MONGOLIAN SPOT (MS) is a hyperpig

PHYSICAL DIAGNOSIS
The
A
Mongolian Spot
Study of Ethnic Differences
and
a
Literature Review
Alberto Cordova, M.D., FAAP
Four hundred thirty-seven consecutively born full-term neonates, delivered
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.
at
MONGOLIAN
SPOT (MS) is a hyperpigmented macula of varying size and form, predominantly blue-green or blue-gray in color.
It is found most frequently in the sacro-gluteal
area of newborns and infants. The macula is
uncommon in the Caucasian race but is very
common in the Mongol and Negro races.
Histologically, characteristic cells are present
in the deeper layers of the corium.
The present report describes its presentation and incidence in a population of diverse
ethnic origins.
MaJteiriah9P and Metthods
Memorial Hospital were the subject of this
study. Each patient was examined for the
presence of MS prior to discharge from the
full-term nursery. The patients were separated into the following ethnic groups: Negro,
Hispanic, Caucasian, and Asian (Oriental
background). The Negro population consisted of those of African origin, many of
whom were of mixed Negro and Caucasian
ancestry. The term Hispanic refers to infants
whose mothers had Spanish surnames and
were Spanish speaking. As such, it represents
. ~ heterogeneous group, consisting of Caucasians and mixed Caucasians and American
Indians and/or Negroes. The Caucasian
group was of European origin, and the Asian
population was of East Asian descent.
Of the total 437 newborns examined, 259
(59.2%) were Negro, 134 (30.6%) were Hispanic, and 42 (9.8%) were Caucasian. There
were only 2 (0.4%) Asian patients.
_
Four hundred thirty-seven consecutively
born, full-term neonates delivered at Jackson
From the Division of Neonatology, Department of
Pediatrics University of Miami School of Medicine,
Miami, Florida.
Correspondence to: Alberto Cordova, M.D., FAAP,
155 Ocean Lane Dr, Apt 6t4, Key Biscayne, FL 33149.
Received for publication June, 1981; accepted
July, 1981.
714
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Results
TABLE 1.
The frequency, extent, and distribution of
the MS in the different ethnic groups are
recorded in Tables 1 and 2. When all ethnic
groups were considered together, MS were
present in 35% of the males and in 38% of
the females. MS were more frequently present
in the upper extremities than in the lower
extremities. In both upper and lower extremities, the extensor aspects were almost
exclusively involved, with the exception of one
infant in which it was present on the flexor
surface of the ankle. Of the 318 (73%) newborns with MS, the right side only was affected
in 4% and the left side in 8%. The aberrant
MS (appearing on the face or head) was found
on the forehead of one Negro and one Hispanic neonate. These two cases represent
0.63% of the total number of neonates with
MS. In the present study MS were not found
on the abdomen, chest, neck, palms of the
soles. J
hands,
The shape of MS
or
commonly either irwith
its borders graduregular indefinite,
with
the
ally blending
surrounding skin. The
color most frequently observed in all ethnic
groups was blue-green. For the Negro population the color was commonly greenishblue. The next most common color in the
total population was blue-gray. Brown coloration in the form of brown specks on a background of blue was present in ten Negro newborns (Fig. 1).
In a concurrent review of 124 newborn
records taken at random, MS was not described by house officers in any of the cases,
although the probability of its occurrence
would have been approximately 90 cases,
based on the present study’s data.
was
or
Frequency and Extent of Mongolian Spots
encountered infrequently in the present
study. To the author’s knowledge, the presence of brown specks in Negro newborns with
MS has not been reported and may be of
were
5
anthropologic significance.
The mongolian spot is a benign pigmented
lesion; its inclusion in the description of
neonates’ skin has been frequently neglected
by house staff and attending physicians, as
noted in this study’s chart review. Although
MS has no apparent pathologic significance,
it may be of medico-legal importance, for it
may be confused with inflicted bruises. Two
reports have been published recently that
underscore the importance of recording in
newborn histories the presence of MS.6.1
Smalek’ reported several infants born with
MS whose sudden death was erroneously
diagnosed as child abuse secondary to trauma
to the lower back area. The MS can be distinguished from a bruise in that the MS does
not change color and may take months or
years to disappear. For this reason, a record
of its presence is important in order to prevent
its confusion with these bruises and the misdiagnosis of battered child syndrome.
