|Year : 2017 | Volume
| Issue : 2 | Page : 131-133
Idiopathic scrotal calcinosis
Gurpreet Kaur1, Deepika Phogat1, Arti Trehan2
1 Department of Pathology, INHS Asvini, Mumbai, Maharashtra, India
2 Army Hospital (R&R), New Delhi, India
|Date of Web Publication||13-Feb-2018|
Dr. Deepika Phogat
Department of Pathology, INHS Asvini, Colaba, Mumbai - 400 005, Maharashtra
Source of Support: None, Conflict of Interest: None
Idiopathic scrotal calcinosis (ISC) is a rare and benign disorder first described by Lewinski in 1883. It is characterized by the presence of multiple yellowish-white calcified asymptomatic nodules gradually increasing in size, appearing in the scrotal skin. It appears mainly in men aged 20–40 years. Histopathology ISC reveals calcium deposits within the dermis that may be associated with giant cell reaction. The calcified intradermal nodules that are seen in this condition occur in the presence of normal calcium and phosphate metabolism. Many studies done on this entity spanning over the past years have failed to demonstrate its origin. Though few studies suggest this entity to be a result of dystrophic calcification in a preexisting cyst, many other studies failed to demonstrate histological evidence to the same. Hence, whether this condition is idiopathic or as a result of dystrophic calcification of preexisting cysts including epidermal, eccrine epithelial cyst, or degenerated dartos muscle remains a controversial issue. Associations with connective tissue diseases such as scleroderma, dermatomyositis, and systemic lupus erythematosus have also been known. Surgical excision is the gold standard treatment for this disease. We report two cases, one in a 59-year-old and other in a 47-year-old male, with no known comorbidities, both presenting with asymptomatic multiple hard scrotal nodules. Both the cases revealed dermal deposits of calcium with normal serum calcium and phosphate levels. There was no evidence of keratinous material, epithelial-lined cystic spaces, or preexisting duct-like structures within or around the foci of calcification, suggesting this entity to be distinct from dystrophic calcification in a preexisting cyst. Surgical excision was done with no evidence of recurrence.
Keywords: Calcinosis, cosmetics, genital diseases, male, scrotum, surgery
|How to cite this article:|
Kaur G, Phogat D, Trehan A. Idiopathic scrotal calcinosis. J Mar Med Soc 2017;19:131-3
| Introduction|| |
Idiopathic scrotal calcinosis (ISC) is an uncommon benign lesion, characterized by calcium deposition in the scrotum with normal calcium levels. Clinical diagnostic confusion may arise from other scrotal lesions such as epidermal inclusion cyst, cutaneous horn, calcified onchocercoma, and other benign tumors such as lipoma, steatocystoma, fibroma, angiokeratoma, and lymphangioma circumscriptum. Histopathological examination (HPE) is confirmatory and should be done in every patient.
| Case Reports|| |
A 59-year-old man presented with a history of multiple nodules over the scrotum for past 2 years. The nodules were painless and were gradually increasing in size and number. There was no preceding history suggestive of sexually transmitted disease, trauma, or any inflammatory disease. He had no history of diabetes or any connective tissue disorder. Physical examination revealed multiple nodules of varying size in the scrotum [Figure 1]a.
|Figure 1: (a and b) Grossly cut surface of the scrotal lesion showing white gritty deposits of calcium in the first case and second case|
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The nodules were nontender and firm to hard in consistency with normal overlying skin. Routine hematological, biochemical (including serum calcium, phosphorus, and parathyroid hormone), and serological investigations were within the normal limits. HIV and Hepatitis B surface antigen (HBsAg) were nonreactive.
The excision of these nodules was performed and the histological examination showed a normal epidermis and intradermal globular bluish nodules of amorphous, homogeneous substance, corresponding to deposits of calcium [Figure 2]a. The nodule was surrounded by fibrocollagenous tissue, without any identifiable epithelial structure/keratin or cyst. Surrounding these in some foci was a foreign body giant cell reaction along with inflammatory reaction consisting of lymphocytes and plasma cells. Despite a careful search, no keratinous material, cystic spaces, and preexisting duct-like structures could be demonstrated within or around the foci of calcification.
|Figure 2: (a) Histopathological examination showing stratified squamous epithelium with underlying amorphous calcific deposits (depicted by red arrows) (b) Calcific deposits surrounded by fibrocollagenous tissue with occasional blood vessels. Note the absence of any epithelial lining/keratin around the calcific deposits|
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A 47-year-old male presented with multiple painless scrotal swellings for 10 years which had gradually progressed in size. Similar to case 1, there was no preceding history suggestive of sexually transmitted disease, trauma, or any inflammatory disease, diabetes, or connective tissue disorder. Local examination showed multiple scrotal nontender nodules which were firm to hard in consistency, the largest measuring 1.5 cm × 1 cm [Figure 1]b. Routine hematological, biochemical, and serological investigations were within the normal limits. HIV and HBsAg were nonreactive.
