Ann Dermatol Vol. 22, No. 4, 2010 DOI: 10.5021/ad.2010.22.4.

460

CASE REPORT

An Unusual Case of Congenital Dermal Melanocytosis

Sanghoon Lee, M.D., Dae-Hyun Kim, M.D., Gunhong Lee, M.D., Kyu-Uang Whang, M.D.,
Jong Suk Lee, M.D., Young-Lip Park, M.D.

Department of Dermatology, College of Medicine, Soonchunhyang University, Seoul, Korea

Dermal melanocytosis is characterized by the presence of CASE REPORT

ectopic melanocytes in the dermis. The most common forms
include the Mongolian spot, blue nevus, nevus of Ota, and A 21-month-old Korean girl presented with an asympto-
nevus of Ito. Some types of dermal melanocytosis do not fit matic pigmented patch on her left face, neck, chest,
into any of these morphologic categories, however. Our case shoulder, and back that had been present since birth. The
demonstrated an extensive amount of uniform deep blue pigmented patch had grown proportionately to the pa-
patches of nevi with unilateral distribution on the left face, tient’s size throughout her life; otherwise, there had been
neck, chest, shoulder, and back. On histopathologic exami- no appreciable change in its color or texture. The patient
nation, a number of elongated melanocytes scattered had no family history of pigment disorders, and her phy-
throughout the dermis were found. We herein report a case sical and mental development was normal. On physical
of congenital unilateral dermal melanocytosis. (Ann Der- examination, the patient had an extensive amount of
matol 22(4) 460∼462, 2010) uniform deep blue patches that covered the left side of her
face, neck, chest, shoulder, and back in a unilateral pa-
-Keyword- ttern, with a few dark blue-brown macules in the lesions.
Congenital unilateral dermal melanocytosis There were also scattered satellite lesions of a similar
appearance on her backbone area (Fig. 1). The epidermal
surface was unaltered, and no underlying induration was
INTRODUCTION clinically apparent. Except for the skin lesions, there were
no remarkable findings on physical examination. There
Dermal melanocytosis is characterized by the presence of was no discoloration or other abnormality on ophthalmo-
ectopic melanocytes in the dermis. The most common logic examination.
forms include the Mongolian spot, blue nevus, nevus of A biopsy specimen from a lesion on the patient’s back
Ota, and nevus of Ito. Some cases of dermal melanocytosis demonstrated a number of elongated melanocytes located
do not fit into any of these morphologic categories, throughout the dermis. Melanocytes were numerous in the
however. Dermal melanocytosis rarely occurs in unusual upper and middle dermis. Immunohistochemical analysis
regions or covers extensive areas of the body1. Atypical showed that melanocytes and melanin were positive for
types of dermal melanocytosis reported in the literature S-100 and Fontana-Masson stains, but cells were negative
include dermal melanocyte hamartoma and congenital for CD68, which can be a marker for melanophages. Me-
segmental dermal melanocytosis2,3. lanocytes were scattered among the collagen bundles, and
We herein report the clinical and histological features of some of these cells were aggregated around blood vessels.
an unusual case of congenital dermal melanocytosis. Their elongated cytoplasms were loaded with fine me-
lanin (Fig. 2). Nevus cell nests were not seen in the epi-
dermis or dermis. Appendages were normal.
Received December 7, 2009, Revised February 12, 2010, Accepted for
publication February 12, 2010
Corresponding author: Young-Lip Park, M.D., Department of Der-
matology, College of Medicine, Soonchunhyang University, 1174
Jung-dong, Bucheon 420-767, Korea. Tel: 82-32-621-5452, Fax: 82-
32-621-5018, E-mail: ylpark@schmc.ac.kr

460 Ann Dermatol

 An Unusual Case of Congenital Dermal Melanocytosis

Fig. 1. An extensive amount of uni-

form deep blue patches were seen to
cover the left unilateral side of the
face, neck, chest, shoulder, and back.

In our case, the skin lesion involved the face and acro-
mioclavicular region, respectively sites for the nevus of
Ota and the nevus of Ito, but our patient’s lesion exten-
sively covered the chin and neck with no involvement of
the eye. In nevi of Ota and Ito, the melanocytes are
mainly situated in the upper dermis, and the basal layer of
the epidermis may be hyperpigmented. However, in our
case, melanocytes were also found in the middle and
lower dermis.
Typical Mongolian spots occur in the sacrococcygeal
region as pale-blue to slate-gray macular hyperpigmentation.
Occasionally, however, they occur outside the lumbosacral
region on places such as the middle or upper part of the
back as aberrant Mongolian spots. Histopathologically,
Fig. 2. Melanocytes were numerous in the upper and middle the melanocytes of Mongolian spots predominantly reside
dermis. Melanocytes were scattered among the collagen bundles, in the lower portions of the dermis. These features are
and some of these cells were aggregated around the blood clinically and histopathologically different from our case.
vessels. Their elongated cytoplasms were loaded with fine melanin.
Melanophages were not seen (H&E, ×40, inset ×400). The blue nevus usually presents as a well-demarcated,
blue-black papule, nodule or plaque that is generally
acquired. Histologic features of the blue nevus include a
DISCUSSION high concentration of dermal melanocytes in the middle
and lower third of the dermis. Melanophages and variable
Except for blue nevi, the various types of dermal mela- degrees of fibrosis are present. These features are different
nocytosis are similar in their histopathologic characteristics; from our case.
they just differ in the concentration and location of the A few cases of unusual congenital dermal melanocytosis
melanocytes. The characteristics they share include elon- have been documented (Table 1). In 1981, Bashiti et al.1
gated dermal melanocytes that are widely scattered be- reported a female infant with generalized blue-gray
tween collagen fibers in the dermis. These melanocytes do discoloration of the skin. In the same year, Burkhart and
not alter the normal topography of the skin3. Gohara2 described an 18-month-old male with diffuse,

Vol. 22, No. 4, 2010 461

S Lee, et al

Table 1. Less common variants of congenital dermal melanocytosis

　 Distribution Morphology Course
Bashiti et al.1 Generalized except for the face, Blue-gray pigmentation Decreased substantially
(1981) palms, soles, genital area, and
nipples were spread
Burkhart and Buttocks bilaterally and right leg Gray-blue patch with several No tendaency to resolution
Gohara2 (1981) in a dermatomal pattern conspicuous macules in the lesion in the first 18 months of life
3
Vélez et al. Right aspect of the trunk in a Mottled gray-blue pigementation Persistent
(1992) segmental distribution with several heterogeneous in color, with
thoracic dermatomes affected numerous maculae of darker hue
Grézard et al.4 In a dermatomal distribution on the Gray-blue pigmentation Slowly spreading
(1999) two sides of the back from the fourth to
the eight dorsal dermatome and extended
laterally on the external part of breasts
Present case Left unilateral aspect of face, neck, chest, Uniform, deep blue patches No tendency to resolution
shoulder, and back in a segmental with a few dark blue- in the first 21 months
pattern with scattered satellite lesions brown macules in the lesion of life
on the back-bone area

bilateral gray-blue pigmentation on his buttocks that ex- cytosis,” to name the lesion on our patient.
tended in a dermatomal pattern down the entire length of We herein report a case of congenital unilateral dermal
his right leg. They used the term “dermal melanocyte ha- melanocytosis.
martoma” to describe this phenomenon. In 1992, Vélez et
al.3 documented the case of a 28-year-old white woman REFERENCES
with extensive, speckled, gray-blue pigmentation in a se-
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462 Ann Dermatol