Original ArticlesA Variant of Lymphomatoid Papulosis Simulating Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma. Description of 9 CasesSaggini, Andrea MD* †; Gulia, Andrea MD* ‡; Argenyi, Zsolt MD§; Fink-Puches, Regina*; Lissia, Amelia MD∥; Magaña, Mario MD¶; Requena, Luis MD♯; Simonitsch, Ingrid MD**; Cerroni, Lorenzo MD*Author Information *Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Austria †Department of Dermatology, University of Rome Tor Vergata, Rome ‡Department of Dermatology, University of L'Aquila ∥U.O. Anatomia Patologica, Azienda Ospedaliero-Universitaria di Sassari, Italy §Department of Dermatology and Pathology, University of Washington Medical Center, Seattle ¶Service of Dermatology, Hospital General de Mexico, Universidad Nacional Autónoma de Mexico ♯Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain **Department of Clinical Pathology, Medical University of Vienna, Austria Correspondence: Lorenzo Cerroni, MD, Department of Dermatology, Medical University of Graz, Auenbruggerplatz 8, A-8036 Graz, Austria (e-mail: [email protected]). The American Journal of Surgical Pathology: August 2010 - Volume 34 - Issue 8 - p 1168-1175 doi: 10.1097/PAS.0b013e3181e75356 Buy Metrics Abstract Lymphomatoid papulosis (LyP) is a recurrent, self-healing eruption belonging to the spectrum of cutaneous CD30+lymphoproliferative disorders. Three main histologic subtypes of LyP are recognized: type A (histiocytic), type B (mycosis fungoides—(MF)-like), and type C (anaplastic large cell lymphoma–like). We reviewed 26 biopsies from 9 patients (M:F=6:3, median age: 29; mean age 27,2; age range 10 to 38) who presented with clinical features typical of LyP but with histopathologic aspects that resembled primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma. In all but 1 case atypical lymphoid cells showed expression of CD30, and in 8 of 9 cases a T-cell cytotoxic phenotype could be observed (βF1+, CD3+, CD4−, CD8+). Expression of at least 1 cytotoxic marker (TIA-1, granzyme B) was observed in all cases. Polymerase chain reaction analysis of the T-cell receptor genes revealed a monoclonal rearrangement in 2 of 5 cases tested. Follow-up data available for 8 patients (mean follow-up time: 84 mo, median: 32.5 mo; range: 1 to 303 mo) revealed that none of them developed systemic involvement or signs of other cutaneous lymphomas. This cytotoxic variant of LyP may be histopathologically indistinguishable from primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, and may be the source of pitfalls in the diagnosis and classification. We propose the term LyP type D for this unusual variant of the disease. Accurate clinicopathologic correlation is required in this setting, with crucial implications regarding prognosis and management of patients. © 2010 Lippincott Williams & Wilkins, Inc.