A Case of Cutaneous Malignant Lymphoma in a Limb with Elephantiasis

Abstract

We report a 67-year-old female patient who had cutaneous malignant lymphoma in her extremity with elephantiasis. About 30 years previously, she was affected with a gynecologic malignant tumor and treated by radiotherapy. Since then, her left lower limb had been affected with lymphedema. She visited our hospital about 18 years previously because of severe lymphedema with a widespread lichenoidal change and sclerosis in her left lower limb, and was diagnosed as severe lymphedema and elephantiasis. Since the diagnosis, she had been followed at our outpatient clinic. Because the diameter of her affected limb became three times larger than that of her normal limb and developed phlegmone, she was admitted into the hospital. During the treatment of the phlegmone, three erythematous papules appeared on her left thigh. They were not improved by steroid ointment, and a biopsy revealed cutaneous B cell malignant lymphoma. Since no other mass lesions were found by systematic examination, we diagnosed it as primary cutaneous diffuse large B cell lymphoma, leg type. In spite of chemotherapy, the papules rapidly enlarged and spread with tumor formation over her left lower limb. Moreover, her general condition promptly deteriorated, and she died 2 months after the diagnosis. It is known that patients with chronic lymphedema may be affected with malignant tumors, such as lymphangiosarcoma in their prolonged course. We should carefully follow chronic lymphedema, keeping in mind that it may develop not only lymphangiosarcoma, but also cutaneous malignant lymphoma.