EBV-positive mucocutaneous ulcers: a presentation of two cases and a brief literature review

Abstract

AbstractMucocutaneous ulcers associated with the Epstein Barr virus constitute an EBV-induced B-cell lymphoproliferative disorder first described in 2010 by Stefan D. Dojcinov et al. These lesions can occur in association with a spectrum of immunosuppressive conditions, including primary immune deficiency, Human Immunodeficiency Virus (HIV) infection, post-transplantation and the use of methotrexate or tumor necrosis factor-alpha (TNF-a) antagonists. Patients clinically present with slowly developing indurated cutaneous and/or mucosal ulcers, especially in the oropharynx. Histopathology reveals circumscribed ulcers containing a mixture of lymphocytes, plasma cells, histiocytes, eosinophils and large transformed cells resembling Hodgkin and Reed-Sternberg cells. The adjacent squamous epithelium presents reactive nuclear atypia and pseudoepitheliomatous hyperplasia. The large transformed cells show positivity for CD20, CD30, Oct-2, PAX5 and EBV. These cells are also positive for MUM1, yet lack CD10 expression, with absent or focal positivity for BCL6. Despite the presence of highly atypical cells, the clinical course is indolent, without progression to disseminated disease. We report herein two cases of diagnosed EBV-positive mucocutaneous ulcers to add to the relatively few cases previously described in the literature.