EBV-positive B-cell Ulcerative Proliferation in the Oral Cavity Associated with EBV-negative Follicular Lymphoma in a Patient with Common Variable Immunodeficiency: A Case Report and Review of the Literature

Abstract Lymphoproliferative disorders (LPD) are a group of conditions characterized by excessive production of lymphocytes manifested in various patterns including lymphadenopathy, tumor-like lesions, and lymphomas. LPD may be stimulated by Epstein Barr Virus (EBV) infection that most commonly appears in the setting of immunocompromised status such as long-term use of immunosuppressive medications and in Individuals with primary immunodeficiency disorders. EBV mucocutaneous ulcer (EBVMCU) is a benign LPD reaction that mostly regresses spontaneously while sometimes requires medical or surgical intervention. This article presents a case of oral EBVMCU ulceration that affected an individual with a history of complex primary immunodeficiency disorders consisted of common variable immunodeficiency disease (CVID) associated with T cell dysfunction. This case is unique since the oral lesions led to the diagnosis of concurrent widespread EBV-negative follicular lymphomas, seemingly unrelated to her EBV-positive LPD oral disease. Yet, both occurred in the setting of severe immunosuppression from the primary immunodeficiency disorders.