A 60-year-old man presented with recurrent genital and oral ulcers, necrotic papules on his face and scalp, spiking fever and indurated skin erythema on the trunk. A diagnosis of chronic active Epstein-Barr virus infection and IgG3 subclass deficiency was made, and he was supplemented by intravenous gammaglobulin injection. The spiking fever was resistant to treatment, but the addition of systemic interferon-alpha therapy was partially effective in treating his clinical symptoms, although the patient eventually died from pulmonary effusions and cardiac insufficiency.