Kaposi's sarcoma in rheumatic diseases.

OBJECTIVE

To review the clinical features and outcome of all reported cases of Kaposi's sarcoma in patients with rheumatic diseases.

METHODS

In addition to our patient, we identified cases from a Medline search between the years 1966 and 2002. Cases associated with human immunodeficiency virus infection were excluded.

RESULTS

Including our patient, there were a total of 25 cases reported (11 men and 14 women). Rheumatoid arthritis was present in 8 cases, polymyositis/dermatomyositis in 5, vasculitis syndromes in 5, systemic lupus erythematosus in 3, polymyalgia rheumatica in 2, and 1 each of undifferentiated connective tissue disease and Behcet disease. All but 1 patient had been given systemic corticosteroids for a duration that ranged from 6 weeks to 22 years, and immunosuppressive drugs from 25 days to 3.5 years. The Kaposi's lesions usually involved the skin on the extremities; internal organ involvement occurred in 7 cases. Most lesions responded to a decreasing dosage of corticosteroids and immunosuppressive drugs, or to the administration of radiation or cytotoxic therapy. Six patients died, 4 of which were related to the progression of Kaposi's sarcoma.

CONCLUSION

Kaposi's sarcoma in patients with rheumatologic conditions is rare. The clinical features are similar to those with classical Kaposi's sarcoma. Tumor regression usually occurs with decreasing corticosteroids and/or immunosuppressive drugs, local irradiation, or cytotoxic therapy.