A review of EBV-positive mucocutaneous ulcers focusing on clinical and pathological aspects

Abstract

Epstein-Barr virus (EBV)-positive mucocutaneous ulcers (EBVMCUs) were first described as a lymphoproliferative disorder in 2010. Clinically, EBVMCUs are shallow, sharply circumscribed, unifocal mucosal or cutaneous ulcers that occur in immunosuppressed patients, including those with advanced age-associated immunosenescence, iatrogenic immunosuppression, primary immune disorders, and HIV/AIDS-associated immune deficiencies. In general, patients exhibit indolent disease progression and spontaneous regression. Histologically, EBVMCUs are characterized by the proliferation of EBV-positive, variable-sized, atypical B-cells. According to conventional histopathologic criteria, EBVMCUs may diagnosed as lymphomas. However, EBVMCUs are recognized as pseudomalignant lesions because they spontaneously regress without anti-cancer treatment. Therefore, overtreatment must be carefully avoided and multilateral differentiation is important. In this article, we reviewed previously reported EBVMCUs focusing on their clinical and pathological aspects in comparison with other EBV-positive B-cell neoplasms.

Methotrexate-associated lymphoproliferative disorder demonstrating composite lymphoma of EBV-negative diffuse large B-cell lymphoma and EBV-positive mucocutaneous ulcer

“…The first point is the importance of multiple biopsy for MTX-LPD. MTX-LPD are mainly DLBCL and CHL, but rarely FL, 9 peripheral T-cell lymphoma, 8 etc. The pathological features are different, but all cases have the potential for tumor regression by MTX withdrawal.…”

Select Epstein-Barr Virus–Associated Digestive Tract Lesions for the Practicing Pathologist

“…22 The ulcer mostly occurs in elderly patients at a median age of 66.4 years (range, 16-101 years) and exhibits a female predominance. [22][23][24] Patients usually present with a solitary, painful ulcer unassociated with hepatic, splenic, systemic lymph node, or bone marrow involvement, although regional lymph node involvement and multifocality (17% of the cases) have been described. 21,22,25 The blood viral load is negative in almost all cases.…”

Challenging diagnoses in oral ulcers with large atypical CD30+ cells: EBV-positive mucocutaneous ulcer differentials

“…The authors explained many clinical and pathological aspects of this disease which pathologists must be attentive to during their diagnostic routine. At the end of the article, 1 the authors pointed out that clinicians and pathologists need to be able to distinguish EBVMCU from lymphomas, especially classic Hodgkin lymphoma, because of the Reed-Sternberg cells with positivity for CD30. Although malignancies are important, we must not forget benign lymphoproliferations that may be a differential diagnosis for EBVMCU.…”

“…EBVMCU is recognized as a clinicopathological entity in the last WHO classification of hematological malignancies, 1,4 with Hodgkin-like features and a self-limited indolent course, generally responding well to conservative management, and associated with iatrogenic immunosuppression or age-related immunosenescence. 1,4 EUOM is a rare self-limited oral condition, best regarded as a reactive pattern of unclear etiology, although a local traumatic event has been often incriminated. 2 Both entities manifest themselves as an oral ulcer and the observation of large CD30+ cells could simulate an oral lymphoma, such as CD30+ primary cutaneous lymphoproliferative disorders, 2 and Hodgkin lymphoma 5 (Table 1).…”

“…Challenging diagnoses in oral ulcers with large atypical CD30+ cells: EBV-positive mucocutaneous ulcer differentialsKeywords: oral ulcer, lymphoma, CD30 antigen, immunosuppression, Epstein-Barr virus of Ikeda et al (2019),1 improving clinical and pathological reasoning when faced with these challenging diseases.…”