Intravascular lymphoma presenting as telangectasias: Response to rituximab and combination chemotherapy

Background: Intravascular lymphoma is a rare and aggressive disease with abnormal lymphocytes confined within blood vessels. Cutaneous findings are common in the presentation of this disease. Objectives: The objectives of this article are (1) to describe a case of intravascular lymphoma treated with chemotherapy and rituximab and (2) to review the literature with respect to skin findings and outcomes of these patients after chemotherapy. Patient: Our patient had an unusual presentation of intravascular lymphoma characterized by tender, slightly indurated plaques composed of dense telangectatic vessels. Results: Our patient responded to combination chemotherapy with the addition of a partial course of rituximab (anti-CD20 monoclonal antibody). Conclusion: Intravascular lymphoma commonly presents with skin findings of tender indurated plaques and nodules. Although a number of clinical variations exist, telangectatic plaques are rarely described. Including our case, 50 patients have been reported after treatment with chemotherapy. Approximately 46% of treated patients exhibit either partial or complete response to chemotherapy. This is the first reported case of intravascular lymphoma treated successfully with the addition of rituximab. Overall, this malignancy remains an unusual and aggressive disease. Recognition of the cutaneous findings and early treatment with chemotherapy may alter the course of this disease. Rituximab may be an important addition to combination chemotherapy in treating intravascular lymphoma.