eMedicine Specialties > Dermatology > Pediatric Diseases

Congenital Nevi

Author: Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Coauthor(s): Jerry Rothenberg, MD, Clinical Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; President, Director, New Jersey Dermatopathology Laboratory, Inc; Isabelle Thomas, MD, Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange; Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Apr 24, 2009

Introduction

Background

Congenital nevi are present at birth and result from a proliferation of benign melanocytes in the dermis, epidermis, or both. Occasionally, nevi that are not present at birth but are histologically identical to congenital nevi may develop during the first 2 years of life. This is referred to as congenital nevus tardive.1

Congenital nevi are one of several known risk factors for the eventual development of melanoma. Fortunately, melanoma remains an uncommon malignancy in prepubertal children, with an annual incidence of 0.7 cases per million children aged 0-9 years. Patient concerns regarding changing or worrisome-looking nevi are, nonetheless, very common. Moreover, by the time a child reaches adolescence, the incidence of melanoma increases substantially, with a rate of 13.2 cases per million children aged 15-19 years.2

While many sources have noted the so-called melanoma epidemic in adults, only in recent years have data documented an alarming increase in melanoma in adolescents. This increase, combined with the recognition of clearly identifiable melanoma risk factors in childhood, allows physicians of the 21st century to play a crucial role in the identification of children at risk for melanoma and to aid in the prevention of melanoma through education regarding the risks of ultraviolet light exposure.

Pathophysiology

The etiology of congenital melanocytic nevi remains unclear. The melanocytes of the skin originate in the neuroectoderm, although the specific cell type from which they derive remains unknown.3,4,5 One hypothesis is that pluripotential nerve sheath precursor cells migrate from the neural crest to the skin along paraspinal ganglia and peripheral nerve sheaths and differentiate into melanocytes upon reaching the skin.6 One possible explanation for the presence of congenital melanocytic nevi is that an external insult results in a mutation that affects the morphogenesis of the embryonic neuroectoderm and migration of precursor cells to the skin.

Congenital nevi have been stratified into 3 groups according to size. Small nevi are less than 1.5 cm in greatest diameter, medium nevi are 1.5-19.9 cm in greatest diameter, and large or giant nevi are greater than 20 cm in greatest diameter. Giant nevi are often surrounded by several smaller satellite nevi. An alternate definition is that a small congenital nevus is one for which primary closure is possible after excision.

Congenital nevi may also be seen as a component of neurocutaneous melanosis, a rare congenital syndrome characterized by the presence of congenital melanocytic nevi and melanotic neoplasms of the central nervous system. Rokitansky first described neurocutaneous melanosis in 1861.7 The current diagnostic criteria for neurocutaneous melanosis are (1) large (>20 cm) or multiple (>3) congenital nevi in association with meningeal melanosis or melanoma, (2) no evidence of meningeal melanoma except in patients in whom cutaneous lesions are histologically benign, and (3) no evidence of cutaneous melanoma except in patients in whom meningeal lesions are histologically benign.8

Neurocutaneous melanosis may result from an error in the morphogenesis of the neuroectoderm, which gives rise to the melanotic cells of both the skin and meninges. Clinically, patients may present with increased intracranial pressure due to hydrocephalus or a mass lesion. The prognosis of patients with symptomatic neurocutaneous melanosis is very poor, even in the absence of malignancy. In one review of 39 reported cases of symptomatic neurocutaneous melanosis, death occurred in more than half the patients within 3 years of the onset of neurological symptoms, and most deaths were in patients younger than 10 years.8

Frequency

International

Congenital nevi are present in 1-2% of newborn infants.

Mortality/Morbidity

Congenital nevi, depending on size and location, may have a significant impact on cosmesis. Giant congenital nevi place individuals at an increased risk for the development of melanoma at the site of the nevus. For giant congenital melanocytic nevi, the risk of developing melanoma has been reported to be as high as 5-7% by age 60 years.9,10 One study suggests that the risk of melanoma may be greater in those with giant congenital melanocytic nevi with more satellite lesions or a larger diameter.11 Additionally, melanoma developing within giant congenital nevi may develop during childhood and occur deeper in the tissue where it is harder to detect clinically.

While the general consensus regarding smaller nevi is that they pose a greater risk for the development of melanoma than normal skin, this risk has not been quantified. Also suggested is that melanoma developing within smaller congenital nevi usually occurs at puberty or later and develops more superficially in the skin, where it is easier to detect clinically.

Race

No racial predilection is recognized.

