We report the case of a 60-year-old man who presented with sudden visual loss, a history of postprandial abdominal pain, malabsorption, and skin lesions typical of systemic Degos' disease. Despite anti-aggregants and prednisone the patient's status did not improve. On the basis of the hypothetical dysimmune origin of this disease, we attempted treatment with intravenous immunoglobulins, without success. We then administered infliximab (Remicade), but 2 months after the third injection the patient developed mesenteric infarction and died. We therefore believe that both intravenous immunoglobulins and antiTNFalpha are ineffective for the treatment of Degos' disease.