Full clinical recovery after topical acyclovir treatment of Epstein-Barr virus associated cutaneous B-cell lymphoma in patient with mycosis fungoides.

Primary cutaneous Tand B-cell lymphomas are a heterogeneous group of diseases with varied clinical presentations and prognosis. The use of new molecular, histological, and clinical criteria has improved their recognition. Cutaneous B-cell and T-cell lymphomas are seldom found together in the same patient. Here we report a rare case of mycosis fungoides variant of a cutaneous T-cell lymphoma (CTCL) which later developed Epstein-Barr virus (EBV) associated cutaneous B-cell lymphoproliferative disorder. The patient initially presented with generalized erythroderma, extensive plaques, and axillary lymphadenopathy. Histopathology and immunophenotyping of her tumor from the right breast nodule revealed a T-cell lymphoma consistent with mycosis fungoides. She was initially treated with pentostatin, followed by topical mechlorethamine and topical steroids. After progression of her mycosis fungoides with worsening diffuse skin lesions on this regimen, her treatments were changed to oral bexarotene with an initial partial response followed by stable disease. Three years from her initial presentation, she developed ulcerated cauliflower-like nodules on her forehead. Biopsy of these lesions revealed EBV-positive largeand medium-sized pleomorphic B-cells consistent with EBV-driven B-cell lymphoproliferative disorder. She was treated with topical acyclovir cream on the involved skin areas while continuing with oral bexarotene for mycosis fungoides. Skin lesions gradually diminished and totally disappeared after four weeks of topical acyclovir treatment. Bexarotene treatment was continued for another year until the mycosis fungoides progressed and became wide spread causing her death four and a half years after the initial diagnosis. The coexistence of two cutaneous non-Hodgkin lymphomas of different lineage in the same patient and the complete clinical response of EBV-related B-cell cutaneous component to topical acyclovir makes this rare case particularly interesting. Primary cutaneous Band T-cell lymphomas have been recognized as a heterogeneous group of cutaneous non-Hodgkin lymphomas with distinct variability in clinical presentation, histopathology, immunophenotype, genetic abnormalities, and prognosis (1). Primary cutaneous T-cell lymphomas (CTCL) comprise a constellation of heterogeneous lymphoproliferative disorders characterized by clonal accumulation of neoplastic T lymphocytes in the skin. Mycosis fungoides is the most frequent variant of CTCL, clinically characterized by the development of patches, plaques, or tumors. Primary cutaneous B-cell lymphomas are lymphoid neoplasms arising within the skin without evidence of systemic involvement (2). The etiology of both T-cell and B-cell cutaneous lympho458 www.cmj.hr CASE REPORT C ro a t M e d J 2 0 0 5 ;4 6 (3 ): 4 5 8 -4 6 2 mas is largely unknown. Viral infectious etiologies, including human T-lymphocyte virus-1 (HTLV-1), human herpes virus-8 (HHS-8), herpes simplex virus (HSV), hepatitis C virus, and Epstein-Barr Virus have been proposed as causative factors. However, none of these entities has been conclusively associated with either primary cutaneous T-cell or B-cell lymphomas (3-6). There have been occasional reports of CTCL associated with B-cell lymphoproliferative disorders, including plasma cell dyscrasia, chronic lymphocytic leukemia (CLL), and B-cell lymphomas (7-9). The coexistence of Tand B-cell cutaneous lymphoma in the same patient, however, is very rare. Here we report a case of Epstein-Barr virus (EBV) related B-cell cutaneous lymphoproliferative disorder (LPD) in a patient with CTCL wherein the B-cell LPD had a complete clinical response to topical acyclovir treatment.