Primary Cutaneous CD4+ Small to Medium-Size Pleomorphic T-Cell Lymphoma in a 12-Year-Old Girl


  • N. Volks and I. Oschlies contributed equally to the manuscript.

Address correspondence to Regina Fölster-Holst, M.D., Department of Dermatology, Venereology and Allergology, University Hospital Schleswig-Holstein, Campus Kiel, Schittenhelmstraße 7, 24105 Kiel, Germany, or e-mail:


Primary cutaneous CD4+ small to medium-size pleomorphic T-cell lymphoma (PCSM-TCL) is a rare disease that has been added as a provisional entity to the World Health Organization European Organization for Research and Treatment of Cancer (WHO-EORTC) classification of lymphomas with primary cutaneous manifestations. Patients commonly present with a solitary nodule or plaque on the head or upper trunk, but are usually otherwise in good health. The prognosis is favorable, but the optimal treatment has not been defined. Recent publications have described the expression of programmed death-1 in PCSM-TCL and T-cell pseudolymphoma, suggesting a diagnostic value of this marker in the differential diagnosis of PCSM-TCL in contrast to other types of cutaneous T-cell lymphoma. We present the case of a 12-year-old girl with a tumor of the right supraorbital area. She was treated as an outpatient four times with intralesional triamcinolone acetonide at intervals of 3 to 4 weeks. In addition to the case history, this report includes the clinical and histologic findings and a review of the current literature.