Virus-positive Mucocutaneous Ulcer Research Articles

Epstein-Barr Virus-Positive Mucocutaneous Ulcer With Medication-Related Osteonecrosis of the Jaw Arising in the Mandible of a Rheumatoid Arthritis Patient: A Case Report

Epstein-Barr Virus-Positive Mucocutaneous Ulcer With Medication-Related Osteonecrosis of the Jaw Arising in the Mandible of a Rheumatoid Arthritis Patient: A Case Report

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.

The classification of primary cutaneous lymphomas and lymphoproliferative disorders (LPD) is continuously evolving by integrating novel clinical, pathological and molecular data. Recently two new classifications for haematological malignancies including entities of cutaneous lymphomas were proposed: the 5th edition of the WHO classification of haematolymphoid tumours and the International Consensus Classification (ICC) of mature lymphoid neoplasms. This article provides an overview of the changes introduced in these two classifications compared to the previous WHO classification. The main changes shared by both classifications include the downgrading of CD8+ acral T-cell lymphoma to CD8+ acral T-cell LPD, and the recognition of entities that were previously categorized as provisional and have now been designated as definite types including primary cutaneous small or medium CD4+ T-cell LPD, primary cutaneous gamma/delta T-cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, Epstein-Barr virus-positive mucocutaneous ulcer. Both classifications consider primary cutaneous marginal zone B-cell clonal neoplasm as an indolent disease but use a different terminology: primary cutaneous marginal zone lymphoma (WHO) and primary cutaneous marginal zone LPD (ICC). The 5th WHO classification further introduces and provides essential and desirable diagnostic criteria for each disease type and includes chapters on reactive B- or T-cell rich lymphoid proliferations formerly referred as cutaneous pseudolymphomas, as well as histiocyte and CD8 T-cell rich LPD in patients with inborn error of immunity. As already emphasized in previous lymphoma classifications, the importance of integrating clinical, histological, phenotypic and molecular features remains the crucial conceptual base for defining cutaneous (and extracutaneous) lymphomas.

Diagnostic utility of cytology in a patient with Epstein-Barr virus-positive mucocutaneous ulcer in palatine tonsils: A case report.

Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU) is a newly established immunodeficiency-related disease. Herein, we report a case of EBVMCU and focus on its cytological usefulness for diagnosis. An 82-year-old man manifested pharyngalgia, dysphagia, and oral pain. His medical history included rheumatoid arthritis that had been treated with methotrexate. Clinically, peritonsillar abscess was suspected, but since neoplastic lesions, including malignant lymphoma (ML), could not be excluded, a series of cytohistological examination was attempted. Despite some alarming findings (e.g., frequent mitoses), fine-needle aspiration and touch imprint cytology consistently revealed a heterogeneous population of lymphoid and plasmacytoid cells with mild nuclear atypia. The final diagnosis of EBVMCU was established based on the permanent histologic specimen; however, retrospectively, cytology was more representative of the benign nature of the lesion than histology, helping a great deal to differentiate it from ML. Cytology can be a useful tool for the correct diagnosis of EBVMCU.

Epstein-Barr virus-positive mucocutaneous ulcer in a patient with ulcerative colitis: a recently described entity to take into account.

Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU) is a new entity recently included in the classification of B-cell lymphoproliferative disorders associated with Epstein-Barr virus (EBV). It is related to immunosuppression and it usually appears in the oropharynx or the skin, being the colon an uncommon location. We present the case of a 31-year-old man with ulcerative proctitis being treated with azathioprine (AZA) and adalimumab (ADA), who was admitted to the hospital due to suspicion of a moderate-severe flare of ulcerative proctitis. Microbiological stool analyses were negative, with a positive fecal calprotectin test (2700 mg/kg). Rectoscopy showed severe endoscopic activity, taking multiple biopsies. Intravenous steroids were started at a dose of 60 mg/day. He presented a favorable clinical and analytical evolution, being discharged from the hospital. The histological results were received at gastroenterology consultation, being compatible with EBVMCU. AZA and ADA were withdrawn, whereas descending steroid regimen and oral and topical mesalazine were continued, being the clinical response adequate.

Epstein-Barr virus positive mucocutaneous ulcer – A diagnostic challenge

A significant portion of the global population carries the asymptomatic Epstein-Barr virus (EBV). Transfusions of blood and blood derivatives, organ and tissue transplantation, and oropharyngeal secretions are the main routes of transmission. The World Health Organization recently recognized the EBV mucocutaneous ulcer (EBVMU) as a provisional pathological entity in its classification of hematopoietic and lymphoid tissues. It affects patients who are elderly or immunosuppressed. Histologically, it may be difficult to distinguish from Hodgkin lymphoma (HL). Although skin or mucosa are uncommon sites of HL involvement, diagnosis at these sites should be made with extreme caution. The prognosis for EBVMU is good; cases regress on their own or after immunosuppressive therapy is reduced. We present a 67-year-old woman with a non-healing oral cavity ulcer which was diagnosed as EBV-positive mucocutaneous ulcer.

