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Background

These are relatively uncommon dark discolorations occurring on the trunk. Many fade with time although same can persist. They are more common in individuals of Asian and African descent.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS  
INCIDENCE/
PREVALENCE
 
The Mongolian spot: a study of ethnic differences and a literature review.

Cordova A.

Clin Pediatr (Phila). 1981 Nov;20(11):714-9. Abstract quote  

Four hundred thirty-seven consecutively born full-term neonates, delivered at Jackson Memorial Hospital, were examined for the presence of mongolian spots (MS). The spots were found in 96% of the Negro children, 46% of the Hispanic children, 9.5% of the Caucasian children, and in both of the Asian children in the series.

The sacro-gluteal region was the most frequent site of pigmentation, with the shoulders next in frequency. Almost all the spots on the extremities were located on the extensor surfaces. The color was most frequently blue-green, but was also commonly greenish-blue, blue-gray, or brown.

In a concurrent review of 124 newborn records, MS was not described by house officers in any, although the probability of its occurrence would have been approximately 90 cases, based on the results of the study. A comprehensive review of the history of the description of MS and theories of its development, as well as a review of the clinical aspects, is presented.
AGE  
SEX  
GEOGRAPHY  
Prevalence of Mongolian spots in Nigerian children in Ile-Ife, Nigeria.

Onayemi O, Adejuyigbe EA, Torimiro SE, Oyelami O, Jegede OA.

Department of Dermatology & Venereology, Faculty of Clinical Sciences, College of Health Sciences, Obafemi Awolowo University, Ile-Ife, Nigeria.
Niger J Med. 2001 Jul-Sep;10(3):121-3. Abstract quote  

Three hundred and sixty nine Nigerian neonates and 484 Nigerian children aged 1 month to 14 years were examined for the presence of Mongolian spots. The spots were observed in 381 children (189 boys and 192 girls) with an overall incidence of 44.7% regardless of sex or age. The colour of the Mongolian spots varied from pale blue to grayish blue.

The most frequently involved sites were the gluteal, sacrococcygeal and lumbar areas respectively. In our study, Mongolian spots were present in 74.8% of neonates and in 13.6% of pre-school children. There was a rapid disappearance in the incidence of Mongolian spots with advancing age. No case of Mongolian spot was recorded in children aged 6 years and above. Primipara whose children have Mongolian spots should be reassured that the spots are harmless and would usually disappear in early childhood.

Physicians should however be alert to marks resulting from child abuse as these could mimic the spots.
EPIDEMIOLOGIC ASSOCIATIONS  

 

DISEASE ASSOCIATIONS CHARACTERIZATION
CLEFT LIP  
Cleft lip mongolian spot: mongolian spot associated with cleft lip.

Igawa HH, Ohura T, Sugihara T, Ishikawa T, Kumakiri M.

Department of Plastic and Reconstructive Surgery, Hokkaido University School of Medicine, Sapporo, Japan.
J Am Acad Dermatol. 1994 Apr;30(4):566-9. Abstract quote  

BACKGROUND: Mongolian spots in the cleft area of cleft lip have been found in some Japanese children.

OBJECTIVE: Our purpose was to study the frequency of cleft lip mongolian spot in children with cleft lip of various severity.

METHODS: Sixty-six babies with unilateral cleft lip were divided into three groups: namely, those with microform cleft lip (10 subjects), incomplete cleft lip (30 subjects), and complete cleft lip (26 subjects). The incidence of cleft lip mongolian spot in the three groups was studied.

RESULTS: Thirty-six babies (55%) had a cleft lip mongolian spot. The mongolian spot was observed in no patients with microform cleft lip, in 18 patients (60%) with incomplete cleft lip, and in 18 patients (69%) with complete cleft lip.

CONCLUSION: Cleft lip mongolian spot appears in high incidence when the cleft goes beyond the vermilion border.
HUNTER'S SYNDROME  
Significance of extensive Mongolian spots in Hunter's syndrome.

Ochiai T, Ito K, Okada T, Chin M, Shichino H, Mugishima H.

Department of Dermatology, Surugadai Nihon University Hospital, 1-8-13 Kanda, Surugadai, Chiyoda-ku, Tokyo 101-8309, Japan.
Br J Dermatol. 2003 Jun;148(6):1173-8. Abstract quote  

BACKGROUND: The importance of early diagnosis in infants with a mild form of Hunter's syndrome should be emphasized. If applied sufficiently early, haematopoietic stem cell transplantation (HSCT) or recombinant enzyme therapy may improve the prognosis. At present, however, diagnosis of the mild form of Hunter's syndrome tends to be delayed, especially in infants with relatively normal intelligence.

OBJECTIVES: To investigate the occurrence of Mongolian spots in infants with Hunter's syndrome, and to clarify the relationship between the Mongolian spots and Hunter's syndrome clinically and histopathologically.

METHODS: Seven Japanese boys with Hunter's syndrome who had received HSCT at ages 4-11 years were observed. The cutaneous manifestations of Mongolian spots before HSCT were evaluated, and compared with those after HSCT. In two patients, the hyperpigmentation from the Mongolian spots was examined by light and electron microscopy.

RESULTS: Pre-HSCT observation revealed that all the patients had an extensive Mongolian spot. These were present at birth and have shown no signs of resolution during the post-HSCT period. Electron microscopic findings showed that pigment-bearing dermal melanocytes contained many free melanosomes in stage IV. These were surrounded by extracellular sheaths and encircled by elastic fibres.

