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Primary Cutaneous Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disorder-2 Cases With Unusual, Prolonged Clinical Course

Park, Sanghui MD*; Lee, Dong-Youn MD, PhD†; Kim, Won Seog MD, PhD‡; Ko, Young-Hyeh MD, PhD§

American Journal of Dermatopathology: December 2010 - Volume 32 - Issue 8 - pp 832-836
doi: 10.1097/DAD.0b013e3181d68381
Extraordinary Case Report

Hydroa vacciniforme-like lymphoma and extranodal-type natural killer (NK)/T-cell lymphoma are prototypes of Epstein-Barr virus (EBV)-associated cutaneous T- or NK-cell lymphomas. Hydroa vacciniforme-like lymphoma with systemic spread and extranodal-type NK/T-cell lymphoma are characterized by aggressive clinical course. We describe 2 patients with primary cutaneous EBV-associated T-cell lymphomas who did not satisfy the criteria for well-defined entities and showed unusual, prolonged clinical course. They presented with skin ulcerations and mass lesions confined to the extremities without systemic involvement. Skin biopsies demonstrated dense superficial and deep perivascular and periappendageal lymphoid infiltrates expressing CD3 and CD8, but not CD56. The EBV genomes were found within the tumor cells, and monoclonal T-cell receptor gene rearrangement was present. We suggest that these cases represent a peculiar subtype of EBV+ cutaneous T-cell lymphoma, with a tendency to localize in the skin of the extremities and has an indolent clinical course.

From the *Department of Pathology, Gil Medical Center, Gachon University, School of Medicine and Science, Incheon, Korea; †Department of Dermatology; ‡Division of Hematology and Oncology, Internal Medicine; and §Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Reprints: Young-Hyeh Ko, MD, PhD, Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Ilwon-dong 50, Kangnam-gu, Seoul, Korea (e-mail: yhko310@skku.edu).

© 2010 Lippincott Williams & Wilkins, Inc.