An update on EBV-related cutaneous lymphoproliferative disorders: a systematic review.

Abstract

Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. This systematic review summarizes the main clinical, histological, immunophenotypic and molecular characteristics of B- and T-cell EBV+ LPD that may compromise the skin at diagnosis. B-cell EBV+ LPD include primary cutaneous lymphomas such as EBV-MCU, as well as cutaneous systemic lymphomas that may present such as lymphomatoid granulomatosis (LG), EBV+ diffuse large B cell lymphoma, NOS, plasmablastic lymphoma (PBL), and extracavitary primary effusion lymphoma (EC-PEL). EBV+, EBV-positive polymorphic B cell LPD, or post-transplant lymphoproliferative disorders (PTLD). Regarding T-cell EBV+ LPD, most of these entities are categorized within T/NK-cell lymphoproliferative processes and lymphomas of childhood, including extranodal T/NK lymphoma, and even more exceptional forms such as EBV-positive T-cell centrofollicular lymphoma and intravascular T/NK-cell lymphoma. Diagnosis is based on integrating the clinical, histological, immunohistochemical, and genetic criteria discussed throughout this article. Management is multidisciplinary, posing a challenge for dermatologists and hematologists involved in the management of these patients. Having scientific evidence available in this field is of paramount importance because overtreatment must be carefully avoided.