TABLE 2.
Body Areas
with
Mongolian Spots
Diseussion
These data on the presence of MS in different ethnic groups are similar to those of a
larger series reported by Jacobs and V47alts~n.~
This study confirms that the left side is more
frequently involved than the right side.2 The
presence of MS on the face and flexor surfaces, which are considered ~xceptional~i4
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FIG. 1. Brown specks over the background of blue MS on a
Negro newborn.
History
The earliest recorded description that
could be found of the mongolian spot was
written by a missionary of the New World,
Father Gumilla.8 In his book, written in 1745,
he described the MS as follows: &dquo;Likewise
the little. Indians are born with a spot on the
posterior part of their waist, dark in color,
with shades between blue and brown, which
gradually fades at the same time that the baby
loses its white color and acquires its own
natural color. This mark or spot is real and is
something that I have seen and examined
repeat~dly.; its size is more or less that of a
’hard peso’ of our mint.&dquo; Perhaps Hippocrates9 was referring to the MS when he wrote
&dquo;If during pregnancy the mother received a
blow in-the abdomen, the infant will be marked
in the part corresponding to the place in
which the blow had been received.&dquo; Certainly
for centuries MS was well known to Japanese
physicians, who gave different interpretations as to their cause and significance.10
This very common trait found in newborns of
the Mongol and Negro races was not recognized by Western physicians and anthropologists until 1885, when it was first described by Baelz&dquo; in an ~.~~’h~’~p~~4~~1~~~~.~-~~~~
under the title &dquo;Somatic Characteristics of the
Japanese.&dquo; He also made the first histologic
study of the &dquo;mongolian cell&dquo; (MC). Baelz considered the macroscopic pigmentation a distinct racial characteristic of the Mongol and
other non-Caucasian races, and called it
mongolian spot (&dquo;Mongolen Flecke&dquo;).
One early and notable dissenter of the exclusive non-Caucasian theory of the MS was
Adachi. Adachi’s1° first communication in
1901, and in his classic paper published two
years later under the title &dquo;Skin Pigment in
the Human and in the Apes,&dquo;12 brought the
subject of MS to its present and generally
accepted anthropologic significance. In this
last paper he reported his microscopic findings in 76 cadavers, &dquo;white&dquo; Europeans, from
embryo to 2.25 years of age. Out of these, he
found the characteristic large pigment cells in
10 children, 2 newborns, and 1 embryo. In
none of those cases could the presence of the
MS be demonstrated macroscopically. Adachi
concluded that the MC is present in different
degrees in all races. He also made minute
topographical and histologic studies of dermal
pigment spots of several species of monkeys,
and surmised that the MS in humans is of the
same type as those spots found in monkeys.
Adachi’s findings were later confirmed by the
work o£EI BahraWy3 in 1922, who emphasized
that the so-called mongolian spot is a normal
finding in Europeans.
In the first 20 years of this century, more
than a hundred papers were published presenting clinical reports, national statistics and
theories on the anthropologic significance of
the MS. Very little new has been added since.
Theories of
Origin
The Mongolian theory explains the presence of the MS in Europeans by the invasion
from Asia of the Huns and Mongols .13 Its
presence in other parts of Asia, Malaysia,
and Africa is explained also by migrations of
the Man~als.~~ An opposite view of the origin
of the mongolian spot was presented by Ashmead.15 According to this author, the MS
originated first among the Negroes and from
them spread to the Mongol and Caucasian
races. The atavistic theory finds support in
works of Adachi&dquo; and Ratsimamanga. 26
According to the latter, the MS would be an
atavistic rudimentary formation of the simian
tail. Larsen and Godfrey17 formulated their
genetic theory according to the rules of
Mendelian inheritance. Their findings showed
considerable agreement between observed
results and the ratio forecast by their theory
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Statistical Reports
Racial Groups
on
Different
fected.3,4,17 MS occasionally are found in the
extremities in those cases with extensive involvement, particularly in the shoulders. 4,17
The presence of MS in the head or neck has
been called aberrant mongolian spot. Wateff,2
in a study of 3,500 infants, reports three
cases involving the head.
