Epstein-Barr Virus-Positive Mucocutaneous Ulcer in an Immunosuppressed Patient

Abstract

Immunosuppressive medications, frequently used to treat inflammatory bowel disease, have been linked to the development of Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPD). We describe a case of an EBV-positive mucocutaneous ulcer involving the palate in an elderly woman with inactive Crohn’s disease. This patient had been on high-dose azathioprine for a decade. Following diagnosis of her LPD and discontinuation of azathioprine, her oral ulcers resolved completely.

A rare case of Epstein–Barr virus-positive mucocutaneous ulcer that developed into an intestinal obstruction: a case report

“…In Because no diagnostic criteria have been established yet for EBV-MCU, it is diagnosed on the basis of clinical background factors and histopathological findings [2][3][4][5][6][7][8][9][10][11][12][13]. It has been reported that the characteristic histopathological findings of EBV-MCU include infiltration of various cells, various immunohistochemical patterns such as CD20 and CD30 positivity, the detection of EBER-positive cells by ISH, among which HRS-like B cells are found [2][3][4][5][6][7][8][9][10][11][12][13]. In the present case, the initial histopathological findings of the biopsy specimen collected from the lesion were nonspecific, which made the diagnosis difficult.…”

“…It is suggested that the onset of EBV-MCU is related to the decreased immunity of a patient, the causes of which include a history of organ transplantation, use of immunosuppressive agents, and aging [2][3][4][5][6][7][8][9][10][11][12][13]. Roberts et al reviewed the cases of 51 patients with EBV-MCU (including those of the 26 patients reported by Dojcinov et al) and reported that the causes of EBV-MCU were the use of immunosuppressive agents (methotrexate, azathioprine, cyclosporine, mycophenolate, or tacrolimus) in 56% of the patients, aging (median age, 80 years; range, 64-101 years) in 40%, and congenital immunosuppression (hypogammaglobulinemia and T-cell deficiency) in 4% [9].…”

“…It has been reported that most patients with EBV-MCU had a benign course after the dose reduction or discontinuation of immunosuppressive agents or during the follow-up [2,4,5,9,10,13]. According to the summary of 51 patients with EBV-MCU reported by Roberts et al, 19 of the 45 patients with complete treatment records were treated only by reducing the doses of immunosuppressive agents, and 17 of them attained a complete remission.…”

“…Epstein-Barr virus-positive mucocutaneous ulcer (EBV-MCU) is a new category of mature B-cell neoplasms and was formally adopted in the World Health Organization (WHO) classification of lymphoid neoplasms updated in 2016 [1]. The results of previous studies suggest that the onset of EBV-MCU is related to the decreased immunity of the patient, the causes of which include primary or acquired immunosuppression, iatrogenic immunosuppression by immunosuppressive agents, and aging [2][3][4][5][6][7][8][9][10][11][12][13]. Ulcers occur in the oropharyngeal mucosa, skin, and gastrointestinal tract.…”

“…Ulcers occur in the oropharyngeal mucosa, skin, and gastrointestinal tract. They may regress spontaneously and often have a benign course after the dose reduction or discontinuation of immunosuppressive agents or during follow-up [2,4,5,9,10,13]. We report the case of an elderly male patient who required surgical resection for the intestinal obstruction arising from EBV-MCU.…”