DERMATOLOGICA SINICA 32 (2014) 67–68
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Dermatologica Sinica
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CORRESPONDENCE
A large fungating verruciform xanthoma of the scrotum in association
with arteriovenous malformation mimicking giant condyloma
Dear Editor,
A 97-year-old man with underlying hypertension and benign
prostate hyperplasia, had a large tumor on the right side of his
scrotum for decades, during which time the tumor gradually
enlarged. Easy bleeding upon irritation was recently noted, which
prompted him to seek medical help. Physical examination revealed
a 5 cm 3 cm 3.4 cm, pinkish fungating tumor, with a warty
surface and focal hemorrhagic crust. Engorged blood vessels were
noted near the stalk (Figure 1). Sonography showed hypervascularity. Projectile bleeding from the stalk base was noted during tumor
excision.
Histopathological examination revealed a large, exophytic fungating tumor consisting of numerous individual fibroepithelial
polyp-like structures (Figure 2A). The epidermis showed acanthosis, relatively uniform elongation of thin rete ridges, and spongiosis
with infiltration of neutrophils, but without granular layer or hyperkeratosis (Figure 2B). There was no koilocytosis or keratinocytic
atypia. The connective tissue core showed marked edema, many
dilated blood vessels, and a variable amount of inflammatory infiltrate consisting of abundant plasma cells, some small lymphocytes,
and numerous histiocytes with abundant, pale foamy cytoplasm,
which are CD68-positive (Figure 2C). The stalk of the lesion contained many dilated or congested large blood vessels, some of
which had an uneven thickness in their walls (Figure 2D). Based
on these findings, a diagnosis of fungating verruciform xanthoma
(VX) in association with arteriovenous malformation was made.
No recurrence was observed at the 6-month follow-up.
VX is an uncommon type of mucocutaneous lesion, mostly
affecting the oral cavity. Cutaneous involvement is rare, and usually presents as a pink to orange nodule or plaque, with a warty
surface primarily affecting the anogenital area. The rate of scrotal
involvement is higher in Japanese than in Western populations.1
The main histopathological features are epidermal hyperplasia
without keratinocytic atypia or viral inclusion body and
numerous foamy or xanthomatous cells in the accentuated papillary dermis. The exophytic lesion frequently shows hyper- or
parakeratosis and neutrophilic exocytosis. The foamy cells are
positive for CD68 and weakly positive for factor XIIIa, but not
S-100 protein, thus supporting that these foamy cells are histiocytes of macrophage/monocyte or dermal dendrocyte lineage.2
The inflammatory infiltrate also contains plasma cells and lymphocytes. The reticular dermis typically shows vascular ectasia,
but does not have xanthomatous cells, which is different from
lipidemic xanthoma.
The etiology of VX remains elusive. Since it has been reported to
be associated with many inflammatory and neoplastic conditions,
it is considered a reactive phenomenon rather than a neoplastic
process.2 Several possible pathogenic mechanisms are proposed,
including chronic irritation resulting in keratinocyte damage and
xanthomatous cell response, congenital defects, abnormal immune
status, and anatomic factors.2 The present case is very similar to
the case described by Kishimoto et al, in which the authors hypothesized that the formation of VX might be related to the
vascular ectasia deep in the lesion in the reticular dermis.3 Their
case was a 75-year-old Japanese man presenting with a yellowish,
verrucous, pedunculated nodule on the scrotum. The lesion was
accompanied by a pulsating subcutaneous induration covered by
lightly purplish skin. The deep dermis showed severe vascular
ectasia, including capillaries and venous blood vessels and variable
numbers of thin-walled and thick-walled blood vessels in close association with one another, i.e., arteriovenous malformation. They
hypothesized that deep dermal vascular ectasias are initially
induced by an unknown etiology, causing the activation of both
endothelial cells and pericytes in the papillary dermis. Then, these
activated cells release various cytokines and growth factors, which
induce hyperplasia of epidermal keratinocytes and accumulation
of macrophages. Finally, accumulated macrophages scavenge lipids
and are converted to xanthomatous cells. The underlying arteriovenous malformation affects the nutrients and metabolism of overlying blood vessels, and may lead to the deep dermal vascular
ectasia.
Figure 1 A large fungating pedunculated tumor with verrucous surface is present at
the scrotum. An engorged violaceous vessel is connected to the base of the stalk.
1027-8117/$ – see front matter Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.dsi.2013.03.002
68
Correspondence / Dermatologica Sinica 32 (2014) 67–68
Figure 2 (A) A large fungating tumor consisting of numerous fibroepithelial polyp-like structures; (B) the epidermis shows characteristic hyperplasia with neutrophilic infiltration;
(C) there are abundant foamy cells in the papillary dermis bounded by elongated thin rete ridges. (D) arteriovenous malformation present in the stalk of the lesion shows uneven
thickness in the vessel wall. Inset: These foamy cells are CD68-positive, which are histiocytes of macrophage/monocyte or dermal dendrocyte lineage.
Another unusual feature of the present case is that the lesion is
fairly large. There are only a few reported cutaneous lesions with
sizes larger than the present one,4–7 and almost all of the lesions
were plaques instead of polypoid, as in the present case. The large
size of the current lesion may be because it had been allowed to
grow over several decades. A large verrucous tumor over a scrotal
area usually leads to the first impression of giant condyloma,
among other differential diagnoses such as verrucous carcinoma,
squamous cell carcinoma, seborrheic keratosis, giant molluscum
contagiosum, condyloma latum, and xanthoma.8 Therefore, it is
important for clinicians and pathologists to be aware of this disease
and to avoid unnecessary overtreatment.
In summary, we present a rare case of giant VX on the scrotum,
mimicking giant condyloma. This is the second reported case of VX
in association with arteriovenous malformation in the literature.
Kuei-Chung Liu, Chao-Kai Hsu, Sheau-Chiou Chao,
Julia Yu-Yun Lee*
Department of Dermatology, National Cheng Kung University Hospital,
College of Medicine, National Cheng Kung University, Taiwan
* Corresponding author. Department of Dermatology,
National Cheng Kung University Hospital, College of Medicine,
National Cheng Kung University, 138 Sheng-Li Road, Tainan City, Taiwan.
Tel.: þ886 6 235 3535/5419; fax: þ886 6 276 6180.
E-mail address: [email protected] (J.Y.-Y. Lee)
Conflicts of interest: The authors declare that they have no financial or non-financial
conflicts of interest related to the subject matter or materials discussed in this article.
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Received: Nov 16, 2012
Revised: Mar 7, 2013
Accepted: Mar 13, 2013