Epstein–Barr Virus-Positive Mucocutaneous Ulcer: A Unique and Curious Disease Entity

Abstract

Epstein–Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) was first described as a lymphoproliferative disorder in 2010. EBVMCU is a unifocal mucosal or cutaneous ulcer that often occurs after local trauma in patients with immunosuppression; the patients generally have a good prognosis. It is histologically characterized by proliferating EBV-positive atypical B cells accompanied by ulcers. On the basis of conventional pathologic criteria, EBVMCU may be misdiagnosed as EBV-positive diffuse large B-cell lymphoma or other lymphomas. However, its prognosis differs from that of EBV-associated lymphomas, in that patients with EBVMCU frequently show spontaneous regression or complete remission without chemotherapy. Therefore, EBVMCU is now recognized as a low-grade malignancy or a pseudo-malignant lesion. Avoiding unnecessary chemotherapy by distinguishing EBVMCU from other EBV-associated lymphomas will reduce the burden and unnecessary harm on patients. On the basis of these facts, EBVMCU was first described as a new clinicopathological entity by the World Health Organization in 2017. In this review, we discuss the clinicopathological characteristics of previously reported EBVMCU cases, while focusing on up-to-date clinical, pathological, and genetic aspects.

EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 1)

“…EBVMCU involves extra-nodal sites, occurring in the oropharynx (52%), skin (29%) and GIT (19%) [ 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ].…”

“…In immunocompromised hosts, conditions favoring EBVMCU may be represented either by a local trauma (for instance, tooth extraction) or chronic mucosal irritation [ 13 , 15 ]. The superficial nature of the disease and its indolent course might be explained by the occurrence of a slight and localized lapse in immune-surveillance over EBV.…”

“…The histological spectrum of EBVMCU is variable; some cases, with a more polymorphic pattern, simulate cHL and others, with a monomorphic and diffuse pattern of growth, mimic EBV-positive DLBCL-NOS [ 15 , 26 ].…”

“…EBVMCU is characterized by a self-limited course, with either spontaneous remission or regression upon reduction or removal of the immunosuppressive therapy [ 13 , 15 ].…”

“…The review consists of three parts. In this first part, EBVMCU [ 8 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 ], EBV-positive diffuse large B-cell lymphoma not otherwise specified (EBV-positive DLBCL, NOS) [ 8 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 ] and classic Hodgkin lymphoma (cHL) [ 8 ] are discussed.…”