Sex

Congenital nevi occur in both sexes, with no known predilection.

Age

To be considered congenital nevi, lesions must be present at birth.

Clinical

History

The presence of congenital nevi may be reported by adults, adolescents, or the parents of infants and children.

Physical

Nevi may be located anywhere on the body. Classification as a congenital nevus depends in large part on an accurate history or photographs or medical reports from birth.

Causes

The etiology of congenital melanocytic nevi has not been elucidated. One possible cause is a mutation due to an external insult.12 An association between infantile hemangiomas and congenital melanocytic nevi has been suggested.13 Future investigation may yield more definitive causative factors.

More on Congenital Nevi

Overview: Congenital Nevi
Differential Diagnoses & Workup: Congenital Nevi
Treatment & Medication: Congenital Nevi
Follow-up: Congenital Nevi
Multimedia: Congenital Nevi
References
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References

  1. Clemmensen OJ, Kroon S. The histology of "congenital features" in early acquired melanocytic nevi. J Am Acad Dermatol. Oct 1988;19(4):742-6. [Medline].

  2. Krengel S, Hauschild A, Schafer T. Melanoma risk in congenital melanocytic naevi: a systematic review. Br J Dermatol. Jul 2006;155(1):1-8. [Medline].

  3. Ansarin H, Soltani-Arabshahi R, Mehregan D, Shayanfar N, Soltanzadeh P. Giant congenital melanocytic nevus with neurofibroma-like changes and spina bifida occulta. Int J Dermatol. Nov 2006;45(11):1347-50. [Medline].

  4. Cruz MA, Cho ES, Schwartz RA, Janniger CK. Congenital neurocutaneous melanosis. Cutis. Oct 1997;60(4):178-81. [Medline].

  5. Silfen R, Skoll PJ, Hudson DA. Congenital giant hairy nevi and neurofibromatosis: the significance of their common origin. Plast Reconstr Surg. Oct 2002;110(5):1364-5. [Medline].

  6. Cramer SF. The melanocytic differentiation pathway in congenital melanocytic nevi: theoretical considerations. Pediatr Pathol. 1988;8(3):253-65. [Medline].

  7. Rokitansky J. Ein ausgezeichneter Fall von Pigment-mal mit ausgebreiteter Pigmentierung der inneren Hirn- und Ruchenmarkshaute. Allg Wien Med Z. 1861;6:113-6.

  8. Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol. May 1991;24(5 Pt 1):747-55. [Medline].

  9. Rhodes AR, Wood WC, Sober AJ, Mihm MC Jr. Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus. Plast Reconstr Surg. Jun 1981;67(6):782-90. [Medline].

  10. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. May 2005;52(5):793-7. [Medline].

  11. Hale EK, Stein J, Ben-Porat L, et al. Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry. Br J Dermatol. Mar 2005;152(3):512-7. [Medline].

  12. Bastian BC, Xiong J, Frieden IJ, et al. Genetic changes in neoplasms arising in congenital melanocytic nevi: differences between nodular proliferations and melanomas. Am J Pathol. Oct 2002;161(4):1163-9. [Medline].

  13. Wu PA, Mancini AJ, Marghoob AA, Frieden IJ. Simultaneous occurrence of infantile hemangioma and congenital melanocytic nevus: Coincidence or real association?. J Am Acad Dermatol. Feb 2008;58(2 Suppl):S16-22. [Medline].

  14. Zaenglein AL, Heintz P, Kamino H, Zisblatt M, Orlow SJ. Congenital Spitz nevus clinically mimicking melanoma. J Am Acad Dermatol. Sep 2002;47(3):441-4. [Medline].

  15. 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. Aug 2007;143(8):1007-14. [Medline].

  16. 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. Sep 1973;4(3):395-418. [Medline].

  17. Rhodes AR, Silverman RA, Harrist TJ, Melski JW. A histologic comparison of congenital and acquired nevomelanocytic nevi. Arch Dermatol. Oct 1985;121(10):1266-73. [Medline].

  18. Everett MA. Histopathology of congenital pigmented nevi. Am J Dermatopathol. Feb 1989;11(1):11-2. [Medline].

  19. Berg P, Lindelof B. Congenital nevocytic nevi: follow-up of a Swedish birth register sample regarding etiologic factors, discomfort, and removal rate. Pediatr Dermatol. Jul-Aug 2002;19(4):293-7. [Medline].

  20. Hoffman D, Ratner D. Diagnosis and management of a changing congenital melanocytic nevus. Skinmed. Sep-Oct 2006;5(5):242-5. [Medline].

