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An 86-year-old Mediterranean man presented with a four-year history of asymptomatic lesions on his legs. On examination, he had violaceous infiltrated areas on his feet and below his knees (Figure 1A; Appendix 1, available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.101646/-/DC1). Histology confirmed a diagnosis of Kaposi sarcoma (Appendix 2, available at www.cmaj.ca/lookup/suppl/doi:10.1503/cmaj.101646/-/DC1), and immunohistochemical testing for human herpesvirus 8 was positive (Figure 1B). Further evaluations, including blood cell count and biochemistry, chest radiography and endoscopic studies, were unremarkable. Our patient declined treatment, and, after one year of follow-up, we have not observed any change in the lesions.
Kaposi sarcoma is a multifocal angioproliferative neoplasm that is always linked to human herpesvirus 8.1 Four variants have been described. The “classic” type, seen in our patient, is most commonly found among middle-aged and elderly men of Mediterranean and Jewish lineage, whereas an endemic African type is more common in some south-central African countries. The other variants are related to immunosuppression (reported in 0.4% of patients with transplants) and to HIV infection. The prevalence in HIV infection has dropped dramatically, from 35% of patients with AIDS before 1995 to less than 3% of HIV-positive patients receiving highly active antiretroviral therapy in 2008.2
Kaposi sarcoma presents as small, violaceous papules and macules situated most commonly on the lower limbs and, less frequently, on the arms, trunk, eyelids and genital area. Diabetes mellitus, lymphomas and secondary infections are frequent comorbidities.2 If Kaposi sarcoma is suspected, a careful physical examination and skin biopsy of lesions is indicated, with radiologic and endoscopic studies as needed for staging.
In classic Kaposi sarcoma, treatment must be individualized and includes nonintervention,2 radiotherapy, surgery, topical imiquimod,3 cryotherapy, laser, photodynamic therapy and antiviral drugs or, if extensive skin or mucosal lesions are present, liposomal doxorubicin or other chemotherapeutic agents.4 The classic form of Kaposi sarcoma usually has an indolent course and a good prognosis, with few deaths directly attributable to the condition itself.2
Competing interests: None declared.
This article has been peer reviewed.