SCROTAL CALCINOSIS: PRIMARY AND SECONDARY
Eric Eugene Santos, MD and Deba Prasad Sarma, MD
Abstract
Objective: Idiopathic scrotal calcinosis is a rare benign lesion
characterized by the development of multiple subcutaneous calcific
nodules within the scrotal wall. The exact etiology and pathogenesis
of this disorder remains controversial. Originally thought of as an
idiopathic disease with no definitive cause, reported histological
evidence now supports the possibility of the disease originating from
calcific degeneration of epidermal cysts. Based on these findings, an
alternative classification scheme is offered.
Materials and Methods: Case reports and a review of literature.
Results: After an extensive literature search, at least 67 documented
cases of scrotal calcinosis have been reported worldwide since the
first reported case by Lewinski in 1883. Of those 67 cases, 22% (15)
have been attributed to calcific degeneration of epidermal cysts,
including epidermoid, pilar, and indeterminate cysts. Most of the
remaining cases were classified as idiopathic.
Conclusion: The presence of calcified epidermal cysts with
identifiable epithelial linings within a minority of documented cases
challenges the long-held etiologic assumption of the idiopathic nature
of this benign disorder. We propose that scrotal calcinosis be divided
into two general categories: primary (idiopathic) and secondary
scrotal calcinosis. In cases where an identifiable cause (e.g.
calcification of epidermal cysts, calcific degeneration of the dartoic
muscle, dystrophic calcification of subcutaneous soft tissue
secondary to chronic inflammation or infection) can be determined,
the term ‘secondary scrotal calcinosis’ may be used. ‘Primary scrotal
calcinosis’ is reserved for those cases where a definitive cause can
not be found.
Introduction
Scrotal calcinosis is a rare, benign lesion
characterized by the development and presence of
one or more calcified nodules within the scrotal
wall. Usually diagnosed from childhood to early
adulthood, the lesion typically pursues a benign
course. Surgical intervention is primarily reserved
for cosmetic and/or social reasons. Although a
handful of cases have been reported in the literature
dating back to the first reported case by Lewinski in
1883, the exact etiology and pathogenesis remains
largely unknown. Despite multiple attempts by
authors to attribute the development of these lesions
to certain disease processes, most recently to
calcification of pre-existing epidermal cysts, the
medical establishment still regards these particular
scrotal skin lesions as idiopathic in nature.
Creighton University Medical Center, Omaha, Nebraska, USA
Method Discussion
Two recent case reports and a review of literature. Since the early 1980’s, there has been debate over the exact
etiology of calcifications within scrotal skin (Figure 1).
Originally thought of as a truly idiopathic disease entity,
there have been multiple authors who have suggested
otherwise, particularly from cases reported during the mid-
1980’s to 1990’s. One of the most prominent suggested
etiologies of scrotal calcinosis to develop within the mid-
1980’s, is that the disease results from calcification of pre-
existing epidermal cysts within the scrotal skin (Figures 2).
Although some authors have identified intact fragments of
epithelial lining around the areas of calcification(s), not all
cases were able to demonstrate associated keratin
fragments with immunohistochemical staining techniques3.
Reasons for not being able to find an epithelial lining have
been proposed before by Sarma1 and include: 1)
destruction of epithelial cells/lining by inflammation
(secondary to cyst rupture) and 2) inadequate sampling. In
addition, since the condition is benign, attempts at deeper
cuts of the tissue block looking for epithelial lining, are
FIGURE 1 most likely ignored. Perhaps an alternative classification
system is needed that encompasses the various etiologies
that have been reported to date. Secondary scrotal
calcinosis is reserved for those cases that have an actual
cause, such as calcification of epidermal cysts, or
Results secondary to degeneration of muscle (dartoic muscle)
tissue, or secondary to chronic inflammation. Primary
After a review of literature there have been 67 reported cases of scrotal calcinosis, including he scrotal calcinosis is reserved for those cases where an exact
two most recent cases diagnosed at our institution. Of those 67 cases, the etiologies are attributed etiology cannot be obtained (i.e. idiopathic).
as follows:
Etiology # of Cases
Idiopathic 49 References
Calcification of Epidermal cyst(s) 15 1. Sarma, Deba P., and Thomas G. Weilbaecher. "Scrotal Calcinosis:
- Epidermoid (6) Calcification of Epidermal Cysts." Journal of Surgical Oncology 27 (1984): 76-
79.
- Epidermal Inclusion Cyst (6)
- Epithelial Cyst (1) 2. Yahya, Husain, and Abdulmu'min H. Rafindadi. "Idiopathic Scrotal Calcinosis: a
Report of Four Cases and Review of the Literature." International Journal of
- Indeterminate (1) Dermatology 44 (2005): 206-209.
- Multiple cysts (1) 3. Yahya, Husain, and Abdulmu'min H. Rafindadi. "Idiopathic Scrotal
Calcification of dartoic muscle 2 Calcinosis: a Report of Four Cases and Review of the Literature."
International Journal of Dermatology 44 (2005): 206-209.
Other 1
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