CONCLUSIONS: Our results indicate a strong clinical correlation between the extensive Mongolian spots and Hunter's syndrome. Ultrastructural findings also clearly suggest that the hyperpigmentation is a long-lasting symptom. The recognition of the extensive Mongolian spots is essential as it may lead to early diagnosis in patients with a mild form of Hunter's syndrome.

 

PATHOGENESIS CHARACTERIZATION

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
Extensive aberrant Mongolian spot.

Park KD, Choi GS, Lee KH.

Department of Dermatology, Yonsei University College of Medicine, Seoul, Korea.

J Dermatol. 1995 May;22(5):330-3. Abstract quote  

A 9-month-old male infant had generalized diffuse blue-gray pigmentation over most of his body, sparing the scalp, face, neck, palms, soles, periumbilical area, genital area, and nipples.

Within the lesion, there were several conspicuous macules of considerably darker hue.

Histologic examination revealed numerous dermal melanocytes. By 16 months of age, the child's blue-gray pigmentation had decreased substantially.
ADULT ONSET  
Adult onset Mongolian spot.

Carmichael AJ, Tan CY, Abraham SM.

Skin Hospital, Edgbaston, Birmingham, UK.

Clin Exp Dermatol. 1993 Jan;18(1):72-4. Abstract quote  

We report a case of adult onset, unilateral, progressive, grey-blue pigmentation on the back of an Asian male.

Histology showed the pigmentation to be due to deep dermal melanocytes.

The spectrum of clinical presentations of dermal melanocytosis is reviewed and the unique features of this case outlined.
HEAD AND NECK  
Mongolian spots with involvement of the temporal area.

Leung AK, Kao CP, Lee TK.

Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada.
Int J Dermatol. 2001 Apr;40(4):288-9. Abstract quote  

A Chinese baby girl was born after an uncomplicated pregnancy and a normal spontaneous vaginal delivery to a gravida 2, para 1, 32-year-old mother at 37 weeks' gestation. Neither parent had a history of alcohol or drug ingestion. There was no history of consanguinity. The Apgar scores were 7 at 1 min and 9 at 5 min. Her birth weight was 5 lb 14 oz and her length was 46 cm. At birth, she was noted to have grayish areas on the face and lower back. The infant was seen at 3 months of age because of persistent regurgitations.

On average, she regurgitated 7-8 times a day. Physical examination showed that the infant was not in distress. Her weight was 6 lb 4 oz and length 47 cm. She had a Mongolian spot measuring 0.8 x 1.2 cm in the left temporal area (Fig. 1) and another Mongolian spot measuring 4 cm in diameter in the lumbar area. The Mongolian spots were grayish in color and the pigmentation was uniform in intensity. The rest of the examination was essentially normal. The infant was diagnosed to have Mongolian spots and gastroesophageal reflux. The latter was treated with postural therapy, thickened feedings, and metoclopramide 0.35 mg q.i.d.

The infant was seen again at 4 months of age for a reassessment. There was no noticeable change in shape, size, or color of the Mongolian spots.
Extensive mongolian spots with involvement of the scalp.

Leung AK, Kao CP.

Department of Pediatrics, the University of Calgary, and the Alberta Children's Hospital, Canada.
Pediatr Dermatol. 1999 Sep-Oct;16(5):371-2. Abstract quote  

A Chinese infant boy had extensive Mongolian spots in the occipital, gluteal, sacrococcygeal, and lumbar areas. The occurrence of a Mongolian spot in the scalp area has not been previously reported.

Awareness of such an occurrence is important so that a false accusation of child abuse is not made.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
Dermal dendritic melanocytic proliferations: an update.

Zembowicz A, Mihm MC.

Dermatopathology Unit, Department of Pathology, Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary, Harvard Medical School, Harvard, MA 02114, USA.
Histopathology. 2004 Nov;45(5):433-51. Abstract quote  

Dermal dendritic melanocytic proliferations are a broad group of congenital and acquired melanocytic lesions characterized by the presence of dermal spindled and dendritic cells resembling melanocytes migrating from the neural crest to the epidermis.

Historically, they were subdivided into dermal melanocytoses (naevus of Ota, Ito, Mongolian spot and related conditions), blue naevi and malignant blue naevi. The purpose of this review is to provide an update on recent developments in the field with emphasis on new entities and their differential diagnosis.
BLUE NEVUS  
TATTOO  

 

PROGNOSIS CHARACTERIZATION
Mongolian spots in the newborn: do they mean anything?

Snow TM.

Wake Forest University Baptist Medical Center, Winston-Salem, North Carolina, US
A.
Neonatal Netw. 2005 Jan-Feb;24(1):31-3. Abstract quote  

Mongolian spots are the most frequently seen birthmarks in infants. They have almost universally been regarded as benign cutaneous manifestations that have no clinical significance.

Recently, however, there have been studies suggesting that Mongolian spots manifest more often in children with certain inborn errors of metabolism, including mucopolysaccharidosis and GM1 gangliosidosis.
ADULT  
Persistent Mongolian spots in Chinese adults.

Leung AK, Kao CP, Leung AA.

Department of Pediatrics, University of Calgary, and Alberta Children's Hospital, Calgary, Alberta, Canada.
Int J Dermatol. 2005 Jan;44(1):43-5. Abstract quote  

Four Chinese females aged 21-35 years with Mongolian spots are reported. Two patients had the spots on their arms while the other two had the spots on their shoulders.

The persistence of Mongolian spots in Chinese adults has not been previously reported.

 

TREATMENT CHARACTERIZATION
GENERAL  
   

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated June 15, 2005

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