Congenital Dermal Melanocytosis (Mongolian Spot)

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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.

Mongolian spot is a congenital, developmental condition exclusively involving the skin. ^[1] 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.

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. A recent study on the prevalence of birthmarks and transient skin lesions in 1000 Spanish newborns conducted at a neonatal clinic has revealed a proportion of 0.189 for Mongolian spots, with a confidence interval of 0.164-0.213. ^[2]

More recently, a prospective clinical study of cutaneous findings in newborns in the United States has shown an overall incidence of 14% for dermal melanocytosis over the sacrum and buttocks. When these patients were analyzed according to ethnicity, the incidence was 29% in Asians, followed by others (unspecified ethnicity), Hispanics, African Americans, and whites. ^[3]

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. ^[4]

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). ^[5]

In cross-sectional study on 203 healthy neonates in a Brazilian public hospital, a greater incidence was found in nonwhite, black, and Asian babies. ^[6]

A case-controlled study conducted in Turkey on 50 patients with Down syndrome showed that Mongolian spot was among the most common mucocutaneous disorders; it was seen in 22% of patients and in 6% of healthy controls with a P -value of 0.018. ^[7]

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

No sex predilection is reported for Mongolian spot.

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

Mongolian spots usually fade in the first year of life, but, at times, they may persist indefinitely. However, melanocytes may persist in the dermis when examined histologically. ^[8] Aberrant Mongolian spots located in areas distal from the lumbosacral region may persist, unlike the typically located ones, which have a tendency to resolve. ^[9]

Gupta and Thappa followed 1524 infants with Mongolian spots and found that 42% of these lesions disappeared completely by age 1 year. They demonstrated that large sizes (>10 cm), extrasacral location, dark-colored lesions, and multiple patches are more likely to persist beyond 1 year. The authors also postulated that infants with Mongolian spots persisting beyond age 1 year might be at increased risk for inborn errors of metabolism. ^[10]

Mongolian spot is not associated with mortality or morbidity. However, three cases of congenital aplasia cutis were reported by Japanese authors in concomitance with dermal melanocytosis, but not with the typical distribution of the Mongolian spot, in two males and one female. ^{[11, 12]}

Gupta D, Thappa DM. Mongolian spots: How important are they?. World J Clin Cases. 2013 Nov 16. 1 (8):230-2. [Medline].

Monteagudo B, Labandeira J, León-Muiños E, Carballeira I, Corrales A, Cabanillas M. [Prevalence of birthmarks and transient skin lesions in 1,000 Spanish newborns]. Actas Dermosifiliogr. 2011 May. 102(4):264-9. [Medline].

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 Aug. 161(2):240-5. [Medline].

Cordova A. The Mongolian spot: a study of ethnic differences and a literature review. Clin Pediatr (Phila). 1981 Nov. 20(11):714-9. [Medline].

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. 2010 Jul-Aug. 27(4):397-8. [Medline].

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

Bilgili SG, Akdeniz N, Karadag AS, Akbayram S, Calka O, Ozkol HU. Mucocutaneous disorders in children with down syndrome: case-controlled study. Genet Couns. 2011. 22(4):385-92. [Medline].

Franceschini D, Dinulos JG. Dermal melanocytosis and associated disorders. Curr Opin Pediatr. 2015 Aug. 27 (4):480-5. [Medline].

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. 2010 Jun. 12(3):138-42. [Medline].

Gupta D, Thappa DM. Mongolian spots–a prospective study. Pediatr Dermatol. 2013 Nov-Dec. 30 (6):683-8. [Medline].

Uehara M, Hatano Y, Kato A, Shimizu F, Sato S, Kashima K. Two cases of congenital aplasia cutis with dermal melanocytosis. J Dermatol. 2012 May. 39(5):501-3. [Medline].

Fujita Y, Yokota K, Akiyama M, Machino S, Inokuma D, Arita K. Two cases of atypical membranous aplasia cutis with hair collar sign: one with dermal melanocytosis, and the other with naevus flammeus. Clin Exp Dermatol. 2005 Sep. 30(5):497-9. [Medline].

Leung AK, Kao CP, Leung AA. Persistent Mongolian spots in Chinese adults. Int J Dermatol. 2005 Jan. 44(1):43-5. [Medline].