-
The first well-authenticated case of MS in
European child of Caucasian descent was
reported in 1903 by Adachi and Fujisawa. 18
Its incidence varies from 5.17% in Italy’9 in a
study of 2,$t~5 children to isolated case reports
from Northern Europe. In the United
States, Brennemann studied 40 Negro children under I year of age. The MS was present
in 90% of the cases. Fischer and Y u20 reported
MS in 98% of newborns in China and 90% in
Japan. Among Jewish children, MS has varied
from 0.4% among the Ashkenazim, 2% in the
Sephardim, and 90% in those of Yemenite
parentage. 21 In South America, Ferreira22
reported an MS incidence of 4% among
Caucasian, 70% among Negroes, and 73%
among those of mixed/race.
a
Clinical
Time of
Size and
The macula has been
size may vary from a dot of a
few milimeters to 6 or more centimeters in
diameter.33
angular.3 The
Persistency into Childhood
Appearance’
at
birth.
MS has been described as mostly blue with
different shades.ll,15,17 Recently, Ayala 23
studied the expression of color in 52 mestizos,
of mixed European and American Indian
ancestry, using photometric reflectance readings. Twenty-five infants had the MS, 18 did
not. Photometric readings were taken at the
spot when present and at the laterodorsal
region as a control for nonpigmented skin in
both groups. The mean skin reflectance value
for infants with and without the spot did not
give a significant difference between the two
groups. This objective study contradicts the
widely held clinical impression that the spots
are more likely to be present and more extensively in those babies of more intense
general pigmentation
Distribution
The
The mark often disappears during the first
second year of life. 17 In the Negro race, it
is hard to determine after 4 years of age.~4
Those marks distant from the sacral region
are said to be more apt to persist than the
typical sacral one.25 However, in a recent study
of 996 Japanese adults, the persistency rate
was 4.1 °~o, and the buttocks was the site of
&Bdquo;
predilection.&dquo;
American Literature
Color
region,
and Adulthood
or
The
present
variously described as
irregularly round, oval, roughly triangular,
heartshaped, resembling a tennis racket, and
Study
patches appear at birth or shortly thereafter, rarely later. 17 From the author’s experience, the MS in term newborns is always
Shape
most common
which
site is the sacro-gluteal
is the only part af-
frequently
The American Medical and Anthropological literature on this subject has been notably
sparse, a fact acknowledged by one of its
early writers.~ The first clinical and pathologic
report was that of Brennemann in 1907.~
Since then it has received little attention. In
1890, five years before Baelz’sl1 publication
first introduced the subject to Western medical literature, the total population of the
United States was 30,710,613. Ten per cent,
or 3,753,073, were Negro; American Indians
comprised about 0.4%, or 122,534. The other
groups included in the demographic study,
the Chinese and the Japanese, were too small
in number to be significant.21 If we then take
the conservative figure of 90% as the percentage in which MS is present in Negro and
Indian babies, that leaves us with approximately I l .~°~~, of the total American population who had this typical pigmentation. This
oversight becomes more evident when we
realize that in the well-known , series of
717
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pediatric text books that published its first
edition in 1919,~~ MS was not mentioned,
and it was not until the fourth edition
published in 194529 that the MS was first
described. In the sixth edition, published in
1954~30 the anthropologic connotation of the
term mongolian for the first time was
recognized as incorrect.
_
Histology
The first histologic description of the
cell (MC) was that of Baelz’s.l1
He made reference to an accumulation of
chromatophoric pigmented cells deep in the
corium from sections of the skin taken from
over the sacrum.
Probably the finest description of the &dquo;mongolian cell&dquo; was that of El Bahrawy3; he refers
to them as &dquo;long spindle shaped irregularly
wavy cells, five to ten microns thick and
thirty to fifty or one hundred microns in
length. The center of the cell is the widest
portion and contains a pale, oval, lightly
stained nucleus which shows up in unstained
sections as a pale spot. The cells may have
long processes and may be bipolar or irregular. Some cells are slender and others
pear-shaped.&dquo; It may be difficult or even
impossible to distinguish between the MC
and other types of dermal melanocytes.
