nevi and small congenital nevi, but low frequency of mutation in medium-sized congenital nevi. J Invest Dermatol 2006; 126:2111.
  6. Wu J, Rosenbaum E, Begum S, Westra WH. Distribution of BRAF T1799A(V600E) mutations across various types of benign nevi: implications for melanocytic tumorigenesis. Am J Dermatopathol 2007; 29:534.
  7. Dessars B, De Raeve LE, Morandini R, et al. Genotypic and gene expression studies in congenital melanocytic nevi: insight into initial steps of melanotumorigenesis. J Invest Dermatol 2009; 129:139.
  8. Charbel C, Fontaine RH, Malouf GG, et al. NRAS mutation is the sole recurrent somatic mutation in large congenital melanocytic nevi. J Invest Dermatol 2014; 134:1067.
  9. 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.
  10. Mark GJ, Mihm MC, Liteplo MG, et al. Congenital melanocytic nevi of the small and garment type. Clinical, histologic, and ultrastructural studies. Hum Pathol 1973; 4:395.
  11. Precursors to malignant melanoma. National Institutes of Health Consensus Development Conference Statement, Oct. 24-26, 1983. J Am Acad Dermatol 1984; 10:683.
  12. Rabinovitz HS, Barnhill RL. Benign melanocytic neoplasms. In: Dermatology, 3rd ed, Bolognia JL, Jorizzo JL, Schaffer JV (Eds), Elsevier-Saunders, Philadelphia 2012.
  13. Kokta V, Hung T, Al Dhaybi R, et al. High prevalence of angiotropism in congenital melanocytic nevi: an analysis of 53 cases. Am J Dermatopathol 2013; 35:180.
  14. Makkar HS, Frieden IJ. Congenital melanocytic nevi: an update for the pediatrician. Curr Opin Pediatr 2002; 14:397.
  15. Krengel S, Scope A, Dusza SW, et al. New recommendations for the categorization of cutaneous features of congenital melanocytic nevi. J Am Acad Dermatol 2013; 68:441.
  16. Haliasos EC, Kerner M, Jaimes N, et al. Dermoscopy for the pediatric dermatologist part III: dermoscopy of melanocytic lesions. Pediatr Dermatol 2013; 30:281.
  17. Changchien L, Dusza SW, Agero AL, et al. Age- and site-specific variation in the dermoscopic patterns of congenital melanocytic nevi: an aid to accurate classification and assessment of melanocytic nevi. Arch Dermatol 2007; 143:1007.
  18. Seidenari S, Pellacani G, Martella A, et al. Instrument-, age- and site-dependent variations of dermoscopic patterns of congenital melanocytic naevi: a multicentre study. Br J Dermatol 2006; 155:56.
  19. Ingordo V, Iannazzone SS, Cusano F, Naldi L. Dermoscopic features of congenital melanocytic nevus and Becker nevus in an adult male population: an analysis with a 10-fold magnification. Dermatology 2006; 212:354.
  20. Minagawa A, Koga H, Saida T. Dermoscopic characteristics of congenital melanocytic nevi affecting acral volar skin. Arch Dermatol 2011; 147:809.
  21. Marghoob AA, Schoenbach SP, Kopf AW, et al. Large congenital melanocytic nevi and the risk for the development of malignant melanoma. A prospective study. Arch Dermatol 1996; 132:170.
  22. Rhodes AR, Albert LS, Weinstock MA. Congenital nevomelanocytic nevi: proportionate area expansion during infancy and early childhood. J Am Acad Dermatol 1996; 34:51.
  23. Ruiz-Maldonado R, Tamayo L, Laterza AM, Durán C. Giant pigmented nevi: clinical, histopathologic, and therapeutic considerations. J Pediatr 1992; 120:906.
  24. Strauss RM, Newton Bishop JA. Spontaneous involution of congenital melanocytic nevi of the scalp. J Am Acad Dermatol 2008; 58:508.
  25. Mundinger GS. Images in clinical medicine. Halo phenomenon. N Engl J Med 2014; 370:262.
  26. Rolland S, Kokta V, Marcoux D. Meyerson phenomenon in children: observation in five cases of congenital melanocytic nevi. Pediatr Dermatol 2009; 26:292.
  27. Phadke PA, Rakheja D, Le LP, et al. Proliferative nodules arising within congenital melanocytic nevi: a histologic, immunohistochemical, and molecular analyses of 43 cases. Am J Surg Pathol 2011; 35:656.