A Case of Epstein-Barr Virus-Positive Mucocutaneous Ulcer Arising in an Anal Lesion

A Case of Epstein-Barr Virus-Positive Mucocutaneous Ulcer Arising in an Anal Lesion

Epstein-Barr virus-positive mucocutaneous ulcer of the gingiva: A self-limiting disorder of the immune compromised that can present with aggressive clinico-radiological and histomorphological features

Oral ulcerations of the gingiva or mucosa require differential diagnosis. One of these diagnoses may be Epstein-Barr virus (EBV)-positive mucocutaneous ulcer.Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) is a rare diagnosis and was first described in 2010. It is described as an Epstein-Barr virus (EBV)-mediated B-cell proliferation and occurs in patients with immunosuppression, e.g., therapy with azathioprine, methotrexate, or cyclosporine, but also in patients with age-related immune senescence or chronic diseases/infections such as diabetes and AIDS/HIV. In 2017, EBVMCU was recognized by WHO and included in the classification of tumors.Mucocutaneous ulcerations often present as indolent, sharply demarcated ulcerations of the gingiva. Over 70 % of these ulcerations occur in the oral and pharyngeal cavities. EBV is secreted in saliva and can develop EBVMCU in association with trauma, such as tooth extraction.We present three patient cases with EBVMCU. We describe the clinical presentation, differential diagnosis and diagnosis, treatment, and clinical outcome.

S4127 Spontaneous Regression of a Rare Gastric Epstein-Barr Virus-Positive Mucocutaneous Ulcer, an Entity in Age-Related Immunosenescence

S4127 Spontaneous Regression of a Rare Gastric Epstein-Barr Virus-Positive Mucocutaneous Ulcer, an Entity in Age-Related Immunosenescence

What is new about primary cutaneous B cell lymphomas.

Primary cutaneous B-cell lymphomas (CBCL) are a heterogeneous group of B-cell lymphomas without evidence of extracutaneous disease at the time of diagnosis. The 2022 World Health Organization classification of mature lymphoid neoplasms differentiates the indolent primary cutaneous marginal zone lymphoproliferative disorder, primary cutaneous follicle center lymphoma and Epstein-Barr virus-positive mucocutaneous ulcer, from the more aggressive primary cutaneous diffuse large B-cell lymphoma, leg-type and intravascular large B-cell lymphoma. The new updates in the 2022 classification are based on recent scientific advances in the understanding and characterization of these entities. This article aims to review the main clinical, cellular and molecular features of the five CBCL subsets along with their management and treatment. The exponentially growing evidence for new treatment options for systemic B-cell lymphomas raises expectations for the field of CBCL as well. However, specific prospective high quality research on CBCL is still crucial to further define their management and update international guidelines.

Clinicopathological analysis of three cases EB virus-positive mucocutaneous ulcer

目的： 探讨EB病毒阳性皮肤黏膜溃疡（EBVMCU）的临床及病理学特征。 方法： 回顾性分析2017至2021年烟台毓璜顶医院确诊的3例EBVMCU，采用免疫组织化学、原位杂交染色和分子重排技术检测，并复习相关文献。 结果： 3例患者中男性1例，女性2例，年龄分别为65、75和69岁。3例均为年龄相关性EBVMCU。病变局限于口腔黏膜2例、胃1例。1例有局部淋巴结肿大，3例均无肝、脾、骨髓受累，经保守治疗或化学药物治疗，预后均良好。3例EBVMCU患者在形态学上表现混杂的炎性细胞背景中见中至大的异型细胞浸润，部分形态似R-S细胞；异型细胞表达CD20、CD30、MUM1，Ki-67阳性指数高。3例病例原位杂交EB病毒编码的RNA（EBER）均阳性，免疫球蛋白分子重排结果均阴性。 结论： EBVMCU少见，发生在高龄、医源性免疫抑制和自身免疫缺陷人群，口腔黏膜是好发部位。3例病例未出现免疫球蛋白克隆性分子重排。.

EBV-positive mucocutaneous ulcer arising in methotrexate-treated rheumatoid arthritis patients: a clinicopathological study of 12 cases with analysis of PD-L1 expression.

Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU) is a newly recognized disease entity characterized by EBV-positive atypical B-cell proliferation. EBVMCU is a localized self-limited disease that affects mucosa and skin, especially the oral cavity. EBVMCU develops in immunosuppressive patients, such as those with methotrexate (MTX)-administrated rheumatoid arthritis (RA). Here we clinicopathologically analyzed 12 EBVMCU patients in a single institution. All cases were administrated MTX for RA, and five cases occurred in the oral cavity. All cases except one had demonstrated spontaneous regression after withdrawal of the immunosuppressive agent. We found 4 of 5 cases in the oral cavity had preceding traumatic events in the same site within a week before the onset of EBVMCU. Although there is no detailed and large study that has analyzed the trigger of EBVMCU, a traumatic event would indeed be a significant trigger for EBVMCU in the oral cavity. The cases were histologically classified; six cases were diffuse large B-cell lymphoma-type, five were polymorphous-type, and one was Hodgkin-like lesion type due to morphological appearance and immunophenotype. The PD-L1 expression was also examined by two antibodies for PD-L1 (E1J2J and SP142). Both antibodies revealed identical results for PD-L1 expression, and three cases were positive for PD-L1. The application of SP142 for evaluating the immune status of lymphomagenesis has also been proposed. Nine of 12 cases were negative for PD-L1, which implies that most EBVMCU cases may be caused by an immunodeficiency, rather than an immune-evasion, mechanism. However, as three cases were positive for PD-L1, immune escape may underly the pathogenesis in a subset of EBVMCU cases.

Epstein-Barr virus–positive mucocutaneous ulcer of the stomach

Epstein-Barr virus–positive mucocutaneous ulcer of the stomach

EPSTEIN-BARR VIRUS–POSITIVE MUCOCUTANEOUS ULCER: A CASE REPORT

Epstein-Barr virus–positive mucocutaneous ulcer (EBVMCU) is a newly recognized clinicopathologic entity included in the group of mature B-cell neoplasms in the 2017 revision of World Health Organization Classification for Lymphoproliferative Disorders. A nonsmoking 92-year-old man presented with an ulcerated lesion on the maxillary alveolar ridge. An incisional biopsy was performed and reported an undifferentiated malignancy composed of cell clusters with scarce cytoplasm and very pleomorphic nuclei. The immunohistochemical profile expressed CD20, CD30, CD15, CD45 and Epstein-Barr virus-encoded latent membrane protein (EBV-LMP) antigens, consistent with EBVMCU. Within 2 weeks, the lesion became painful and the palate was infiltrated. Tomography showed loss of bone tissue and permeative aspect in the right maxilla. After 8 weeks of rituximab, the patient was asymptomatic and presented regression of the lesion, maintaining only the bone exposed area. This case highlights that proper clinical history and ancillary studies can avoid misinterpretation and overtreatment of EBVMCU oral lesions. Epstein-Barr virus–positive mucocutaneous ulcer (EBVMCU) is a newly recognized clinicopathologic entity included in the group of mature B-cell neoplasms in the 2017 revision of World Health Organization Classification for Lymphoproliferative Disorders. A nonsmoking 92-year-old man presented with an ulcerated lesion on the maxillary alveolar ridge. An incisional biopsy was performed and reported an undifferentiated malignancy composed of cell clusters with scarce cytoplasm and very pleomorphic nuclei. The immunohistochemical profile expressed CD20, CD30, CD15, CD45 and Epstein-Barr virus-encoded latent membrane protein (EBV-LMP) antigens, consistent with EBVMCU. Within 2 weeks, the lesion became painful and the palate was infiltrated. Tomography showed loss of bone tissue and permeative aspect in the right maxilla. After 8 weeks of rituximab, the patient was asymptomatic and presented regression of the lesion, maintaining only the bone exposed area. This case highlights that proper clinical history and ancillary studies can avoid misinterpretation and overtreatment of EBVMCU oral lesions.

Epstein–Barr virus-positive mucocutaneous ulcer: A rare case report from Riyadh, Saudi Arabia

Epstein–Barr virus-positive mucocutaneous ulcer: A rare case report from Riyadh, Saudi Arabia

Epstein-Barr virus-positive mucocutaneous ulcer arising in the oral cavity of MTX-treated patients: Report of three cases and review of 25 cases reported in Japan

Epstein-Barr virus-positive mucocutaneous ulcer arising in the oral cavity of MTX-treated patients: Report of three cases and review of 25 cases reported in Japan

Age Related Immunosenescence Epstein-Barr Virus-positive Mucocutaneous Ulcer of the Palate Mimicking Medication-related Osteonecrosis of the Jaw.