  21. Warner PM, Yakuboff KP, Kagan RJ, Boyce S, Warden GD. An 18-year experience in the management of congenital nevomelanocytic nevi. Ann Plast Surg. Mar 2008;60(3):283-7. [Medline].

  22. De Raeve LE, Roseeuw DI. Curettage of giant congenital melanocytic nevi in neonates: a decade later. Arch Dermatol. Jul 2002;138(7):943-7. [Medline].

  23. Michel JL, Caillet-Chomel L. [Treatment of giant congenital nevus with high-energy pulsed CO2 laser]. Arch Pediatr. Nov 2001;8(11):1185-94. [Medline].

  24. Michel JL. Laser therapy of giant congenital melanocytic nevi. Eur J Dermatol. Jan-Feb 2003;13(1):57-64. [Medline].

  25. Reynolds N, Kenealy J, Mercer N. Carbon dioxide laser dermabrasion for giant congenital melanocytic nevi. Plast Reconstr Surg. Jun 2003;111(7):2209-14. [Medline].

  26. Chong SJ, Jeong E, Park HJ, Lee JY, Cho BK. Treatment of congenital nevomelanocytic nevi with the CO2 and Q-switched alexandrite lasers. Dermatol Surg. May 2005;31(5):518-21. [Medline].

  27. Lapiere K, Ostertag J, Van De Kar T, Krekels G. A neonate with a giant congenital naevus: new treatment option with the erbium:YAG laser. Br J Plast Surg. Jul 2002;55(5):440-2. [Medline].

  28. Kono T, Ercocen AR, Kikuchi Y, Isago T, Honda T, Nozaki M. A giant melanocytic nevus treated with combined use of normal mode ruby laser and Q-switched alexandrite laser. J Dermatol. Jul 2003;30(7):538-42. [Medline].

  29. Kono T, Ercocen AR, Nozaki M. Treatment of congenital melanocytic nevi using the combined (normal-mode plus Q-switched) ruby laser in asians: clinical response in relation to histological type. Ann Plast Surg. May 2005;54(5):494-501. [Medline].

  30. Kono T, Ercocen AR, Chan HH, Kikuchi Y, Nozaki M. Effectiveness of the normal-mode ruby laser and the combined (normal-mode plus q-switched) ruby laser in the treatment of congenital melanocytic nevi: a comparative study. Ann Plast Surg. Nov 2002;49(5):476-85. [Medline].

  31. Kim S, Kang WH. Treatment of congenital nevi with the Q-switched Alexandrite laser. Eur J Dermatol. Mar-Apr 2005;15(2):92-6. [Medline].

  32. MacLachlan WWG. Extensive pigmentation of the brain associated with nevi pigmentosi of the skin. J Med Res. 1913;29:433-47.

  33. Marghoob AA. Congenital melanocytic nevi. Evaluation and management. Dermatol Clin. Oct 2002;20(4):607-16, viii. [Medline].

  34. Patterson WM, Lefkowitz A, Schwartz RA, Lambert WC, Rao BK. Melanoma in children. Cutis. May 2000;65(5):269-72, 275. [Medline].

  35. Wiltz H, Kollmer WL, Rauscher GE, Sharma PK, Cohen PJ, Schwartz RA. Excision of the large congenital melanocytic nevus facilitated by the use of the tissue expander. J Surg Oncol. Jun 1988;38(2):104-7. [Medline].

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Further Reading

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Keywords

congenital nevi, congenital melanocytic nevi, congenital nevus, nevocellular nevi, nevocellular nevus, garment nevus, garment nevi, giant hairy nevus, giant hairy nevi, bathing-trunk nevus, bathing trunk nevus, bathing trunk nevi, bathing-trunk nevi, giant pigmented nevus, nevus pigmentosus et pilosus, giant pigmented nevi, neurocutaneous melanosis, melanoma, melanoma risk factors, skin cancer

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Contributor Information and Disclosures

Author

Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Christopher J Steen, MD is a member of the following medical societies: Alpha Omega Alpha and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Jerry Rothenberg, MD, Clinical Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; President, Director, New Jersey Dermatopathology Laboratory, Inc
Disclosure: Nothing to disclose.

Isabelle Thomas, MD, Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange
Isabelle Thomas, MD is a member of the following medical societies: American Academy of Dermatology and Sigma Xi
Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Franklin Flowers, MD, Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, University of Florida College of Medicine
Franklin Flowers, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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