Clinical dermatology • Concise report doi: 10.1111/j.1365-2230.2007.02434.

Leprosy-associated eccrine syringofibroadenoma of Mascaro

H. L. Tey, W. S. Chong and S. N. Wong
National Skin Center, Singapore

Summary Eccrine syringofibroadenoma of Mascaro is a rare benign tumour. There are five
subtypes, one of which is known to occur in reaction to inflammatory and neoplastic
dermatoses. We describe a patient with previous lepromatous leprosy presenting with
multiple flesh-coloured verrucous plaques over the right foot. Histology was consistent
with eccrine syringofibroadenoma. To our knowledge, this is the first reported case of
multiple eccrine syringofibroadenomata occurring in association with leprosy. The
tumours in this case are most likely reactive in nature, subsequent to multiple
traumatic events with tissue remodelling in an insensate foot affected by leprosy. It is
less probable that the tumours are a result of scarring from recurrent infections. We
also raise the possibility of a neuroeccrine interaction, with sympathetic neuropathy in
leprosy as a contributing factor in the pathogenesis.

Eccrine syringofibroadenoma of Mascaro (ESFA) is a
rare tumour consisting of proliferating ductal structures
resembling the acral portion of the sweat gland. It was
first described by Mascaro in 1963, and usually occurs
in the seventh and eighth decades of life.1 The clinical
presentation is variable and nonspecific, ranging from
solitary lesions to multiple papules and nodules.1,2 The
site of occurrence varies widely, including the face,
trunk and extremities. We present a case of multiple
ESFA occurring in the foot of a patient who had leprosy.
Report
A 78-year-old Chinese woman presented to our centre
with growths on her right foot of about 5 years’
duration. She had been treated for lepromatous leprosy
15 years previously, and about 5 years later, she had
developed an infection on the left leg, which had
occurred after an injury to the insensate leg. The
infection had resulted in an eventual below-knee
Correspondence: Dr Hong Liang Tey, MBBS, MRCP, Grad Dip Geri Medical,
Registrar, National Skin Center, Singapore, 1, Mandalay Road, Singapore
308205.
E-mail: hltey@nsc.gov.sg
Conflict of interest: none declared.
Accepted for publication 10 February 2007
amputation, and since then, she had been a wheelchair
user. Over the past few years, she had also had
recurrent cellulitis of the right lower limb and episodes
of neuropathic ulcers. Other comorbidities were chronic
schizophrenia, epilepsy and hypertension, for which she
was being treated with haloperidol, phenytoin and
amlodipine.
On clinical examination, there were multiple flesh-
coloured, verrucous plaques on the distal side of the
right foot, over the plantar side of the toes, the lateral
border of the foot, and the sole (Fig. 1). There was
resorption of the toes. There was extensive scarring,
with areas of postinflammatory hyperpigmentation and
hypopigmentation seen over the dorsum of the foot
extending up to the knee. The skin over the lower limb
was also dry and eczematous. Neurological examination
revealed loss of pain and temperature sensation over the
right foot up to the knee.
The clinical differential diagnoses of viral warts,
squamous cell carcinoma, tuberculosis verrucosa cutis,
and chromoblastomycosis were considered. Two skin
biopsies performed on two anatomically separated
verrucous plaques revealed similar histological features.
There were thin, anastomosing epithelial cords and
strands of clear cells connected to the undersurface of
the epidermis (Fig. 2). There was a fibrovascular
stroma, and ductal structures were seen within the

