ARTICLE
Auteur(s) :, Tugba
Rezan Ekmekci1,*, Adem Koslu1, Damlanur
Sakiz2
1Department of Dermatology, Sisli Etfal Research and
Training Hospital, Istanbul, TurkeyFax: (+90) 212 234 11 21.
2Department of Pathology, Sisli Etfal Research and
Training Hospital, Istanbul, Turkey
accepté le 29 Novembre 2004
Eccrine spiradenoma (ES) is an uncommon tumour of eccrine sweat
gland(s) [1]. Typically, it presents as a bluish, painful,
slow-growing, solitary nodule, ranging from 0.3 to 5 cm in
diameter, located above the waistline on the anterior surface of
the body [2-4]. Nodules can appear at any age, but the highest
incidence is seen in young adults. The sex incidence is equal.
Spontaneous pain or tenderness on palpation is a frequent and
striking symptom of ES [1]. It presents rarely as multiple tumors
[4]. In this report, a child with multiple ES following Blaschko’s
lines is described.
Case report
An 8-year-old girl presented with asymptomatic papulonodular
lesions on the left half of the face. Lesions were reported to be
present at birth; no family member had similar lesion(s), in
multiple or solitary form. Examination revealed multiple,
pink-purple, grouped, papulonodular lesions ranging from 1 to 5 mm
in size on the left half of the nose and forehead following the
lines of Blaschko. The total size of the involved area was 4 × 5 cm
on the forehead and 4 × 2 cm on the nose ( (figure 1) ). On
palpation, the lesions were firm and nontender. The patient was
otherwise well. Examination of a biopsy specimen taken from a
papule on the forehead revealed a dermal neoplasm characterized by
well-defined nests without connection to the epidermis. Each dermal
nest was surrounded by an ill-defined fibrous pseudocapsule ( (figure 2) ). The
lobules contained two types of epithelial cells arranged in
intertwining cords and small lumina. Cells with small dark nuclei
were present at the periphery of the cords, while cells with large,
pale nuclei were located in the center of the cord and around small
lumina ( (figure 3) ). Based
upon these clinical and histological findings the diagnosis was
established as multiple ES.
Discussion
Multiple ES are uncommon, accounting for less than 2% of all cases
of spiradenoma [5]. They can occur in localized groups, linear or
segmental pattern [4, 6-8].
On the forehead the lines of Blaschko run in a funnel-like
configuration from the scalp to the eyebrows and converge on the
nasal root. From the nasal root they run in 4 main directions. In
the first direction they follow a perpendicular direction to the
tip of the nose and the philtrum [9]. We named this case
“Blaschkoid ES” because the lesions follow exactly this direction.
The first case of multiple ES within the lines of Blaschko was
described by Bourrat et al. [10]. In our opinion, the cases
reported in a zosteriform distribution fit into the Blaschko’s
lines [5, 6, 11].
Linear/segmental ES has been proposed to be an organic hamartoma
because of its association with other appendageal components [5].
We do not think that this case is an organic hamartoma.
The histological feaures of ES are quite characteristic. The
tumor consists of multiple sharply demarcated lobules. Two types of
epithelial cells are present within the lobules and are arranged in
intertwining bands [4]. In the literature, neoplasms that feature
spiradenomas and dermal cylindromas either in the same patient
and/or even within the same tumor mass are described [12].
Most of multiple ES are acquired and sporadic. However, to our
knowledge, two cases of congenital and three cases of familial ES
have been reported [2, 3, 6].
Malignant transformation of ES is rare. Typically, sudden growth
is noted in a tumor that has been present for many years. There is
no age, gender, or site predilection [13]. The incidence of
malignant transformation may increase with the tumor burden of a
pre-existing benign spiradenoma. Likewise, 6% of malignant
spiradenomas develop from multiple benign spiradenomas [14].
The presented case is an extremely unusual form of ES, because
the lesions are multiple, nontender, congenital and follow
Blaschko’s lines.
The most effective treatment of ES is complete surgical
excision; however, this is sometimes not feasible if tumors are
multiple or extensive [5]. Radiotherapy, has been reported to be
effective in two sisters with multiple ES [1]. Carbon dioxide laser
treatment has been proposed in one case of familial ES [2]. The
tissue expansion technique of healthy, neighbouring skin on the
forehead and cheek may be useful for wider or complete excision of
the lesion in this case. It was decided that therapy should be
postponed considering the young age of our patient and likely
advances in medical technology.
References
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