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Generalized Eruptive Syringoma

Article  in  Indian Journal of Dermatology · March 2015

DOI: 10.4103/0019-5154.152586 · Source: PubMed

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Ganesh Avhad Hemangi Jerajani

Grant Medical College MGM Medical College & Hospitall
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Abstract
Eruptive syringoma is a very rare variant of syringoma. It is a benign adnexal tumor of the
intraepidermal portion of eccrine sweat ducts. Here we report a 32-year-old female presented
with classical asymptomatic eruptive syringomas involving her face and extremities.
Key Words: Eruptive variant, syringoma, tadpole appearance
What was known?
Though syringoma is fairly common condition but its eruptive variant is rare, which present around the puberty. It usually occur after inflammatory condition and
present with itchy multiple shiny papules.
Introduction
The word syringoma is derived from Greek language
syrinx meaning tube. Syringomas are benign adnexal
tumors of eccrine origin. It shows differentiation of
intra-epidermal portion of eccrine duct and having four
major variants as proposed by Friedman and Butler:[1]
Localized, generalized including eruptive and multiple,
familial and associated with Down syndrome. The usual
site of predilection is periorbital and neck. While other
sites like axillae, abdomen and extremities are also
involved. Other rare forms like plaque type, linear,
vulvar, penile, chondroid, scalp, acral, solitary over ankle
were also reported in the literature.[2-4]
Case Report
A 32-year-old female presented in outpatient department
with multiple skin to brown colored papules all over the
face and upper extremity since last 1 month [Figure 1a
and b]. The lesion developed spontaneously over
forehead and gradually spread involving remaining face
and the upper extremity. Since that time there was
no change in the lesion. There was no similar history
in the family. No significant medical or surgical history
was present. She denied any use of medication prior to
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DOI: 10.4103/0019-5154.152586
E-IJD CASE REPORT
Generalized Eruptive Syringoma
Ganesh Avhad, Priyanka Ghuge1, Jerajani HR
From the Department of
Dermatology, L.T.M. Medical
College and General Hospital,
Sion, 1Department of Dermatology,
Jaslok Hospital, Mumbai, India
Address for correspondence:
Dr. Ganesh Avhad,
Room No 110, New RMO Hostel,
L.T.M. Medical College and
General Hospital, Sion,
Mumbai - 400 022, India.
E-mail: g_avhad@yahoo.co.in
development of lesion. We kept differential diagnosis
as trichoepitheliomas, hyperplasia of sebaceous glands,
eruptive xanthomas and urticaria pigmentosa. Darrier
sign was negative. General examination was normal
including laboratory and hematological investigations.
On cutaneous examination multiple, symmetrical skin
colored to brownish shiny papules of diameter 2-4 mm
was present all over the face and upper extremity. The
remaining physical examination was normal without
any history of photosensitivity. Mucosal surface was
not involved. Systemic examination was unremarkable.
Skin biopsy of the facial lesion showed a normal
epidermis with benign proliferation of multiple eccrine
ducts lined by two rows of epithelial cells embedded in
fibrotic stroma of the upper dermis and some of which
having tadpole-like appearance and having an amorphous
material in the ductal lumina [Figure 2]. So on the basis
of clinicohistopathological correlation, the diagnosis of
eruptive syringoma was made.
Discussion
Eruptive syringoma is a rare, uncommon clinically distinct
variant of syringoma. It was first described in 1887 by
Jacquet and Darier. It usually presents before or during
puberty affecting about 0.6% of the general population.
It occurs more frequently in females as compared to
males. The lesions are usually bilaterally symmetrical that
occurs in successive crops with flat topped, angulated,
skin to brownish shiny papules having both follicular
and non-follicular involvement. It is usually associated
with pruritus which was absent in our case. It usually
spares palms, soles and mucosal surfaces.[5] Eruptive
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Avhad, et al.: Generalized eruptive syringoma
a b
Figure 1: (a) Multiple, bilaterally symmetrical, flat topped, skin to brown colored
papules all over the face and upper extremity (b) Multiple, flat topped skin to brown
colored papules over the face and upper extremity
Figure 2: Multiple eccrine ducts embedded in fibrotic stroma of dermis with tadpole-
like appearance (H and E, ×40)
syringoma may be associated with Down’s syndrome,
milium, elevation of carcinoembryonic antigen, diabetes,
psychiatric disorders, Ehler Danlos syndrome, prurigo
nodularis, melanocytic nevi and sarcoidosis. It may rarely
simulate urticaria pigmentosa.[6,7]
The pathophysiology for development of eruptive
syringoma is still not fully understood. But the proposed
and accepted theory involves previous cutaneous
inflammatory process leading to hyperplastic reactive
process in eccrine duct due to inflammation because
many cases of eruptive syringomas were reported after
cutaneous inflammatory process like contact dermatitis,
shaving, laser hair removal, alopecia areata, radiation
dermatitis etc., but some authors believes that it is a
neoplastic process which is less acceptable theory for
development of lesion.[8,9]
Treatment
Eruptive syringomas often creates significant cosmetic
concerns for patients and may be unacceptable in the
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society. It may go under spontaneous regression over the
period of time but t he course is largely unpredictable
Therefore treatment of syringoma is mainly cosmetic and
not standardized which includes excision, dermabrasion,
cryo-therapy, electrodesiccation; chemical peels
especially trichloroacetic acid, oral and topical retinoids,
topical atropine, carbon dioxide laser, pulsed dye laser
and anti-glandular eccrine monoclonal antibodies. But
the risk of scarring especially over face remains with
surgical and chemical modalities of treatment. But none
is prevent risk of recurrence with variable result.[10,11]
What is new?
We describe a case of generalised eruptive syringoma having late onset of
presentation and was totally asymptomatic without any prior inflammatory disease.
References
1. Friedman SJ, Butler DF. Syringoma presenting as milia. J Am
Acad Dermatol 1987;16:310-4.
2. Soler-Carrillo J, Estrach T, Mascaró JM. Eruptive syringoma: 27
new cases and review of the literature. J Eur Acad Dermatol
Venereol 2001;15:242-6.
3. Yoshii N, Kanekura T, Churei H, Kanzaki T. Syringoma-
like eccrine sweat duct proliferation induced by radiation.
J Dermatol 2006;33:36-9.
4. Teixeira M, Ferreira M, Machado S, Alves R, Selores M. Eruptive
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syringomas. Indian J Dermatol 2009;54:65-7.
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postpubertal eruptive syringoma triggered with antiepileptic
drugs. J Drugs Dermatol 2010;9:707-10.
8. Guitart J, Rosenbaum MM, Requena L. ‘Eruptive syringoma’:
A misnomer for a reactive eccrine gland ductal proliferation? J
Cutan Pathol 2003;30:202-5.
9. Dyall-Smith DJ, Connors TJ, Scurry J. Generalised eruptive
syringoma – A papular dermatosis. Australas J Dermatol
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10. Patrizi A, Neri I, Marzaduri S, Varotti E, Passarini B.
Syringoma: A review of twenty-nine cases. Acta Derm Venereol
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11. Seirafi HH, Akhyani M, Naraghi ZS, Mansoori P, Dehkordi HS,
Taheri A, et al. Eruptive syringomas. Dermatol Online J
2005;11:13.
How to cite this article: Avhad G, Ghuge P, Jerajani HR. Generalized
eruptive syringoma. Indian J Dermatol 2015;60:214.
Received: February, 2013, Accepted: April, 2013.
Source of support: Nil, Conflict of Interest: Nil.