Excision of scrotal skin with the multiple nodules was done and sent for HPE. The skin surface showed multiple nodules which were firm to hard in consistency. Cut section revealed chalky white deposits. Microscopy of the nodule showed deposition of basophilic calcific material surrounded by collagenous tissue [Figure 2]b. No demonstrable epithelial lining or keratin was found to suggest calcification in a preexisting keratinous cyst as in the first case.
Both the cases were followed up after 1 year of surgery with no evidence of recurrence.
| Discussion|| |
ISC is a rare and benign condition first described by Lewinski in 1883.
It appears mainly in men aged 20–40 years., The youngest and the oldest reported patients were a 9-year-old and an 85-year-old, respectively.
Clinically, ISC consists of hard, yellowish nodules within the dermis of the scrotal skin. Nodules vary in size from 1 mm to several centimeters and number (solitary or multiple). The nodules are usually painless as in the two cases described in this article and patients seek medical advice mainly for cosmetic reasons.
However, few patients may present with pruritus, ulcerations, and discharge of chalky material or superimposed secondary bacterial infection.
A case of ISC presenting with prostatitis-like symptoms (chronic pelvic and perineal pain) has also been reported by Tsai et al.
Due to the location and the usual asymptomatic nature, patients often present late in the disease course. Although Hicheri et al. reported rapidly evolving variant which occurred within 3 months, the disease usually takes an indolent course, developing over several years as seen in case 2.
While the lesions are initially skin colored, they become yellowish as they grow. This process takes such a long period of time that patients present with a history of slow-growing nodules usually for >10 years. The duration of the lesions varies from 3 months to 46 years in the literature.,
Whether ISC is idiopathic or as a result of dystrophic calcification of preexisting cysts including epidermal cyst and eccrine epithelial cyst remains a controversial issue.
Füzesi et al. did not find any cellular structure in the calcified nodules and concluded that ISC is a degenerative phenomenon resulting from alterations in the chemical microenvironment leading to the deposition of calcium and phosphates.
King et al. also with lack of any evidence of epithelial lining or keratin around the calcific deposits on HPE failed to suggest it to be dystrophic calcification in preexisting cysts.
Dini and Colafranceschi  used an immunohistochemistry panel against low-molecular-weight cytokeratin, CAM5.2, cytokeratin (CK) AE1/AE3, and carcinoembryonic antigen; only a slight positivity for CK AE1/AE3 in one calcified nodule was observed within the whole lesion, again going against the hypothesis of its origin from calcification in preexisting cysts.
Wright et al. by performing antikeratin antibodies in multiple sections of 63 lesions from 9 patients could not show any epithelial structure in or around the calcified nodules.
Karaca et al. also reported two cases with no remnants of epithelial cysts.
Song et al. studied 51 scrotal nodules and could identify only three epidermal cysts. The majority of the remaining cysts were labeled as indeterminate cysts because they had no demonstrable cyst lining around the calcific deposits.,
Both case 1 and case 2 also failed to demonstrate any epithelial lining or keratinous material around the calcific deposits on HPE.
Although a few studies like by Dubey et al. suggest the origin of ISC as a result of calcification of preexisting cysts, the entity still remains to be idiopathic according to many other studies and is still regarded as a distinct and rare entity.
Some studies suggest the associations of ISC with connective tissue diseases such as scleroderma, dermatomyositis, and systemic lupus erythematosus, though no such associations were found in case 1 and case 2.
Treatment of ISC is limited to surgical excision of the affected scrotum with reconstruction. The excision must be limited to the scrotal skin as the calcified nodules are localized in the dermis although some studies show high recurrence rates after primary excision.
Cases 1 and 2 were followed up till 1 year after surgery and did not report any recurrence.
| Conclusion|| |
ISC is a rare and benign entity first described by Lewinski in 1883, presenting most commonly as gradually progressive scrotal nodules in men between 20 and 40 years of age. Histopathology demonstrates calcium deposits in the dermis without evidence of any preexisting epithelial/keratinous cyst lining around the calcific deposits, with normal serum calcium and phosphate levels.
Pathogenesis of this entity is controversial and is still considered to be idiopathic. Surgery is the gold standard of treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]