Kaposi's Sarcoma in Association with Behcet's Disease: Case Report and Literature Review

Objectives: To describe a case of Behcet's uveitis associated with Kaposi's sarcoma occurring simultaneously in a patient and to review the literature on iatrogenic Kaposi's sarcoma. Methods: We describe the case of a 44-year-old Moroccan man, who developed a Kaposi's sarcoma 8 months after immunosuppressive therapy for ocular Behçet's disease. He was treated with corticosteroids and cyclophosphamide (Exdoxan, Baxter) pulse for 6 months followed by oral azathioprine (Imurel, Glaxo Smith Kline). Literature searches were performed on iatrogenic Kaposi's sarcoma and other cases of such association and the potential pathogenic mechanisms involved. Results: Iatrogenic Kaposi's sarcoma is widely reported to develop after renal transplantation during immunosuppressive therapy. Less commonly, Kaposi's sarcoma occurs in patients receiving long-term corticosteroids or immunosuppressive therapy for rheumatic diseases. It is considered to be induced by activation of latent human herpes virus 8. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with ocular Behçet's disease. Interferon-α is of value for patients with both conditions. Conclusion: This case report underscores the relationship between environmental and infectious factors, drug-induced immunosuppression, and the development of Kaposi's sarcoma. © 2007 Elsevier Inc. All rights reserved.