Epstein-Barr Virus–associated Lymphoproliferative Disorders in the Skin
Section snippets
Overview
Epstein-Barr virus (EBV), formally designated human herpesvirus 4, is a ubiquitous gamma herpes virus that infects approximately 90% of the population worldwide.1 In most cases, infection occurs in childhood and is asymptomatic, although a minority who are infected in adolescence or adulthood present with infectious mononucleoisis.2, 3 Following acute infection the virus assumes a latent state in which it persists in circulating B lymphocytes without active viral production. In the latent state
Epstein-Barr virus–associated B-cell lymphoproliferative disorders in the skin
Well-defined EBV-associated lymphomas of B-cell origin, such as Burkitt lymphoma and classic Hodgkin lymphoma, only rarely involve the skin and, when present, this is usually the result of direct extension from underlying nodal disease or a late manifestation of extensive dissemination.11, 12, 13 This article focuses on 3 entities that are either localized to the skin at presentation or represent conditions that frequently present in the skin, or involve cutaneous sites during the course of the
Epstein-Barr virus–associated T-cell/natural killer–cell proliferations encountered in the skin
B cells are the natural hosts for EBV, and most EBV-associated LPDs are of B lineage. However, EBV can also infect T and NK cells in some patients during acute infection and EBV-associated T-cell and NK-cell LPDs are well documented.2, 4, 48, 49, 50 Most of these occur in patients from east Asia and Latin America and include chronic active EBV (CAEBV) infection and the T-cell lymphomas that may follow, extranodal NK-cell/T-cell lymphoma of nasal type,2, 49 aggressive NK-cell leukemia, and a
Summary
EBV-positive cutaneous LPDs may be of B, T, or NK cell type and encompass a broad spectrum of disease. They are rare, and there may be considerable pathologic overlap between cases with shared cells of origin; that is, those of B-cell lineage often resemble one another, as do those of T/NK type. Nonetheless, separation of these entities is important, in view of their different clinical courses and treatments. As is often the case with cutaneous lymphomas, clinicopathologic correlation is key
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Cited by (9)
Cutaneous T-Cell Lymphoma and Cutaneous B-Cell Lymphoma
2019, Abeloff’s Clinical OncologyReexamining post-transplant lymphoproliferative disorders: Newly recognized and enigmatic types
2018, Seminars in Diagnostic PathologyCitation Excerpt :EBV-positive mucocutaneous ulcer (EBV-MCU) is a newly recognized clinico-pathologic entity occurring in patients with age-related or iatrogenic immunosuppression, that presents in mucocutaneous sites (more frequently the oral cavity), follows an indolent clinical course (with frequent spontaneous regression), and is associated with EBV.21 EBV+ chronic mucocutaneous ulcer (EBV-MCU) had been recently described in patients with immunosuppression secondary to azathioprine, cyclosporine, TNF-inhibitors, or methotrexate.22–28 Some cases have also been reported after solid and allogeneic bone marrow transplant.29,30
Acute Epstein–Barr virus infection resembling cutaneous T-cell lymphoma
2023, Journal of Cutaneous PathologyMultiple Progressive Necrotic Lesions in a Young Man: A Quiz
2022, Acta Dermato-VenereologicaCutaneous Lymphomas and Lymphocytic Infiltrates
2021, Atlas of Dermatology, Dermatopathology and Venereology: Cutaneous Infectious and Neoplastic Conditions and Procedural DermatologyEBV-positive mucocutaneous ulcer in a patient with systemic lupus erythematosus
2020, Rheumatology (United Kingdom)
Disclosure: The author has nothing to disclose.