Leukocytoclastic Vasculitis Presenting in Association with a Spindle Cell Sarcoma of the Heart. A Paraneoplastic Syndrome?

The rare coexistence of vasculitis and malignancy is intriguing, if not completely understood. Overall, malignancy is found in approximately 5% of patients who present with vasculitis. Leukocytoclastic vasculitis (LCV) is reported as a paraneoplastic event primarily in association with hematologic malignancies. Rarely, LCV has occurred concomitant with solid tumors, including sarcomas. We report a case of a 62‐year‐old African American woman who presented with LCV in association with an occult cardiac sarcoma. Past medical history includes left sided breast cancer treated with chemotherapy and radiation. She presented with worsening of a persistent, non‐pruritic rash involving her lower extremities, diagnosed as LCV on skin biopsy. Within four months she developed congestive heart failure, and a right ventricular mass was identified. Heart biopsies revealed a high grade cardiac sarcoma. Within 2–3 days, she experienced an exacerbation of her vasculitis, accompanied by hemorrhagic bullae. Vasculitis is considered to be a paraneoplastic syndrome if a temporal relationship can be established with a co‐existing malignancy. Here, a clear cut temporal relationship between coexistence, and intervention related exacerbation, is well documented. Temporal exacerbation of the LCV after cardiac biopsies suggests activation of the immune system in response to antigenic determinants within the cardiac tumor.