Idiopathic scrotal calcinosis explained

Synonym:	Idiopathic calcified nodules of the scrotum^[1]
Specialty:	dermatology

Idiopathic scrotal calcinosis is a cutaneous condition characterized by calcification of the skin resulting from the deposition of calcium and phosphorus occurring on the scrotum.^[2] However, the levels of calcium and phosphate in the blood are normal.^[3] Idiopathic scrotal calcinosis typically affects young males, with an onset between adolescence and early adulthood. The scrotal calcinosis appears, without any symptoms, as yellowish nodules that range in size from 1 mm to several centimeters.

Without known links to other lesions or systemic pre-conditions, scrotal calcinosis was considered idiopathic. It is not related to calcium phosphate imbalance or renal insufficiency.^[4] By 2010, studies supported that epidermoid cysts are believed to be caused by dystrophic calcification. This process involves subclinical inflammation, rupture, calcification, and cyst wall obliteration.^[5]  

Presentation

• Single or multiple hard, marble-like nodules of varying size affecting scrotal skin.
• Nodules vary in size from a few millimeters to a few centimeters.
• Usually start to appear in childhood or early adult life
• Over time, nodules increase in number and size
• Nodules may break down and discharge chalky material
• Rarely, lesions may be polypoid
• Usually asymptomatic

Etiology

The cause is not well defined.^[6] Originally considered idiopathic condition. Now accepted that majority of cases develop from dystrophic calcification of cyst contents.

Diagnostic

• Clinically Relevant Pathologic Features
• Lesions slowly progress throughout life
  • They slowly increase in number and size
• Nodules are mobile and do not attach to underlying structures

Pathologic Interpretation Pearls

• Globular and granular purple deposits within dermis surrounded by giant cell granulomatous reaction
• Sometimes remnants of cystic lesion can be identified
• Very distinctive appearance with almost no histologic differential diagnosis.

Treatment

Treatment may involve surgery,^[7] which is currently the only recommended intervention. Surgery should include the removal of even small nodules, to prevent the recurrence of the scrotal calcinosis.

Prognosis

• Benign condition
• Slow progression throughout life
• Lesions remain discrete and do not become confluent

Epidemiology

• Incidence: uncommon
• Age: children and young adults

History

Scrotal calcinosis was first described in 1883 by Lewinski.^[8]

See also

• Calcinosis cutis
• Skin lesion
• List of cutaneous conditions

Notes and References

1. Book: Rapini, Ronald P. . Bolognia, Jean L. . Jorizzo, Joseph L. . Dermatology: 2-Volume Set . Mosby . St. Louis . 2007 . 978-1-4160-2999-1 .
2. Book: James, William D. . Berger, Timothy G. . Andrews' Diseases of the Skin: clinical Dermatology . Saunders Elsevier . 2006 . 978-0-7216-2921-6 . etal.
3. Grenader. Tal. Shavit, Linda. Scrotal Calcinosis. New England Journal of Medicine. Aug 18, 2011. 365. 7. 647. 10.1056/NEJMicm1013803. 21848465.
4. Shapiro . L. . Platt . N. . Torres-Rodríguez . V. M. . Idiopathic calcinosis of the scrotum . Archives of Dermatology . 102 . 2 . 199–204 . 0003-987X . 5464321.
5. Dubey . Suparna . Sharma . Rajeev . Maheshwari . Veena . 2010-02-15 . Scrotal calcinosis: idiopathic or dystrophic? . Dermatology Online Journal . 16 . 2 . 5 . 1087-2108 . 20178701.
6. Dubey S, Sharma R, Maheshwari V . Scrotal calcinosis: idiopathic or dystrophic? . Dermatol. Online J. . 16 . 2 . 5 . 2010 . 10.5070/D34QV0S2PG . 20178701 .
7. Karaca M, Taylan G, Akan M, Eker G, Gideroglu K, Gul AE . Idiopathic Scrotal Calcinosis: Surgical Treatment and Histopathologic Evaluation of Etiology . Urology . 76. 6. 1493–1495. April 2010 . 20381842 . 10.1016/j.urology.2010.02.001 .
8. Khallouk A, Yazami OE, Mellas S, Tazi MF, El Fassi J, Farih MH. Idiopathic scrotal calcinosis: a non-elucidated pathogenesis and its surgical treatment.. Reviews in Urology. 2011. 13. 2. 95–7. 21935341. 3176555.