Congenital Nevi

Living reference work entry


This chapter provides a review of different types of congenital lesions of the skin including their natural history, diagnosis, classification, and treatment options. Most lesions can be approached surgically through a variety of techniques. This chapter aims to provide a familiarity with the diagnosis and help the reader develop a management strategy which satisfies both a functional and aesthetic outcome for these patients.


Congenital nevi Blue nevi Café au lait spots Malignancy 


  1. Adler N, Margulis A, Bauer BS. Congenital pigmented nevi of the auricle: clinical experience and approach to treatment. Plast Reconstr Surg. 2009a;124(6):1932–9.PubMedGoogle Scholar
  2. Adler N, Dorafshar AH, Bauer BS, Hoadley S, Tournell M. Tissue expander infections in pediatric patients: management and outcomes. Plast Reconstr Surg. 2009b;124(2):484–9.PubMedGoogle Scholar
  3. Agero AL, Benvenuto CA, Dusza SW, Halpern AC, Marghoob AA. Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi: a study of cases from an Internet-based registry. J Am Acad Dermatol. 2005;53:959–65.PubMedGoogle Scholar
  4. Alkureishi LWT, Purnell CA, Park P, et al. Long-term outcomes after pediatric free flap reconstruction. Ann Plast Surg. 2018;81(4):449–55.PubMedGoogle Scholar
  5. Alper JC, Holmes LB. The incidence and significance of birthmarks in a cohort of 4.641 newborns. Pediatr Dermatol. 1983;1:58–68.PubMedGoogle Scholar
  6. Alper JC, Holmes LB, Mihm MC. Birthmarks with serious medical significance: nevocellular nevi, sebaceous nevi and multiple café au lait spots. J Pediatr. 1979;95:696–700.PubMedGoogle Scholar
  7. Alster TS. Complete elimination of large café au lait birthmarks by the 510-nm pulsed dye laser. Plast Reconstr Surg. 1995;96:1660–4.PubMedGoogle Scholar
  8. Barkovich AJ, Frieden IJ, Williams ML. MR of neurocutaneous melanosis. Am J Neuroradiol. 1994;15:859–67.PubMedGoogle Scholar
  9. Bauer BS, Margulis A. The expanded transposition flap: shifting paradigms based on experience gained from two decades of pediatric tissue expansion. Plast Reconstr Surg. 2004;114(1):98–106.PubMedGoogle Scholar
  10. Bauer BS, Vicari FA. An approach to excision of congenital giant pigmented nevi in infancy and early childhood. Plast Reconstr Surg. 1988;82:1012–21.PubMedGoogle Scholar
  11. Berg P, Lindelof B. Congenital melanocytic naevi and cutaneous melanoma. Melanoma Res. 2003;13:441–5.PubMedGoogle Scholar
  12. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of neurocutaneous melanocytosis in 1008 persons. J Am Acad Dermatol. 2006;54:767–77.PubMedGoogle Scholar
  13. Bittencourt FV, Marghoob AA, Kopf AW, Koenig KL, Bart RS. Large congenital melanocytic nevi and the risk for development of malignant melanoma and neurocutaneous melanocytosis. Pediatrics. 2000;106:736–41.PubMedGoogle Scholar
  14. Boyce S, Alster TS. CO2 laser treatment of epidermal nevi: long-term success. Dermatol Surg. 2002;28(7):611–4.PubMedGoogle Scholar
  15. Carpo BG, Grevelink JM, Grevelink SV. Laser treatment of pigmented lesions in children. Semin Cutan Med Surg. 1999;18:233–43.PubMedGoogle Scholar
  16. 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(4):1067–74.PubMedGoogle Scholar
  17. Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceous: a study of 596 cases. J Am Acad Dermatol. 2000;42(2):263–8.PubMedGoogle Scholar
  18. Dragieva G, Hafner J, Kunzi W, Dummer R, Meuli M, Stiefel D, Schiestl C. Malignant melanoma in a large congenital melanocytic nevus 9 years after dermabrasion in childhood. Dermatology. 2006;212(2):208–9.PubMedGoogle Scholar
  19. Elder D, Elenitsas R. Benign pigmented lesions and malignant melanoma. In: Elder D, editor. Lever’s histopathology of the skin. 8th ed. Philadelphia: Lippincott-Raven Publishers; 1997.Google Scholar
  20. Illig L, Weidner F, Hundeiker ME. 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.PubMedGoogle Scholar
  21. Jones E, Heyl T. Naevus Sebaceous. A report of 140 cases with special regard to the development of secondary malignant tumours. Br J Dermatol. 1970;82:99–117.PubMedGoogle Scholar
  22. Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol. 1991;24:747–55.PubMedGoogle Scholar
  23. Kopf AW, Bart RS, Hennessey P. Congenital nevocytic nevi and malignant melanomas. J Am Acad Dermatol. 1979;1:123–30.PubMedGoogle Scholar
  24. Krengel S, Hauschild A, Schafer T. Melanoma risk in congenital melanocytic naevi: a systematic review. Br J Dermatol. 2006;155:1–8.PubMedGoogle Scholar
