An Epstein-Barr virus-associated lymphoproliferative lesion of the skin presenting as recurrent necrotic papulovesicles of the face

Seoul National University, Sŏul, Seoul, South Korea
British Journal of Dermatology (Impact Factor: 4.28). 05/1996; 134(4):791-6. DOI: 10.1046/j.1365-2133.1996.99812.x
Source: PubMed


We describe four patients with lymphoproliferative lesions confined to the skin for several years. They presented with recurrent necrotic papulovesicles of the face. Latent Epstein-Barr virus (EBV) infection was detected in the lymphoid cells from the skin lesions by in situ hybridization. The disease in three patients progressed to T-cell lymphoma. We believe that these patients represent a subset of peripheral T-cell lymphoma with a tendency to localize in the skin.

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    • "Hydroa vacciniforme-like lymphoma (HVLL) has also been called angiocentric cutaneous T-cell lymphoma of childhood [3], and hydroa-like cutaneous T-cell lymphoma [4-6]. Most reports come from Asian area, including Japan, China and Korea [7]. "
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    ABSTRACT: Virtual slides The virtual slide(s) for this article can be found here: Hydroa vacciniforme-like lymphoma (HVL) is a rare type of Epstein-Barr virus (EBV)-positive lymphoma of cytotoxic T-cell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme (HV). In current study, we report an adult patient with the tumor. The patient presented similar morphologic, immunophenotypic and genotypic changes of the disease with that occurred in children, whereas clinically, he showed a prolonged clinical course without hepatosplenomegaly or generalized lymphadenopathy. Whether there are some differences in biologic behavior between children and adults still remains unknown and it is necessary to collect more data to observe and to investigate in the future.
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    ABSTRACT: T and NK cell proliferative diseases associated with acute and chronic Epstein–Barr virus (EBV) infection are uncommon and raise several clinical issues regarding diagnostic criteria and terminology. This is a summary report of the consensus meeting held in the 4th Asian Hematopathology Workshop. The umbrella term “EBV-positive T/NK lymphoproliferative disease in childhood-type” covers the entire spectrum of EBV-associated lesions in childhood, ranging from reactive to neoplastic processes. Systemic T/NK cell lymphoproliferative disease (LPD) of childhood type is defined as a fulminant disease associated with the proliferation of polyclonal, oligoclonal, or monoclonal T or NK cells and includes aggressive NK cell-associated leukemia in children. Chronic active EBV disease-type T/NK cell LPD is divided into three groups—polymorphic/polyclonal, polymorphic/monoclonal, and monomorphic/monoclonal—based on the histology and clonality of T or NK cells. A monoclonal EBV-positive T/NK cell type of proliferation with the clinical features of chronic active EBV disease rather than the fulminant course of systemic EBV-positive T/NK cell LPD of childhood is considered “chronic active EBV disease-type T/NK cell LPD”. Hydroa vacciniforme (HV)-like T cell LPD is defined as a spectrum of EBV-infected T cell proliferative diseases with homing to the skin and is further classified into a classic type, a severe type, and malignant lymphoma. Criteria to define each category of HV-like T cell LPD remain to be clarified.
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