Acral erythema limited to the fingers or the hands has been described in systemic disease. We report the case of a 17-year-old patient who had undergone lung transplantation for cystic fibrosis. Six weeks after transplantation, rapidly growing large-cell immunoblastic non-Hodgkin's lymphoma of the lungs and mediastinum was diagnosed. Sharply demarcated, painless, glovelike erythema was found on both hands. Therapy was reduction of immunosuppression and 12 cycles of extracorporeal photophoresis. After 4 months, lymphoma was in clinical and radiologic remission. Bilateral erythema of the hands also had disappeared. Acral erythema has been reported in association with chemotherapy, collagen vascular disease, infections as with HIV, hepatitis C virus, parvovirus B19, or cytomegalovirus. None of the described associations were detected in this case.