Clinical Images: Subacute panniculitis-like T cell lymphoma in systemic lupus erythematosus

The patient, a 46-year-old man, presented with acute onset of numerous painful subcutaneous nodules on the arms. He had a 12-year history of systemic lupus erythematosus with complications that included class III and V lupus nephritis, diffuse alveolar hemorrhage, and hemolytic anemia. Prior treatments had included cyclophosphamide and rituximab, and treatments at this time were hydroxychloroquine, mycophenolate mofetil, and belimumab. Examination revealed many tender subcutaneous nodules without an associated rash or overlying skin changes. Biopsy of several nodules revealed small to medium-sized pleomorphic lymphoid cells with hyperchromatic nuclei surrounding individual adipocytes (A). Genetic analysis demonstrated clonal T cell receptor β-chain gene rearrangement (TRB V+J1/2 and TRB D+J1/2). Whole-body positron emission tomography-computed tomography showed small foci of hypermetabolism along the skin surface in the bilateral upper extremities but no pathologic lymphadenopathy (B). He was diagnosed with subacute panniculitis-like T cell lymphoma (SPTCL). He was treated with high-dose oral prednisone, and over several months, his subcutaneous nodules regressed (C); he was tapered off of corticosteroids and has not had a relapse over the past year. SPTCL is a rare cytotoxic primary cutaneous lymphoma and can be challenging to differentiate from lupus erythematosus panniculitis; it has been speculated that the two conditions may exist on a spectrum (1, 2). Indolent cases of SPTCL can be treated with agents such as systemic corticosteroids, methotrexate, retinoids, cyclosporine, and mycophenolate mofetil. For more aggressive disease behaving like gamma–delta T cell lymphoma, chemotherapy is indicated (3). Disclosure Form: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.