Epstein-Barr virus (EBV) -positive mucocutaneous ulcer (EBVMCU) was first described as a lymphoproliferative disorder in 2010. In recent years, EBVMCU has been reported in the field of oral surgery. On the other hand, medication-related osteonecrosis of the jaw (MRONJ) is an osteomyelitis that occurs in patients receiving antiresorptive agents including bisphosphonates (BP) and/or denosumab developing with bacterial infections such as dental diseases and mucositis. MRONJ caused by EBVMCU in the elderly has not been reported. Here, we report a rare case of MRONJ caused by EBVMCU in the elderly. The patient, an 82-year-old woman, had received BP for more than 2years. An ulcerative lesion was found in the palatal mucosa; biopsy performed from the site confirmed the diagnosis of EBVMCU. At follow-up, the lesion disappeared spontaneously. At the 6-month follow-up, bone formation was observed at the site of the lesion, and the sequestrum was removed. At the 12-month follow-up healing of the EBVMCU region was seen indicating a good prognosis.

Two Cases of Epstein-Barr Virus-Positive Mucocutaneous Ulcer Mimicking Head and Neck Cancers in 18F-FDG PET/CT.

Epstein-Barr virus-positive mucocutaneous ulcer is a newly recognized clinicopathological entity among mature B-cell neoplasms according to the 2016 revision of the World Health Organization diagnostic criteria. Here, we present FDG PET/CT images of 2 Epstein-Barr virus-positive mucocutaneous ulcer cases. Both cases shown in the images mimicked head and neck cancers, which are similar to carcinomas of the tonsil and gingiva, respectively, and both lesions showed intense FDG uptake on PET scan.

Clinicopathological characteristics of lymphoproliferative disorders in 232 patients with rheumatoid arthritis in Japan: A retrospective, multicenter, descriptive study.

To describe the clinicopathological characteristics of lymphoproliferative disorders (LPDs) in patients with rheumatoid arthritis (RA). In this multicenter case series, we retrospectively reviewed the medical records of RA patients who were newly diagnosed as having LPDs with or without biopsy confirmation between 2000 and 2017 in eight hospitals in Japan. We included 232 patients with LPDs. The median age was 67 years (interquartile range [IQR], 60-73 years), and 77.1% were female. At the time of LPD diagnosis, 94.8% and 62.6% of the patients were methotrexate users and in remission or had low RA disease activity, respectively; lymphadenopathy and extranodal involvement were present in 77.1% and 51.9%, respectively. Major extranodal sites were the lungs and oral/oropharyngeal mucosa. The most common LPD pathological subtype was diffuse large B-cell lymphoma (40.5%), followed by classic Hodgkin lymphoma (10.8%), Epstein-Barr virus-positive mucocutaneous ulcer (7.7%), and reactive lymphoid hyperplasia (6.2%). The clinical and laboratory characteristics varied across the pathological subtypes. LPD occurred mainly in methotrexate users, while RA disease activity did not seem to be associated with LPD development. Although the clinical manifestations vary among pathological subtypes, manifestations of LPD in patients with RA can include lymphadenopathy, extranodal mass, and mucocutaneous ulcer.

Characteristics of rheumatoid arthritis with immunodeficiency-associated lymphoproliferative disorders to regress spontaneously by the withdrawal of methotrexate and their clinical course: A retrospective, multicenter, case-control study.

To investigate clinical characteristics and time course of lymphoproliferative disorders (LPDs) in rheumatoid arthritis (RA) patients after methotrexate (MTX) discontinuation, in those who achieved spontaneous regression (SR). We retrospectively reviewed clinical data from RA patients with LPDs obtained from eight institutions between 2000 and 2017 and compared clinical and pathological findings between SR and non-SR groups. Among 232 RA patients with LPDs, 216 were treated with MTX at the onset of LPD and 144 (66.7%) achieved SR after MTX discontinuation. Higher MTX doses, high titers of anti-CCP antibodies (>13.5 U/mL), and lower LDH and soluble IL-2 receptor levels were associated with SR. Lymphocyte count was decreased at LPD onset and increased at 2 weeks after MTX discontinuation in the SR group. Epstein-Barr virus-positive mucocutaneous ulcer, reactive lymphoid hyperplasia and unclassifiable B-cell lymphoma, were more frequent in the SR than in the non-SR group. In multivariable analysis, diffuse large B-cell lymphomas was an independent predictive factor for non-SR. In the patients with SR, 73.9% achieved partial or complete regression as early as 2 weeks after MTX discontinuation. SR and non-SR in RA patients with LPDs after MTX discontinuation were associated with certain clinical characteristics.

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

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.