Epstein-Barr Virus–associated Lymphoproliferative Disorders in the Skin

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Abstract

Epstein-Barr virus (EBV)–associated lymphoproliferations involving the skin are a rare but important group of diseases with a broad spectrum of behavior, ranging from self-limiting spontaneously resolving disorders to highly aggressive malignancies. They may be of B, T, or natural killer (NK) cell type and include EBV-positive mucocutaneous ulcer, lymphomatoid granulomatosis, EBV-positive diffuse large B-cell lymphoma, hydroa vacciniforme–like lymphoproliferative disorder, and extranodal NK/T-cell lymphoma of nasal type. Recognition and distinction of these entities is important in view of their differing prognoses and treatments. An association with EBV may be the first indication that a patient is immunosuppressed.

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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    Disclosure: The author has nothing to disclose.

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