Dorsey, 31 in a discussion about differentiation
between the blue nevus, MS, and the nevus
of Ota, made the following statement: &dquo;The
cells in MS are identical with blue nevus cells
and give a positive dopa reaction. Unlike the
cells of blue nevi, which occur in clumps and
wavy groups, the cells of the MS are widely
spaced in the dermis and do not disturb the
normal architecture of the skin. It would
appear that the blue nevus, the MS, and the
nevus of Ota are closely related and*
possibly represent different stages of the same
mongolian
process.&dquo;
e
D9tJerreIT’à~Bta~
Diagnosis
From the standpoint of clinical differentiation, the four types of dermal melanocytes
are more readily
distinguished from one another. &dquo;Blue nevi are rare in children. They
may appear later in life, and their localization
in more than half of the cases is the dorsa
of hands and feet. Blue nevi in the face are
extremely rare in the Caucasian race, but are
common in the darker races.&dquo;31
According to Cole et al. ,32 &dquo;in the blue nevus
the color is not uniform and the surface of the
skin may be irregular. So-called aberrant MS
probably represents the nevus of Ota or blue
nevus.&dquo; The nevus fuscoceruleus ophthalmomaxillaris, or nevus of Ota, occurs in the
form of macular brownish-blue pigmentation involving the skin area innervated by
the first and second branches of the trigeminal
nerve. In the nevus of Ito, the discoloration
of the skin may involve the shoulders and
neck alone. In all three conditions, the blue
nevus, the nevus of Ota, and the nevus of Ito,
the pigmentation persists throughout life.
These lesions may become malignant. However, malignancy has never been reported in
the I~rISa3~
Finally, in the differential diagnosis one
must also consider tattoo markS4 and bruises.6,1
References
Walton RG: The incidence of birthmarks in the neonate. Pediatrics 58:218, 1976
2. Wateff S: Taches pigmentaires chez les enfants
bulgares. Bull Mem Soc Anthrop De Paris 8:
1.
Jacobs AH,
231, 1907
3. El
4.
5.
Bahrawy AA: Uber de mongolenfleck bei
Europaern. Ein Beitrag zur Pigmentlehre. Arch
Dermatol Syphilis 141:171, 1922
Brennemann J: The sacral or so-called "mongolian"
pigment spots of earliest infancy and childhood,
with special reference to their occurrence in the
American Negro. Am J Phys Anthropol 9:12, 1907
Brues AM: People and Races. New York, Mac-
Millan Publishing, 1977, p 108
6. Smalek JE: Significance of mongolian spots. J
Pediatr 97:504, 1980
7. Bittner S, Newberger EH: Pediatric understanding
of child abuse and neglect. Pediatr Rev 2:
203, 1981
8. Gumilla J: El Orinoco Ilustrado, y defendido,
historia natural, civil y geographica de este gran
rio. Madrid, II impresion, 1745, Vol I, p 82
9. Hippocrates: On intercourse and pregnancy: an
English translation of seman and on the development of the child, Translation of De Genitura
and
Natura Pireri. New York, Schuman Press,
1952, p 39
10. Adachi B: Vorlaufige Mitteilung uber den sog.
Mongolenfleck. J Anthropol Soc Tokyo 16:
De
187, 1901
11. Baelz E: Die korperlichen eigenschafter de Japaner.
Mitt De Deutschen Ges Natur-und Volkerk De
Ostassiens 4:40, 1885
12. Adachi B: Das Hautpigment beim Menschen und
bei del Affen. Z Morphol Anthropol 6 : 1, 1903
718
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13. Comas J: Manual of physical anthropology, Eighth
Edition. Springfield, Illinois, Charles C Thomas.
