A rare case of eccrine spiradenoma—treatment and management
D. R. Sekhar
Received: 6 December 2014 /Accepted: 27 April 2015 /Published online: 17 June 2015
The Author(s) 2015. This article is published with open access at Springerlink.com
Abstract A young male patient presented with multiple swell-
ings on his chest and the nape of his neck. Physical examination
revealed multiple small papulonodular swellings measuring
0.5×0.5 cm to 2×2 cm, that were soft without discharge with
no surrounding skin changes or induration. Skin biopsy samples
were diagnosed as benign adnexal neoplasm consistent with
eccrine spiradenoma, trichoepithelioma, and cylindroma, i.e.,
Brooke–Spiegler syndrome. Having confirmed this to be a case
of eccrine spiradenoma, surgical excision was performed and
the raw area was covered with a split thickness skin graft taken
from the right thigh and sutured over the raw area. The sternal
lesion was circumferentially excised and the wound was primar-
ily closed by Z-plasty. Surgical excision is considered the gold
standard for the treatment of these cases, with low rates of re-
currence. Around 50 such cases have been reported in the liter-
ature to date. Although eccrine spiradenomas are usually soli-
tary and small, the findings in our case underscore the fact that a
variety of presentations are possible. With strict clinical suspi-
cion and histological criteria, the correct diagnosis can be
achieved, especially when combined with pertinent clinical in-
formation and laboratory studies.
Level of Evidence: Level V, therapeutic study.
Keywords Brooke–Spiegler syndrome
Eccrine spiradenoma is an adnexal neoplasm that continues to be
designated as a tumor with eccrine differentiation, however, it is
currently considered an apocrine process. The disease usually
appears as solitary gray, purple, pink, red, or blue nodules on
the upper half of the body. These multiple, potentially painful
spiradenomas were reported for the first time by Kerstein et al .
Eccrine spiradenomas are usually benign and mostly occur
in patients aged 15–35 years . To date, about 15 cases of
linear/zosteriform/nevoid/blaschkoid multiple spiradenomas
have been reported [3, 4]. However, 50 cases of malignant
spiradenoma exist in the literature, and Dabska et al. were
the first to describe malignant spiradenoma in 1972.
While eccrine spiradenomas are indeed rare worldwide, the
malignant variant is rarer still.
Eccrine spiradenomas can be painful, but the rate of malig-
nant transformation is very low. Such transformation to ma-
lignancy has been reported to develop spontaneously with a
metastasis rate of 50 %, which can cause death.
In the reported literature, no sex predilection exists for
spiradenomas or malignant spiradenomas. Although the exact
etiology of these lesions is unknown, spiradenomas appear to
be caused by a defective tumor suppressor gene; in Brooke–
Spiegler syndrome (BSS), there is usually a defect in the
CYLDI gene located on chromosome 16 at position 12.1 .
A 25-year-old patient presented with a history of insidious onset
of multiple swellings over the chest and the nape of his neck
since 5 years of age, which gradually progressing and was asso-
ciated with an itching and burning sensation. There were no
other co-morbid illnesses or significant history.
* Subha Dhua
Department of Plastic and Reconstructive Surgery,
Vydehi Institute of Medical Sciences and Research Centre,
Bangalore, Karnataka, India
Eur J Plast Surg (2016) 39:143–146