Primary Cutaneous CD4+ Small-/Medium-Sized Pleomorphic T-Cell Lymphoma: A Cutaneous Nodular Proliferation of Pleomorphic T Lymphocytes of Undetermined Significance? A Study of 136 CasesBeltraminelli, Helmut MD*†; Leinweber, Bernd MD*; Kerl, Helmut MD*; Cerroni, Lorenzo MD*The American Journal of Dermatopathology: June 2009 - Volume 31 - Issue 4 - p 317-322 doi: 10.1097/DAD.0b013e31819f19bb Original Study Abstract Author Information Patients with skin nodules characterized by the infiltrate of pleomorphic small/medium T lymphocytes are currently classified as “primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma” (SMPTCL) or as T-cell pseudolymphoma. The distinction is often arbitrary, and patients with similar clinicopathologic features have been included in both groups. We studied 136 patients (male:female = 1:1; median age: 53 years, age range: 3-90 years) with cutaneous lesions that could be classified as small-/medium-sized pleomorphic T-cell lymphoma according to current diagnostic criteria. All but 3 patients presented with solitary nodules located mostly on the head and neck area (75%). Histopathologic features were characterized by nonepidermotropic, nodular, or diffuse infiltrates of small- to medium-sized pleomorphic T lymphocytes. A monoclonal rearrangement of the T-cell receptor-γ gene was found in 60% of tested cases. Follow-up data available for 45 patients revealed that 41 of them were alive without lymphoma after a median time of 63 months (range: 1-357 months), whereas 4 were alive with cutaneous disease (range: 2-16 months). The incongruity between the indolent clinical course and the worrying histopathologic and molecular features poses difficulties in classifying these cases unambiguously as benign or malignant, and it may be better to refer to them with a descriptive term such as “cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance,” rather than forcing them into one or the other category. On the other hand, irrespective of the name given to these equivocal cutaneous lymphoid proliferations, published data support a nonaggressive therapeutic strategy, particularly for patients presenting with solitary lesions. From the *Research Unit of Dermatopathology, Department of Dermatology, Medical University of Graz, Austria; and †Department of Dermatology, University Hospital of Basel, Basel, Switzerland. The study has been financed by the Swiss National Science Foundation (SNF), the Stiftung Spirig Pharma AG, and the Louis Widmer Fonds. Reprints: Lorenzo Cerroni, MD, Department of Dermatology, Medical University of Graz, Auenbruggerplatz 8, 8036 Graz, Austria (e-mail: firstname.lastname@example.org). © 2009 Lippincott Williams & Wilkins, Inc.