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Journal compilation  2007 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 32, 533–535 533
Leprosy-associated eccrine syringofibroadenoma of Mascaro • H. L. Tey et al.
(a)
(b)
Figure 1 (a) Multiple flesh-coloured verrucous plaques on the
right sole and toes. The lesions are separated by normal skin. (b)
Plaques seen on the lateral border of the right foot. Scarring with
post-inflammatory pigmentary changes is also seen on the dorsum
of the foot.
Figure 2 Thin, anastomosing epithelial cords and strands of pale,
weakly stained epithelial cells are connected to the undersurface of
the epidermis (haematoxylin and eosin stain, original magnifica-
tion · 20).
534 Journal compilation  2007 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 32, 533–535
Figure 3 Ductal structures are seen within the tumour, resembling
eccrine ducts, and the tumour is set in a dense fibrovascular
stroma (haematoxylin and eosin stain, original magnification
· 200).
tumour (Fig. 3). There was no evidence of acid-fast
bacilli or fungal elements. The histology was consistent
with eccrine syringofibroadenoma.
French has proposed five subtypes of ESFA,3 modified
from Starink’s classification.4 These are solitary ESFA,
multiple ESFA with ectodermal dysplasia, multiple ESFA
without associated cutaneous findings, nonfamilial
unilateral linear ESFA and reactive ESFA. The reactive
subtype presents as an epithelial change in relation to
other inflammatory and neoplastic dermatoses such as
chronic ulceration of the foot, burn scars, venous stasis,
nail trauma, bullous pemphigoid, erosive palmoplantar
lichen planus, peristomal dermopathy, sebaceous nae-
vus, epithelioid haemangioendothelioma, and squa-
mous cell carcinoma.
ESFA has been described in association with diabetic
polyneuropathy,5,6 and it has been proposed that the
tumours were of the reactive subtype in these cases.
There has been a report of a solitary ESFA that
developed at the site of a chronic foot ulcer in a patient
with lepromatous leprosy.7 Unlike the patient described
in that report, our patient has multiple ESFA, and the
tumours did not develop on sites of chronic ulcerations.
To our knowledge, our patient is only the second
reported case of ESFA occurring in association with
leprosy in the literature, and is the first reported case of
multiple ESFA in a patient with leprosy.
Leprosy typically causes mononeuritis multiplex, and
in lepromatous leprosy, polyneuropathy with ‘glove-
and-stocking’ anaesthesia can occur. Patients fre-
quently sustain multiple traumatic injuries, and this
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 Leprosy-associated eccrine syringofibroadenoma of Mascaro • H. L. Tey et al.
contributes to the resorption of fingers and toes.
Repeated trauma also results in frequent skin repair
and remodelling, which may produce abnormal epithe-
lial changes and eccrine ductal proliferation, resulting
in ESFA. The occurrence of multiple tumours separated
by normal skin in our patient suggests a predisposition
of the skin in this region to the development of these
tumours, and the sites of involvement at the distal and
plantar aspects of the foot are trauma-prone areas.
These findings suggest that the multiple ESFA tumours
in our patient are probably reactive in nature, subse-
quent to multiple traumatic events in the insensate foot.
In patients with diabetes mellitus, the pathogenesis of
ESFA is probably the same as in patients with leprosy,
the common feature being sensory neuropathy in both
diseases.
Our patient had extensive scarring with post inflam-
matory hypo- and hyper-pigmentation over the dorsum
of the foot up to the knee. These were most probably the
result of recurrent infections related to trauma, neuro-
pathic ulcerations and infected eczema. The eczematous
skin changes were secondary to xerosis from autonomic
neuropathy in leprosy. Multiple ESFA were reported to
arise in scarred regions caused by burning in one
patient.8 Scarring after recurrent infections may also
predispose the skin to development of reactive ESFA.
However, the distal aspect of our patient’s foot, where
most of the tumours occurred, demonstrated much less
scarring compared with the proximal aspect. It was thus
less likely that the ESFA in our patient had occurred as a
result of scarring.
We also postulate another mechanism in which
leprosy may be related to the proliferation of eccrine
structures. Sweat glands are normally innervated by
postganglionic sympathetic nerve fibres. It has been
shown that the innervation induces production of
proteins (cholinergic differentiation factors and vaso-
active intestinal peptide) by the sweat glands, and that
there is a reciprocal induction of developmental differ-
entiation associated with decreased catecholamine pro-
duction in the neurones.9 Leprosy and diabetes mellitus
have been known to affect the autonomic nerves. The
alteration of the functions of sympathetic nerves in both
diseases may possibly affect regeneration of traumatized
eccrine tissues and contribute to cellular proliferation.
Further studies into such neuroeccrine interactions are
needed before any conclusion can be made.
Malignant transformation into squamous cell carci-
noma and porocarcinoma has been suspected to occur
in ESFA.10,11 As such, it is prudent to excise the tumour
completely. However, in our patient, the tumour had
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Journal compilation  2007 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 32, 533–535
involved a fairly extensive area and this made complete
excision difficult. Another option would be to debulk the
tumours to decrease the probability of malignant
transformation. Our patient decided to refuse surgery.
We are following her up to check for malignant changes
in the tumours and are considering the use of other
nonsurgical options including radiotherapy, photo-
dynamic therapy, imiquimod, and 5-fluorouracil.
In conclusion, our patient is, to our knowledge, the
first reported case of multiple ESFA occurring in
association with leprosy. Occurrence of ESFA in leprosy
may be due to several mechanisms, of which a reactive
pattern as a consequence of multiple trauma in sensory
neuropathy appears to be the most likely.
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