Azathioprine induced Epstein-Barr virus positive mucocutaneous ulcer: A case report

Introduction: Epstein-Barr virus positive mucocutaneous ulcer (EBVMUC) is a rare, newly described provisional entity in the 2016 Update of World Health Organization classification of lymphoid neoplasms. The histomorphological and immunophenotypical and molecular features overlap with classical Hodgkin’s lymphoma (cHL) and can be mistaken for the same. Case report: A 70-year-old male, a known case of diabetes mellitus and hypertension, was diagnosed with Wegener’s granulomatosis (granulomatosis with polyangiitis) in 2007. He was treated with prednisolone and cyclophosphamide, the latter drug was replaced with azathioprine in 2010. He was apparently well since then, until he presented in 2016 with an anal ulcer with a fistula tract formation, the ulcer on histomorphology and immunohistochemistry was diagnosed as EBVMUC. Discussion: EBVMUC was first described in patients with iatrogenic induced immunosuppression.They have later been found to be associated with various other causes of immunosuppression, like solid organ transplant recipients and human immunodeficiency virus (HIV), common factor in all these being immunosuppression. Conclusion: The importance of recognizing this entity lies in its morphological and immunophenotypic overlap with classical Hodgkin’s lymphoma (cHL) and unlike latter, most often complete resolution of disease occurs with reduction of immunosuppressive dose. Therefore, correct recognition of the entity is essential to avoid overtreatment as lymphoma.