This report describes the clinical, histologic, and immunologic characteristics of 16 diffuse large cell lymphomas of follicular center cell origin with only skin lesions at presentation. These patients presented with nodular and tumorous skin lesions, which in 10 of 16 cases were confined to a circumscribed area on the trunk. Four patients, all elderly women, presented with skin tumors on the lower legs. Histologically, these 16 lymphomas showed nonepidermotropic diffuse dermal infiltrates, mainly consisting of large follicular center cells, with a variable admixture of small cleaved cells, immunoblasts, T-lymphocytes, and macrophages. The relative numbers of large cleaved and large noncleaved cells, respectively, varied considerably in these lymphomas. Immunophenotypically, almost all lymphomas expressed monotypic surface immunoglobulins and HLA-DR antigens, whereas all lymphomas were reactive with B-cell-associated monoclonal antisera B1, Leu-14, and/or To15. Three of four elderly female patients presenting with disease on the lower legs died. Of the 12 other patients, 11 are currently alive and in complete remission, which suggests a favorable prognosis for this type of cutaneous large cell lymphoma.