  25. Krengel S, Scope A, Dusza S, Vonthein R, Marghoob A. New recommendations for the categorization of cutaneous features of congenital melanocytic nevi. J Am Acad Dermatol. 2013;68(3):441–51.PubMedGoogle Scholar
  26. Kryger ZB, Bauer BS. Surgical management of large and giant congenital pigmented nevi of the lower extremity. Plast Reconstr Surg. 2008;121(5):1674–84.PubMedGoogle Scholar
  27. Lim Y, Shin HT, Choi Y, et al. Evolutionary processes of melanomas from giant congenital melanocytic nevi. Pigment Cell Melanoma Res. 2019.
  28. Magalon G, Casanova D, Bardot J, Andrac-Meyer L. Early curettage of giant congenital naevi in children. Br J Dermatol. 1998;138:341–5.PubMedGoogle Scholar
  29. Marghoob AA. The challenge of managing congenital melanocytic nevi: remove or not remove? Presented at: 2013 International expert meeting on large congential melanocytic nevi and neurocutaneous melanocytosis; 2013 Sept 28–30. Marseille.Google Scholar
  30. 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. Arch Dermatol. 1996;132:170–5.PubMedGoogle Scholar
  31. Marghoob AA, Kopf AW, Bittencourt FV. Moles present at birth: their medical significance. Skin Cancer Found J. 1999;36:95–8.Google Scholar
  32. Marghoob AA, Dusza S, Oliviera S, Halpern AC. Number of satellite nevi as a correlate for neurocutaneous melanocytosis in patients with large congenital melanocytic nevi. Arch Dermatol. 2004;140:171–5.PubMedGoogle Scholar
  33. Margulis A, Bauer BS, Corcoran JF. Surgical management of the cutaneous manifestations of linear nevus sebaceus syndrome. Plast Reconstr Surg. 2003;111(3):1043–50.PubMedGoogle Scholar
  34. Margulis A, Bauer BS, Fine NA. Large and giant congenital pigmented nevi of the upper extremity: an algorithm to surgical management. Ann Plast Surg. 2004;52(2):158–67.PubMedGoogle Scholar
  35. Margulis A, Adler N, Bauer BS. Congenital melanocytic nevi of the eyelids and periorbital region. Plast Reconstr Surg. 2009;124(4):1273–83.PubMedGoogle Scholar
  36. Mehregan A, Pinkus H. Life history of organoid nevi. Special reference to nevus Sebaceus of Jadassohn. Archs Dermatol. 1965;91:574.Google Scholar
  37. Michalowski R. Naevus Sebaceous of Jadassohn –a precancerous lesion. Dermatologica. 1962;124:326.PubMedGoogle Scholar
  38. Mosa A, Ho ES, Heinelt M, et al. Management of congenital melanocytic nevi in the plastic surgery clinic: families’ expectations and their persistent concern about malignancy. Pediatr Dermatol. 2019.
  39. Paradela S, Del Pozo J, Fernandez-Jorge B, Lozano J, Martinez-Gonzalez C, Fonseca E. Epidermal nevi treated by carbon dioxide laser vaporization: a series of 25 patients. J Dermatolog Treat. 2007;18(3):169–74.PubMedGoogle Scholar
  40. Quaba AA, Wallace AF. The incidence of malignant melanoma (0–15 years of age) arising in ‘large’ congenital nevocellular nevi. Plast Reconstr Surg. 1986;78:174–9.PubMedGoogle Scholar
  41. Ratz JL, Balain PL, Wheeland RG. Carbon dioxide laser treatment of epidermal nevi. J Dermatol Surg Oncol. 1986;12(6):567–70.PubMedGoogle Scholar
  42. Rhodes AR, Melski JW. Small cutaneous nevocellular nevi and the risk of cutaneous melanoma. J Pediatr. 1982;100:219–24.PubMedGoogle Scholar
  43. Rhodes AR, Silverman RA, Harrist TJ, Melski JW. A histologic comparison of congenital and acquired nevomelanocytic nevi. Arch Dermatol. 1986;121:1266–73.Google Scholar
  44. Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. 1989;20(3):476–88.PubMedGoogle Scholar
  45. Roh MR, Eliades P, Gupta S, et al. Genetics of melanocytic nevi. Pigment Cell Melanoma Res. 2015;28(6):661–72.PubMedPubMedCentralGoogle Scholar
  46. Sandsmark M, Eskeland G, Ogaard AR, et al. Treatment of large congenital naevi. Scand J Plast Reconstr Hand Surg. 1993;27:223–32.Google Scholar
  47. Scalzo DA, Hilda CA, Toth G, Sober AJ, Mihm MC. Childhood melanoma: a clinicopathological study of 22 cases. Melanoma Res. 1997;7:63–8.PubMedGoogle Scholar
  48. Unlü RE, Tekin F, Sensöz O, Bauer BS. The role of tissue expansion in the management of large congenital pigmented nevi of the forehead in the pediatric patient. Plast Reconstr Surg. 2002;110(4):1191.PubMedGoogle Scholar

Authors and Affiliations

  1. 1.Clinical Associate of Surgery, Section of Plastic and Reconstructive SurgeryUniversity of Chicago, Pritzker School of MedicineChicagoUSA
  2. 2.Clinical Associate, University of Chicago Hospitals, Director of Plastic and Reconstructive SurgeryIllinois Dermatology InstituteSkokieUSA

Personalised recommendations