A Case of Paraneoplastic Acral Vascular Syndrome with Hodgkin Lymphoma that Progressed after Complete Remission and was Improved by Administration of Steroids

Abstract

The patient was a 64-year-old man with a history of diabetes, cerebral infarction, status epilepticus seizure, lower extremity venous thrombosis, and old myocardial infarction. Approximately 3 months before his rst visit, he had continuous high fever and bilateral swollen inguinal lymph nodes,and was diagnosed with Hodgkin lymphoma. Two months after this diagnosis, cyanosis developed on the left 5th digitus pedis, and chemotherapy was started. After two courses, cyanosis of the toe and skin ulcer of the left 5th digitus pedis were conrmed. Angiography suggested no stenosis in the lower extremity artery, and paraneoplastic syndrome was considered based on exclusion diagnosis. As antiplatelet or anticoagulant drugs had been administered, we continued chemotherapy for Hodgkin lymphoma. In addition to the administration of opioid analgesics, spinal cord stimulation therapy was performed to improve pain and microcirculation by inhibiting the efferent sympathetic nerve. However, the effects of this therapy were insufcient. Although complete remission of Hodgkin lymphoma was conrmed on imaging upon completion of the therapy, necrosis of the right 1st and 4th digitus pedis, and left 3rd, 4th, and 5th digitus pedis developed, accompanied by fever. Improvement was conrmed after prednisolone was administered at a dose of 30 mg/day for 19 days. Ischemic symptoms in the distal portion of the extremities associated with malignant tumor are referred to as paraneoplastic acral vascular syndrome, and these symptoms improve when the malignant tumor is treated in approximately 50% of patients. However, aggravation may occur in some patients and careful attention should be paid to this condition. In our case, steroids were effective, suggesting an immunological mechanism. Skin Research, 18 : 231-236, 2019