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Syringomas are cutaneous appendages benign
tumors originated from the ducts of the eccrine sweat glands. The clinical
varieties classified by Friedman and Bulter are the generalized form that
includes the multiple and eruptive syringomas and the localized form, in which
the vulvar syringomas are included.
We present 5 cases of vulvar syringomas, which
were submitted to immunohistochemistry to determine estrogen and progesterone
receptors. The immunohistochemical analysis showed positivity in four of the 5
cases for progesterone receptors, and negativity in the 5 cases for the estrogen
receptors.
Keywords: Vulvar syringoma, Immunohistochemistry,
Estrogen and progesterone
INTRODUCTION
Syringomas are cutaneous
appendages benign tumors originated from the ducts of the eccrine sweat glands.
[1,2].
In 1872, Kaposi and
Biesiadeki first described the syringoma and called it Lymphangioma Tuberosum
Multiplex [3]. Later, immunohistochemical and electron microscopy studies
demonstrated its origin in the eccrine sweat glands [4].
The most frequent location is
on the lower eyelids, but they also occur in other areas less frequently such as
the forehead, neck, perilabial area, axillar region or limbs, and vulva, this
last topography being very rare [1,5-10] and first reported by Carreiro in 1972
[11].
The clinical varieties
classified by Friedman and Bulter are the generalized form that includes the
multiple and eruptive syringomas, the familiar form, the associated to Down
syndrome, and the localized form [12,13] in which the vulvar syringomas are
included [12].
Clinically syringomas are
recognized as multiple papular-appearing lesions from yellow-brown to
skin-color, with smooth surface and firm consistency, with a bilateral,
symmetrical linear distribution, 1-3 mm in diameter, and located more
frequently in the labia majora ( Figure
1A,1B) [1,13,14].
The histological features are
proliferation of eccrine ducts in the superficial dermis, covered by a double
layer of cuboidal cells of eosinophilic cytoplasm, as well as microcystic
dilations of eosinophilic PAS-positive content, which simulate a Paisley tie
pattern (Figure 2A,2B) [1,10,12,14].
Some authors suggest that
vulvar syringomas have a hormonal influence, justifying this hypothesis by its
presentation in young women, their presence after puberty, growth during
puberty and pregnancy, as well as exacerbation of pruritus during menstruation
and pregnancy [6,12,15,16].
The presence and absence of
progesterone receptors in vulvar stromal tissue [17], normal eccrine glands and
keratinocytes suggesting a probable correlation in the evolution of syringomas
with prolonged exposure to a hormonal environment with progesterone has been
contradictorily reported [1,6,15,16,18-20]. The biological behavior of vulvar
stromal cells and keratinocytes has not been demonstrated in relation to the
hormonal stimulation of progesterone, although their association seems to be
evident because they are more frequent in women, their evolution indicates that
its appearance is at puberty, it grows in gestational stages and with the use
of contraceptives, and they stay during the premenopausal period [15,16].
The presence of progesterone
receptors in syringomas has been documented by immunohistochemistry in a study
by Wallace and Smoller, who demonstrated positive immunostaining of
progesterone receptor expression in eight of nine cases of extragenital
syringomas; [15] Yorganci et al. also demonstrated positivity for progesterone
receptors in a specific case of vulvar syringoma [16]. There is still
controversy due to other studies with conflicting findings. Trager et al. did
not detect positivity with estrogen or progesterone immunostaining in a case of
syringomas in the neck and vulva in an 8 year-old girl [21]. Huang et al. also
demonstrated negativity of both receptors in 15 cases of vulvar syringomas [6].
Due to this discordant
information, the objective of our work was to identify the presence of estrogen
and progesterone receptors in five cases of syringomas located in the genital
area of women who came to consultation for this condition.
MATERIALS AND METHODS
A complete search was
made in the histopathological results database of the Section of
Dermatopathology of the ‘Dr. Manuel Gea González’ General Hospital with the
words ‘syringoma’ and ‘vulvar syringoma’; we found 5 cases with these diagnoses
in a search of the files reported from January 1995 to September 2017. An
ambispective and cross-sectional study was carried out, the study universe was
every case reported with histopathological diagnosis of vulvar syringomas and
complemented by the immunohistochemical study for hormonal receptors of
estrogen and progesterone.
The paraffin blocks were
obtained, the histological diagnosis of syringomas was confirmed, and the
standard immunohistochemistry technique was performed with an adequate control.
RESULTS
We found 5 cases of
vulvar syringomas that were submitted to immunohistochemistry analysis. The age
of the 5 patients varied from 18 to 55 years-old (mean 36 years-old).
The data can be found in Table
1.
DISCUSSION
From the premise that
the intraepidermal portion of the eccrine ducts marks positivity for
progesterone receptors, the anatomical structure that gives rise to the tumor,
we expect that syringomas will also be positive for progesterone receptors
[15]. The roll of these receptors in the etiology of syringomas is unknown;
despite this we consider that hormonal influence exists since it is well
documented that syringomas are more frequent in women, proliferate at puberty
and grow during pregnancy and the premenstrual period [16].
We found nuclear positivity
for progesterone receptors in 4 out of 5 cases, and negativity for estrogen
receptors in the 5 cases.
The results obtained in our
study agree with that of Wallace and Yorganci [15,16] that supports the
proposal that there is a specific hormonal influence of progesterone for the development
of syringomas of vulvar localization; however, the study of Garau et al.
concluded that there is a variable expression of estrogen and progesterone
receptors in normal vulvar epithelial tissue, which explains the discrepancy of
data in the aforementioned studies [20].
We also detected that there
is uptake of progesterone in other sites of the epithelium, not only in the
eccrine ducts, but also in keratinocytes of the basal layer and in sebaceous
glands, agreeing with the authors who have referred positivity in these
locations [15].
In our sample, the presence
of mild pruritus was reported in two cases, with the other 3 cases being
asymptomatic; however, the most relevant personal concern of the patients was
the aesthetic discomfort, It is important to identify vulvar syringomas to
avoid confusion with their differential diagnoses, particularly with epidermal
cyst, multiple steatocystomas, lichen simplex chronicus, angiokeratomas,
Fox-Fordyce disease, senile angioma, condyloma acuminata and candidiasis [10].
No interventional treatment
was indicated in any of the cases, explanations and measures of genital hygiene
were advised.
CONCLUSION
We conclude and agree
with the previously reported studies of the hormonal influence for the
development of vulvar syringomas.
Since it is a poorly visible
condition and the definitive diagnosis depends directly on the
histopathological study, we consider it to be an underdiagnosed condition.
First-contact physicians, as well as gynecologists and dermatologists, should be aware of these benign tumors and their differential diagnoses for a proper channeling to the next level of care, and to avoid inappropriate diagnoses and unnecessary treatments.
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