  28. Watt AJ, Kotsis SV, Chung KC. Risk of melanoma arising in large congenital melanocytic nevi: a systematic review. Plast Reconstr Surg 2004; 113:1968.
  29. Vourc'h-Jourdain M, Martin L, Barbarot S, aRED. Large congenital melanocytic nevi: therapeutic management and melanoma risk: a systematic review. J Am Acad Dermatol 2013; 68:493.
  30. Kinsler VA, Birley J, Atherton DJ. Great Ormond Street Hospital for Children Registry for congenital melanocytic naevi: prospective study 1988-2007. Part 1-epidemiology, phenotype and outcomes. Br J Dermatol 2009; 160:143.
  31. Foster RD, Williams ML, Barkovich AJ, et al. Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children. Plast Reconstr Surg 2001; 107:933.
  32. 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; 67:495.e1.
  33. Ramaswamy V, Delaney H, Haque S, et al. Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol 2012; 54:563.
  34. DeDavid M, Orlow SJ, Provost N, 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.
  35. Kinsler VA, Chong WK, Aylett SE, Atherton DJ. Complications of congenital melanocytic naevi in children: analysis of 16 years' experience and clinical practice. Br J Dermatol 2008; 159:907.
  36. Lovett A, Maari C, Decarie JC, et al. Large congenital melanocytic nevi and neurocutaneous melanocytosis: one pediatric center's experience. J Am Acad Dermatol 2009; 61:766.
  37. Marghoob AA, Dusza S, Oliveria S, Halpern AC. Number of satellite nevi as a correlate for neurocutaneous melanocytosis in patients with large congenital melanocytic nevi. Arch Dermatol 2004; 140:171.
  38. Krengel S, Breuninger H, Beckwith M, Etchevers HC. Meeting report from the 2011 International Expert Meeting on Large Congenital Melanocytic Nevi and Neurocutaneous Melanocytosis, Tübingen. Pigment Cell Melanoma Res 2011; 24:E1.
  39. Di Rocco F, Sabatino G, Koutzoglou M, et al. Neurocutaneous melanosis. Childs Nerv Syst 2004; 20:23.
  40. Schaffer JV, McNiff JM, Bolognia JL. Cerebral mass due to neurocutaneous melanosis: eight years later. Pediatr Dermatol 2001; 18:369.
  41. Agero AL, Benvenuto-Andrade C, Dusza SW, et al. Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi: a study of cases from an Internet-based registry. J Am Acad Dermatol 2005; 53:959.
  42. Hoang MP, Sinkre P, Albores-Saavedra J. Rhabdomyosarcoma arising in a congenital melanocytic nevus. Am J Dermatopathol 2002; 24:26.
  43. Gosain AK, Santoro TD, Larson DL, Gingrass RP. Giant congenital nevi: a 20-year experience and an algorithm for their management. Plast Reconstr Surg 2001; 108:622.
  44. De Raeve LE, Claes A, Ruiter DJ, et al. Distinct phenotypic changes between the superficial and deep component of giant congenital melanocytic naevi: a rationale for curettage. Br J Dermatol 2006; 154:485.
  45. De Raeve LE, Roseeuw DI. Curettage of giant congenital melanocytic nevi in neonates: a decade later. Arch Dermatol 2002; 138:943.
  46. Ostertag JU, Quaedvlieg PJ, Kerckhoffs FE, et al. Congenital naevi treated with erbium:YAG laser (Derma K) resurfacing in neonates: clinical results and review of the literature. Br J Dermatol 2006; 154:889.
  47. Kishi K, Matsuda N, Kubota Y, et al. Rapid, severe repigmentation of congenital melanocytic naevi after curettage and dermabrasion: histological features. Br J Dermatol 2007; 156:1251.
  48. Sohn S, Kim S, Kang WH. Recurrent pigmented macules after q-switched alexandrite laser treatment of congenital melanocytic nevus. Dermatol Surg 2004; 30:898.
  49. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol 2005; 52:793.
  50. Streams BN, Lio PA, Mihm MC, Sober AJ. A nonepidermal, primary malignant melanoma arising in a giant congenital melanocytic nevus 40 years after partial surgical removal. J Am Acad Dermatol 2004; 50:789.
  51. Rhodes AR, Wood WC, Sober AJ, Mihm MC Jr. Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus. Plast Reconstr Surg 1981; 67:782.
  52. Happle R. Speckled lentiginous naevus: which of the two disorders do you mean? Clin Exp Dermatol 2009; 34:133.
  53. Schaffer JV, Orlow SJ, Lazova R, Bolognia JL. Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 2001; 137:172.