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  T-Cell Lymphoma of the Skin
A Clinical, Microscopic, and Molecular Study
Jorge R. Toro, MD;
Micheal Beaty, MD;
Lynn Sorbara, PhD;
Maria L. Turner, MD;
Jeffrey White, MD;
Douglas W. Kingma, MD;
Mark Raffeld, MD;
Elaine S. Jaffe, MD
Arch Dermatol. 2000;136:1024-1032.
Background Only a few cases of primary   cutaneous T-cell lymphoma (CTCL) have been reported. We encountered 3 cases of this rare condition.
Objectives To characterize CTCL by clinical, microscopic, and molecular methods and to investigate the role of Epstein-Barr virus (EBV) infection in its pathogenesis.
Design Patients were evaluated by clinical examination, and biopsy specimens of lesional skin were examined by light microscopy and immunohistochemistry. Polymerase chain reaction amplification for T-cell receptor gene rearrangements and in situ hybridization for EBV were performed on 3 biopsy specimens.
Setting National Institutes of Health, a tertiary referral center.
Patients Individuals with a clinical and histologic diagnosis of primary CTCL.
Outcome Measures Clinical, light microscopic, and immunohistochemical features, and the presence of T-cell rearrangement and EBV RNA in biopsy specimens.
Results Patients exhibited multiple plaques, tumors, and/or subcutaneous nodules primarily distributed over the extremities. Individuals exhibited an aggressive clinical course with resistance to multiagent chemotherapy and radiation. Microscopic examination revealed epidermotropism in 2 cases, a dermal infiltrate in all 3 cases, and subcutaneous involvement in 1 case. Immunohistochemical studies showed the presence of CD3+TCR+ in 3 patients, CD8+in 1, and CD4+, CD20+, CD56+, and  F1+ in none. All 3 cases exhibited an activated cytotoxic T-cell phenotype positive for T-cell intracellular antigen 1, perforin, and granzyme B. A clonal T-cell receptor chain gene rearrangement was detected in all 3 cases by polymerase chain reaction. In situ hybridization was negative for EBV sequences in all 3 cases.
Conclusion Cutaneous T-cell lymphomas are EBV-negative lymphomas that express a mature cytotoxic phenotype and have an aggressive clinical behavior.
From the Dermatology Branch (Drs Toro and Turner), Hematopathology Section (Drs Beaty, Sorbara, Kingma, and Jaffe), Metabolism Branch (Dr White), and Laboratory of Pathology (Dr Raffeld), National Cancer Institute, National Institutes of Health, Bethesda, Md.
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