Leung AK, Kao CP. Extensive mongolian spots with involvement of the scalp. Pediatr Dermatol. 1999 Sep-Oct. 16(5):371-2. [Medline].

Leung AK, Kao CP, Lee TK. Mongolian spots with involvement of the temporal area. Int J Dermatol. 2001 Apr. 40(4):288-9. [Medline].

Afsar FS, Seremet Uysal S. Unusual localization of mongolian spot in a Caucasian infant. Minerva Pediatr. 2015 Nov 4. [Medline].

Ma H, Liao M, Qiu S, Luo R, Lu R, Lu C. The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis. An Bras Dermatol. 2015 Jun. 90 (3 Suppl 1):10-2. [Medline].

Leung AK, Robson WL. Superimposed Mongolian spots. Pediatr Dermatol. 2008 Mar-Apr. 25(2):233-5. [Medline].

Igawa HH, Ohura T, Sugihara T, Ishikawa T, Kumakiri M. Cleft lip mongolian spot: mongolian spot associated with cleft lip. J Am Acad Dermatol. 1994 Apr. 30(4):566-9. [Medline].

Mosher DB, Fitzpatrick TB, Yoshiaki H, et al. Disorders of pigmentation. Fitzpatrick TB, ed. Dermatology in General Medicine. New York, NY: McGraw-Hill; 1993. Vol 1: 903-95.

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

Huang C, Lee P. Phakomatosis pigmentovascularis IIb with renal anomaly. Clin Exp Dermatol. 2000 Jan. 25(1):51-4. [Medline].

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. 1989 Apr. 99(5):561-6. [Medline].

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. 2006 Mar. 20(3):308-10. [Medline].

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

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

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

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

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

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

Ziegler A, Guichet A, Pinson L, Barth M, Levade T, Bonneau D, et al. Extensive Mongolian spots in 4p16.3 deletion (Wolf-Hirschhorn syndrome). Clin Dysmorphol. 2014 Jul. 23 (3):109-10. [Medline].

Köse O, Huseynov S, Demiriz M. Giant Mongolian macules with bilateral ocular involvement: case report and review. Dermatology. 2012. 224(2):126-9. [Medline].

Ma H, Liao M, Qiu S, Luo R, Lu R, Lu C. The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis. An Bras Dermatol. 2015 May-Jun. 90 (3 Suppl 1):10-2. [Medline].

Thomas AC, Zeng Z, Rivière JB, O’Shaughnessy R, Al-Olabi L, St-Onge J, et al. Mosaic Activating Mutations in GNA11 and GNAQ Are Associated with Phakomatosis Pigmentovascularis and Extensive Dermal Melanocytosis. J Invest Dermatol. 2016 Apr. 136 (4):770-8. [Medline].

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

Pessach Y, Goldberg I, Sprecher E, Gat A, Harel A. An unusual presentation of congenital dermal melanocytosis fitting the rare diagnosis of dermal melanocyte hamartoma. Cutis. 2014 Oct. 94 (4):E16-7. [Medline].

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

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

Shirakawa M, Ozawa T, Tateishi C, Fujii N, Sakahara D, Ishii M. Intense pulsed light therapy for aberrant Mongolian spots. Osaka City Med J. 2012 Dec. 58 (2):59-65. [Medline].

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

Disclosure: Nothing to disclose.

Christina M Bergqvist, MD Resident Physician, Department of Dermatology, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, 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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Ponciano D Cruz, Jr, MD Professor and Vice-Chair, Paul R Bergstresser Chair, 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: Received consulting fee from RCTS for independent contractor; Received honoraria from Mary Kay Cosmetics for consulting; Received grant/research funds from Galderma for principal investigator.

Zeina Tannous, MD Associate Professor and Chair, Lebanese American University; Chief of Dermatology, University Medical Center, Rizk Hospital, Lebanon; Visiting Associate Professor in Dermatology, Harvard Medical School; Clinical Associate in Dermatology, Massachusetts General Hospital

Zeina Tannous, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, International Society of Cosmetic and Laser Surgeons, International Academy of Cosmetic Dermatology, Lebanese Dermatological Society, Lebanese Order of Physicians

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.

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

Disclosure: Nothing to disclose.

Dana I Harb, MD Resident Physician, Department of Dermatology, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

Congenital Dermal Melanocytosis (Mongolian Spot)

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