Sarcoid Ulcer of the Leg: A Challenging Chronic Wound

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Author(s): 
Pamela Paparone, MSN, APN; Paige Paparone, MSBS; Philip Paparone, DO

Abstract: Sarcoidosis, a systemic inflammatory disease of unknown cause(s), is a diagnosis of exclusion. While skin lesions are common, chronically nonhealing wounds caused by this idiopathic disease are rare and often misdiagnosed. Definitive diagnostic tests do not exist. The finding of noncaseating granulomas on biopsy of wounds is useful. Mistreatment or any delay in proper treatment of ulcerative sarcoidosis can be painful to patients. Corticosteroid therapy generally is effective. The authors present the case of a chronic sarcoid ulcer of the leg that posed a significant diagnostic challenge. The need to include sarcoidosis in the differential diagnosis of nonhealing wounds is emphasized.



Address correspondence to:
Philip Paparone, DO
Stockton Medical Complex
72 West Jimmie Leeds Rd.
Absecon, NJ 08205-9407
Phone: 609-652-2240
E-mail: ppaparone@comcast.net



     Sarcoidosis is a multisystem granulomatous disorder. Its etiology remains unknown, its treatment is not standardized, and its prognosis cannot be easily predicted.1 Its prevalence is estimated at 10 to 20 per 100,000 in the population.2 Although the lung is the most commonly affected organ, the skin often is involved. In fact, the term sarcoidosis is derived from Boeck’s 1899 case report of “multiple benign sarkoid of the skin,” because he thought the extensive lesions resembled sarcoma but were benign.3 The patient’s skin nodules that he examined were characterized by compact, sharply defined foci of epithelioid cells with large pale nuclei in addition to a few giant cells. Skin involvement occurs in 25% to 35% of patients with sarcoidosis, and is often overlooked or misinterpreted given the variability of the lesions.4 These lesions include erythema nodosum, macular or papular rash, nodules, hypo- or hyper-pigmented patches, scar sarcoid, lupus pernio, and plaques.5 Ulceration of sarcoid lesions is rare.5–7 We describe the case of an unusual sarcoid ulcer of the leg that presented as a chronically nonhealing wound, which ultimately was treated successfully with corticosteroid therapy.

Case Report

     In April 2005 an 82-year-old white woman presented with a large wound (7 cm x 9 cm) on the left leg 10 cm above the lateral malleolus, and mild cellulitis of the same leg. The patient had chronic venous insufficiency and both legs were edematous. The wound resembled a large, shallow venous ulcer with a serpiginous border. Although it was closer to the knee than most venous ulcers, it had no distinguishing features. The patient gave a history of suffering from a similar wound several years previously that had healed with compression therapy, but which recently reappeared. Prior to her visit to our clinic, the wound had been treated by another physician who unsuccessfully used antibiotic therapy (ciprofloxacin 500 twice daily; switched to clindamycin 300 mg, 3 times daily) and compression therapy. The wound started to heal, but spontaneously recurred, resulting in the patient presenting to our center.

     Our initial approach was to treat the wound as an infected venous ulcer with mild cellulitis. The patient was given a lower dosage of clindamycin (150 mg, 3 times daily), and she was instructed to take ibuprofen (400 mg, 3–4 times daily) to reduce pain caused by the wound. The wound was dressed with a 3-layer compression bandage (Dyna-Flex®, Johnson & Johnson, Somerville, NJ) applied at light tension to facilitate venous return. Four days later, the wound was found to be unchanged. The patient returned 1 week after that, and when the dressing was removed to inspect the wound, less slough tissue was observed, and buds of red granulation tissue had appeared. At this time, the wound was deemed a venous ulcer and the patient was instructed to wear a Dyna-Flex boot for 1 week.

References: 

1. Judson MA. Sarcoidosis: clinical presentation, diagnosis, and approach to treatment. Am J Med Sci. 2008;335(1):26–33.
2. Rybicki BA, Major M, Popovich J Jr, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. 1997;145(3):234–241.
3. Boeck C. Multiple benign sarkoid of the skin. J Cutan Genito Urin Dis. 1899:17:530–550.
4. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153–2165.
5. Verdegem TD, Sharma OP. Cutaneous ulcers in sarcoidosis. Arch Dermatol. 1987;123(11):1531–1534.
6. Albertini JG, Tyler W, Miller OF 3rd. Ulcerative sarcoidosis. Case report and review of the literature. Arch Dermatol. 1997;133(2):215–219.
7. Marchell RM, Judson MA. Chronic cutaneous lesions of sarcoidosis. Clin Dermatol. 2007;25(3):295–302.
8. Menke NB, Ward KR, Witten TM, Bonchev DG, Diegelmann RF. Impaired wound healing. Clin Dermatol. 2007;25(1):19–25.
9. Nwomeh BC, Yager DR, Cohen IK. Physiology of the chronic wound. Clin Plast Surg. 1998;25(3):341–356.
10. Veien NK, Stahl D, Brodthagen H. Cutaneous sarcoidosis in Caucasians. J Am Acad Dermatol. 1987;16(3 Pt 1):534–540.
11. Schwartz RA, Robertson DB, Tierney LM Jr, McNutt NS. Generalized ulcerative sarcoidosis. Arch Dermatol. 1982;118(11):931–933.
12. Boyd RE, Andrews BS. Sarcoidosis presenting as cutaneous ulceration, subcutaneous nodules and chronic arthritis. J Rheumatol. 1981;8(2):311–316.
13. Herrero JE, Mascaró JM Jr, Llambrich A, Herrero C. Sarcoidosis and pyoderma gangrenosum: an exceptional association. The role of trauma and immunosuppressive agents. J Eur Acad Dermatol Venereol. 2005;19(1):97–99.
14. Paparone PP, Paparone W, Paparone PA. Post-traumatic pyoderma gangrenosum. WOUNDS. 2009:21(4):89–94.
15. Yanardag H, Pamuk ON, Karayel T. Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients. Respir Med. 2003;97(8):978–982.
16. Mañá J, Marcoval J, Graells J, Salazar A, Peyrí J, Pujol R. Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol. 1997;133(7):882–888.
17. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS), and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee. Am J Respir Crit Care Med. 1999;160(2):736–755.
18. Judson MA, Thompson BW, Rabin DL, et al. The diagnostic pathway to sarcoidosis. Chest. 2003;123(2):406–412.
19. Katta R. Cutaneous sarcoidosis: a dermatologic masquerader. Am Fam Physician. 2002;15;65(8):1581–1584.