ARTICLE
Auteur(s) : Dilek
Bayramgurler1, Deniz Filinte2, Rebiay
Kiran1
1Dept of Dermatology, Kocaeli Universitesi
Dermatoloji AD Umuttepe, 41080 Kocaeli, Turkey
2Dept of Pathology, Kocaeli Universitesi Dermatoloji AD
Umuttepe, 41080 Kocaeli, Turkey
accepté le 11 Juin 2008
Angioma serpiginosum (AS) is a rare, acquired, vascular
disorder, asymptomatic, and histopathologically characterized by
clusters of dilated capillaries in the papillary dermis with
multiple, red to violaceous, punctate macules clinically [1, 2] The
lesions evolve in groups forming a serpiginous pattern or small
rings [3] and rarely follow the lines of Blaschko [4-6]. The
lesions are mostly located on the lower extremities [1, 3] but any
region of the body might be involved, except the palms, soles and
mucocutaneous junctions [3]. We report a case of AS following the
lines of Blaschko along the whole left lower extremity, also
involving the left sole.
Case report
A 16-year-old girl referred to our out-patient clinic for the
evaluation of asymptomatic purpuric eruptions involving the left
leg since early childhood. The lesions had slowly progressed over
time without any regression. Her past medical history was not
remarkable and there was no family history of a similar lesion.
Dermatological examination revealed multiple, grouped,
punctuate, red to violaceous non-blanchable macules extending from
the left thigh down the left leg and to the left plantar surface in
a linear distribution (figure 1). Genital and
oral mucous membranes were normal.
Histopathological examination of the punch biopsy specimen taken
from the medial part of the sole showed dilated capillaries in the
upper dermis without any pigment incontinence or inflammatory
findings. The epidermis was hyperkeratotic but it was consistent
with the biopsy site (figure 2).
Immunohistochemical examination of the involved blood vessels
showed the absence of estrogen and progesterone receptors. Routine
laboratory examination was normal. Systemic examinations, including
fundoscopic examination of the eyes, also revealed no
abnormalities. Based on her clinical and histopathological findings
the patient was diagnosed as AS and reassured.
Discussion
The typical clinical features of AS are patches that consist of
multiple, punctate, red to purple-colored macules [2, 3, 5]. The
lesions enlarge peripherally while those at the center fade, giving
rise to a serpiginous pattern [3]. It classically begins in
childhood [1] and stabilizes in adulthood, rarely showing partial
or complete regression [5]. More than 90% of the reported cases are
females under the age 16 [3]. The lesions usually appear
unilaterally on the lower limbs and buttocks [1, 3] although
localized trunk and upper limb involvement [5, 6], extensive
cutaneous involvement [7], asymmetric, segmental involvement [8, 9]
or lesions said to follow Blaschko’s lines [4-6] have also been
reported. However palms, soles and mucocutaneous junction
involvement has never been reported in the literature. The unusual
clinical feature of our case was the distribution of the lesions
that corresponded to Blaschko’s lines, but a more interesting
finding was the involvement of the sole. To the best of our
knowledge, our case is the first AS report with sole involvement.
It is not clear yet whether AS represents a nevoid vascular
malformation or a vascular nevus [7, 8]. AS lesions following the
lines of Blaschko have rarely been reported as noted above.
Moreover, Chen et al. [8] presented a 15-year-old girl with AS
presenting in an asymmetric, systematized, segmental pattern in
their recent report. They reported that these 2 cutaneous patterns
reflect cutaneous mosaicism and suggested that AS might be best
categorized as a vascular nevus. On the other hand, familial cases
show a rather symmetrical, non-segmental involvement [10]. Since
more than 90% of cases are females and the disease progresses
during pregnancy, increased levels of estrogens have been blamed in
the etiology [6, 9]. On the other hand, Erkek et al. [9] revealed
no estrogen or progesterone receptors within the involved blood
vessels in an immunohistochemical examination of their middle aged
woman patient, as well as a normal hormonal profile, suggesting no
role of hormonal stimulus in the pathogenesis of AS. Our
immunhistochemical analysis also revealed the absence of estrogen
and progesterone receptors within the involved vessels. We think
that these observations might be a clue that those hormones do not
play a role in the initiation of this condition. An abnormal
vascular response to cold was another factor which has been
proposed in the pathogenesis of AS [6]. There was no cold exposure
in our patient.
Systemic involvement is rare in AS. Only 3 patients, associated
with retinal and spinal nerve involvement in one [11] and retinal
involvement in the other [6, 12], have been reported in the
literature. Ocular examination of our patient revealed no abnormal
findings.
The disease is usually asymptomatic. Spontaneous resolution with
partial regression may occasionally be seen. If the lesions cause
severe disfigurement, pulsed dye or argon laser treatment can be
used with successful results. We present this case to emphasize
that involvement of the sole may also be seen in AS. As reported
before [9], we also think that hormones do not play a direct role
in the pathogenesis of this condition, on the basis of an absence
of estrogen and progesterone receptors in the lesions. AS lesions
arranged in a segmental pattern reminiscent of the lines of
Blaschko, as in our case, are not rare and this finding might be a
manifestation of cutaneous mosaicism which was first reported by
Chen et al. [8] recently.
Acknowledgements
Financial support: none. Conflict of interest: none.
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