1960, pp 268-269
14. Gutierrez PD, Hizon J: Mongolian blue spots
among Filipinos. J Phillipine Islands Med Assoc
8:380, 1928
15. Ashmead AS: The mulberry-colored spots on the
skin of the lower spine of Japanese and other
dark races: a sign of Negro descent. J Cutan Dis
23:203, 1905
16. Ratsimamanga AR: Tache pimentaire hereditaire et
origine des Malgaches. Rev Anthropol 5:218, 1941
17. Larsen NP, Godfrey LS: Sacral pigment spots. A
record of seven hundred cases with a genetic
theory to explain its occurrence. Am J Phys
Anthropol 10:253, 1927
18. Adachi B, Fujisawa: Mongolen kinder fleck bei Europaern. Z. Morphol Anthropol 4:132, 1903
19. Fiorentino A: Sulla frequenza de la macchia
mongolica nei bambini de catania. Pediatria 36:
81, 1928
20. Fischer W, Shen Chen Yu: Kurzer Beitraf zu
kenntnis des Mongolenfleckes. Arch Schiff-u
Trope-Hyg 23:447, 1919
21. Kagan H: The blue ("mongolian") spot among
Jewish groups in Palestine. Lancet 2:678, 1932
22. Ferreira C: La tache bleue mongoliquea Sao Paulo
(Brasil). Arch de Med de Inf Paris 27:101, 1924
23.
24.
Ayala ET: The sacral or Mongolian spot: morphology and expression. Hum Biol 43:562, 1971
Mayerhofer E, Lypolt-Krajnovic M: Uber da
gejaifte Vorkommen des sog. Mongolenfleckes
bei den neugeborenen in Zagreb. Wein Klin
Wochenschr 41:775, 1928
E: La tache bleue congenital mongolique. La
Presse Med 25:209, 1910
26. Hidano A: Persistent mongolian spot in the adult.
Arch Dermatol 103:680, 1971
27. United States. Bureau of the Census. Historical
Statistics of the United States, Colonial time to
1970. Bicentennial Edition. Part I. U.S. Department of Commerce, Bureau of the Census, 1975
28. Griffith JPC: The Diseases of Infants and Children.
25.
Apert
Philadelphia, WB Saunders, 1919
29. Nelson, WE: Textbook of Pediatrics, Fourth Edition.
Philadelphia, WB Saunders, 1945
30. Nelson WE: Textbook of Pediatrics, Sixth Edition.
Philadelphia, WB Saunders, 1954
31. Dorsey CS, Montgomery H: Blue nevus and distinction from mongolian spot and the nevus of Ota.
J Invest Dermatol 22:225, 1954
32. Cole HN, Hubler WR, Lund HZ: Persistent
aberrant mongolian spots. Arch Dermatol Syph
61:224, 1950
Rabies-United States, 1980*
There were no human cases of rabies reported in the United States in 1980. There were 6,405 laboratoryconfirmed cases of animals rabies reported in the United States and its territories (Guam, Puerto Rico,
and the Virgin Islands of the United States). This is the largest total since 1954, when 7,282 cases were reported. The 1980 figure represents an increase of approximately 1,250 cases above the 1979 total and
is 83.5% above the average for the preceding 5 years. Forty-eight states and Puerto Rico reported rabid
animals in 1980; only the District of Columbia, Guam, Hawaii, Vermont, and the Virgin Islands of the
United States reported no cases. Seven kinds of animals accounted for 97% of the total reported cases:
skunks, 4,040 (63%); bats, 723 (11.2%); cattle, 398 (6.2%); raccoons, 393 (6.1%); dogs, 247 (4%); cats,
212 (3.3%); and foxes, 207 (3.2%). Wild animals accounted for 85% of the reported cases, and domestic
animals accounted for 15%. Bats continued to be the most widely distributed vector, with confirmed
cases occurring in 46 states; skunks, which were reported from 28 states, were second. Reported rabies
cases in cattle showed the most dramatic increase-up 75% over the 1979 total and up 112% over the average
for the previous 5 years. The increased number of rabies cases in cattle and other domestic animals appears
to be both temporally and geographically related to the increase of rabies in skunks.
Morbidity and Mortality Weekly Report, April 3, 198 1, vol 30, no
Pathogens Br, Viral Diseases Div, Center for Infectious Diseases, CDC.
12.
Reported by
the
Respiratory
and
Special
719
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