An Update on EBV-related Cutaneous Lymphoproliferative Disorders: a Systematic Review

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. 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. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies. 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-Mucocutaneous Ulcer, as well as systemic lymphomas affecting the skin at diagnosis that may present such as lymphomatoid granulomatosis (LG), EBV diffuse large B cell lymphoma, NOS, plasmablastic lymphoma (PBL), Extracavitary primary effusion lymphoma (EC-PEL) EBV+, EBV-positive polymorphic B cell LPD, and 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. Differential diagnosis is a challenge for dermatologists and pathologists, so having scientific evidence available in this field is of paramount importance because overtreatment must be carefully avoided.