Oral-cutaneous CD4-positive T-cell lymphoma: a study of two patients

We describe two slowly progressive cases of T-cell lymphoma that involved both acral skin and oral cavity. One patient presented with a tongue nodule, completely responded to chemotherapy and then developed recurrent lymphoma involving tongue and skin a few months later that also responded to therapy. The second patient presented with a skin nodule that spontaneously resolved without therapy, and subsequently recurred in tongue and skin a few years later. In both cases, the neoplasms were composed of atypical lymphoid cells with epidermotropism and were of T-helper cell lineage (CD4+). The initial lesions were also negative for CD30. Identical T-cell receptor gene rearrangements were detected in the initial and recurrent lesions of one case. Although these neoplasms were classified as unspecified peripheral T-cell lymphoma because of the unusual distribution of disease, both cases also had histopathologic features of mycosis fungoides. These cases are strikingly similar, and may represent an unusual clinicopathologic type of T-cell lymphoma that can hone to cutaneous and oral mucosal sites with a slowly progressive natural history.