Congenital Dermal Melanocytosis (Mongolian Spot) 

  • Author: Abdul-Ghani Kibbi, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 22, 2011
 

Background

Mongolian spot refers to a macular blue-gray pigmentation usually on the sacral area of healthy infants. Mongolian spot is usually present at birth or appears within the first weeks of life. Mongolian spot typically disappears spontaneously within 4 years but can persist for life.

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Pathophysiology

Mongolian spot is a congenital, developmental condition exclusively involving the skin. Mongolian spot results from entrapment of melanocytes in the dermis during their migration from the neural crest into the epidermis. This migration is regulated by exogenous peptide growth factors that work by the activation of tyrosine kinase receptors. It is postulated that accumulated metabolites such as GM1 and heparan sulfate bind to this tyrosine kinase receptor and lead to severe neurologic manifestations and aberrant neural crest migration.

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Epidemiology

Frequency

United States

More than 90% of Native Americans, 80% of Asians, and 70% of Hispanics have Mongolian spots; less than 10% of whites have Mongolian spots.

International

The prevalence of Mongolian spots varies among different ethnic groups. This condition is most common among Asians. Mongolian spot has also been reported in 80% of East African children, in 46% of Hispanic children, and in 1-9% of white children.[1]

The incidence of Mongolian spot was not significantly associated with sex, gestational age, mother's age group, or delivery type at 2 hospitals in Iran. However, in one of the hospitals in which this observational study was done, mongolian spots were shown to be significantly associated with a high birth weight (≥2500 g).[2]

In recent cross-sectional study on 203 healthy neonates in a Brazilian public hospital, a greater incidence was found in nonwhite, black, and Asian babies.[3]

Mortality/Morbidity

Mongolian spot is not associated with mortality or morbidity.

Race

Mongolian spots are observed in more than 90% of infants of the Mongoloid race (ie, East Asians, Indonesians, Polynesians, Micronesians, Amerindians, Eskimos).

Sex

No sex predilection is reported for Mongolian spot.

Age

Mongolian spot is usually present at birth, but it can also appear within the first weeks of the neonatal period.

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Contributor Information and Disclosures
Author

Abdul-Ghani Kibbi, MD  Professor and Chair, Department of Dermatology, American University of Beirut Medical Center, Lebanon

Disclosure: Nothing to disclose.

Coauthor(s)

Ruba Faik Bahhady, MD  Senior Specialist, Department of Dermatology, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

Zeina Tannous, MD  Consulting Staff, Department of Dermatology, Massachusetts General Hospital, Harvard Medical School

Zeina Tannous, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Mazen Kurban, MD  Staff Physician, Department of Dermatology, American University of Beirut Medical Center

Mazen Kurban, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

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, Northside 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.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  2. Reza AM, Farahnaz GZ, Hamideh S, Alinaghi SA, Saeed Z, Mostafa H. Incidence of Mongolian spots and its common sites at two university hospitals in Tehran, Iran. Pediatr Dermatol. Jul-Aug 2010;27(4):397-8. [Medline].

  3. Zagne V, Fernandes NC. Dermatoses in the first 72 h of life: A clinical and statistical survey. Indian J Dermatol Venereol Leprol. Jul-Aug 2011;77(4):470-6. [Medline].

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  10. Achtelik W, Tronnier M, Wolff HH. [Combined naevus flammeus and naevus fuscocoeruleus: phacomatosis pigmentovascularis type IIa]. Hautarzt. Sep 1997;48(9):653-6. [Medline].

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  12. Kawara S, Takata M, Hirone T, Tomita K, Hamaoka H. [A new variety of neurocutaneous melanosis: benign leptomeningeal melanocytoma associated with extensive Mongolian spot on the back]. Nippon Hifuka Gakkai Zasshi. Apr 1989;99(5):561-6. [Medline].

  13. Torrelo A, Zambrano A, Happle R. Large aberrant Mongolian spots coexisting with cutis marmorata telangiectatica congenita (phacomatosis pigmentovascularis type V or phacomatosis cesiomarmorata). J Eur Acad Dermatol Venereol. Mar 2006;20(3):308-10. [Medline].

  14. Uysal G, Guven A, Ozhan B, Ozturk MH, Mutluay AH, Tulunay O. Phakomatosis pigmentovascularis with Sturge-Weber syndrome: a case report. J Dermatol. Jul 2000;27(7):467-70. [Medline].

  15. Van Gysel D, Oranje AP, Stroink H, Simonsz HJ. Phakomatosis pigmentovascularis. Pediatr Dermatol. Jan-Feb 1996;13(1):33-5. [Medline].

  16. Inamadar AC, Palit A. Persistent, aberrant Mongolian spots in Sjogren-Larsson syndrome. Pediatr Dermatol. Jan-Feb 2007;24(1):98-9. [Medline].

  17. Rybojad M, Moraillon I, Ogier de Baulny H, Prigent F, Morel P. [Extensive Mongolian spot related to Hurler disease]. Ann Dermatol Venereol. Jan 1999;126(1):35-7. [Medline].

  18. Ochiai T, Ito K, Okada T, Chin M, Shichino H, Mugishima H. Significance of extensive Mongolian spots in Hunter's syndrome. Br J Dermatol. Jun 2003;148(6):1173-8. [Medline].

  19. Snow TM. Mongolian spots in the newborn: do they mean anything?. Neonatal Netw. Jan-Feb 2005;24(1):31-3. [Medline].

  20. AlJasser M, Al-Khenaizan S. Cutaneous mimickers of child abuse: a primer for pediatricians. Eur J Pediatr. Nov 2008;167(11):1221-30. [Medline].

  21. Ashrafi MR, Shabanian R, Mohammadi M, Kavusi S. Extensive Mongolian spots: a clinical sign merits special attention. Pediatr Neurol. Feb 2006;34(2):143-5. [Medline].

  22. Kagami S, Asahina A, Watanabe R, et al. Laser treatment of 26 Japanese patients with Mongolian spots. Dermatol Surg. Dec 2008;34(12):1689-94. [Medline].

  23. Shirakawa M, Ozawa T, Ohasi N, Ishii M, Harada T. Comparison of regional efficacy and complications in the treatment of aberrant Mongolian spots with the Q-switched ruby laser. J Cosmet Laser Ther. Jun 2010;12(3):138-42. [Medline].

  24. Ahn JS, Kim SD, Hwang JH, Youn SW, Kim KH, Park KC. Halo-like disappearance of mongolian spot combined with café au lait spot. Pediatr Dermatol. Jan-Feb 1998;15(1):70-1. [Medline].

  25. Levene A. Disseminated dermal melanocytosis terminating in melanoma. A human condition resembling equine melanotic disease. Br J Dermatol. Aug 1979;101(2):197-205. [Medline].

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Multiple Mongolian spots in a child.
 
 
 
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