Acneiform primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma with prominent necrosis

Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma (SMPTCL) is an indolent form of cutaneous lymphoma that usually presents in solitary fashion and is histopathologically characterized by nodular infiltration of small- to medium-sized pleomorphic T-cells. We report the case of a patient who presented with a 5-year history of acneiform lesions on his face. Histopathologic examination of two lesions revealed a nodular infiltrate of small to medium-sized lymphocytes with necrosis in the dermis. The proliferating cells were positive for CD2, CD3 and CD4 and negative for CD8, CD30 and CD56. They were positive for TIA-1 and negative for perforin and granzyme B. The Ki67 proliferation index was approximately 10%. The neoplastic cells expressed programmed death-1 and lacked expression of CXCL-13, bcl-6 and CD10. In situ hybridization for Epstein-Barr virus-encoded RNA yielded a negative result. T-cell receptor gene rearrangement showed identical T-lymphocyte monoclonality in both lesions. In brief, we report a rare case of acneiform SMPTCL with prominent necrosis.