A 70-year-old man was admitted to our hospital for evaluation of a rapidly progressive erythrodermia. He had superficial lymph node swelling and gluteal/inguinal nodosum-like lesions. A skin biopsy of the erythrodermia showed dense mixed infiltrates distributed throughout the whole dermis, predominantly consisting of small lymphocytes and histiocytes with multinucleated giant cells presenting with a granulomatous appearance. The dense infiltrates showed a characteristic angiocentric pattern surrounding the upward vasculature interconnecting the subcutaneous/subpapillary plexus in the dermis. Some infiltrating lymphocytes showed mild atypia with somewhat irregularly shaped nuclei. Their immunologic staining profiles supported the diagnosis of lymphomatoid granulomatosis. Despite the dense angiocentric infiltration in the dermis, typical angiodestructive infiltration with necrotic changes was not seen on pathological examination. In this case, in situ hybridization yielded negative findings for Epstein-Barr virus-encoded RNAs. Three months after the onset of erythrodermia, the patient developed pulmonary lymphomatoid granulomatosis. Corticosteroid pulse therapy was effective for the treatment of severe pulmonary infiltrations and erythrodermia. However, there had been mild recurrence of the condition or hypereosinophilia during the 4 years of follow-up. Low maintenance doses of cyclophosphamide and corticosteroid provided the patient symptomatic relief to date.