Primary cutaneous Epstein-Barr virus-associated T-cell lymphoproliferative disorder - 2 cases with unusual, prolonged clinical course

Hydroa vacciniforme-like lymphoma and extranodal-type natural killer (NK)/T-cell lymphoma are prototypes of Epstein-Barr virus (EBV)-associated cutaneous T- or NK-cell lymphomas. Hydroa vacciniforme-like lymphoma with systemic spread and extranodal-type NK/T-cell lymphoma are characterized by aggressive clinical course. We describe 2 patients with primary cutaneous EBV-associated T-cell lymphomas who did not satisfy the criteria for well-defined entities and showed unusual, prolonged clinical course. They presented with skin ulcerations and mass lesions confined to the extremities without systemic involvement. Skin biopsies demonstrated dense superficial and deep perivascular and periappendageal lymphoid infiltrates expressing CD3 and CD8, but not CD56. The EBV genomes were found within the tumor cells, and monoclonal T-cell receptor gene rearrangement was present. We suggest that these cases represent a peculiar subtype of EBV+ cutaneous T-cell lymphoma, with a tendency to localize in the skin of the extremities and has an indolent clinical course.