EBV-positive mucocutaneous ulcer in a patient with systemic lupus erythematosus

Ritter, Clarice Gabardo; Cruz, Camila De David; Zamboni, Sheron; Busato, Vanessa Barrili; Silveira, Romulo Gomes da; Provenzi, Valentina O.; Bredemeier, Markus

doi:10.1093/rheumatology/kez393

Cited by 3 publications

(3 citation statements)

References 2 publications

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“…13 Reports of cutaneous B-cell MTX-LPD, particularly when lesions are skin-limited, are reasonably rare and they can present with nodules, plaques, or ulcers, 3,14–34 including some cases characterized as EBVMCU. 6,27,35–38 Predominantly, they develop in the context of autoimmune conditions treated with methotrexate, although they have also been reported to arise after treatment of T-cell neoplasms, including mycosis fungoides, Sézary syndrome, and T-cell acute lymphoblastic leukemia. 25,39–43 MTX-LPD can represent a potential serious diagnostic pitfall because their histology may mimic high-grade lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Skin-Limited, Methotrexate-Associated Epstein–Barr Virus–Positive Mucocutaneous Ulcer—A Mimicker of High-Grade Lymphoma. A Report of 4 Cases and Review of the Literature

Macklin

Stonard

Matin

et al. 2023

The American Journal of Dermatopathology

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Immunodeficiency-associated lymphoproliferative disorders (IA-LPDs) constitute a diverse range of conditions including posttransplant lymphoproliferative disorders, other iatrogenic IA-LPDs, and lymphoproliferative disorders associated with an underlying primary immune disorder or HIV infection. IA-LPDs are clinically and pathologically heterogeneous, and there is a lack of standardization of diagnostic terminology. They can represent a potential serious diagnostic pitfall because the histological features of clinically indolent proliferations may mimic those of high-grade lymphoma. However, correct identification of these entities is essential given that complete remission may occur upon reversal of the underlying cause of immunosuppression without the need for systemic therapy. IA-LPDs presenting in the skin are rare but well documented. One form of iatrogenic IA-LPD, methotrexate-associated lymphoproliferative disorder (MTX-LPD), can present with cutaneous nodules, plaques, or ulcers. Predominantly, MTX-LPD develops in the context of long-term treatment of autoimmune conditions, such as rheumatoid arthritis, dermatomyositis, and Sjögren syndrome, and may be associated with underlying Epstein–Barr virus (EBV) infection. We present 4 cases of cutaneous EBV-positive B-cell MTX-LPD and describe their clinical and morphological findings. Comparison of our histological findings to the diagnostic criteria for EBV-positive mucocutaneous ulcer (EBVMCU) revealed significant overlap, highlighting the intersection between MTX-LPD and EBVMCU. Withdrawal of methotrexate resulted in healing of all lesions at a mean time of 2 months. In summary, close clinicopathological correlation is vital to identify MTX-LPD presenting as cutaneous EBVMCU given that the initial treatment strategy is that of withdrawal of methotrexate without the need for immediate systemic therapy.

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Section: Discussionmentioning

confidence: 99%

Skin-Limited, Methotrexate-Associated Epstein–Barr Virus–Positive Mucocutaneous Ulcer—A Mimicker of High-Grade Lymphoma. A Report of 4 Cases and Review of the Literature

Macklin

Stonard

Matin

et al. 2023

The American Journal of Dermatopathology

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“…EBV-MCU has been also attributed to primary immunodeficiencies and to human immunodeficiency virus (HIV) infections, but there are only few cases reported in the literature so far [17,[27][28][29][30].…”

Section: Introductionmentioning

confidence: 99%

“…Overview of EBV-MCU cases of the head and neck, including source of immunosuppression, localization, treatment and outcome[1,[3][4][5][6][7][8]11,12,14,[16][17][18][19]21,[23][24][25][27][28][29][30]32,34,35,[37][38][39][44][45][46][47][48][49][50][51][52][53][54][55][56].…”

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confidence: 99%

Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region

Bott

Oschlies²,

Radeloff

et al. 2022

Current Oncology

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EBV-positive mucocutaneous ulcer (EBV-MCU) was classified as a rare new entity of the lymphoproliferative B-cell diseases by the WHO in 2017 and must be distinguished from head and neck squamous cell carcinoma by early biopsy. The aim of the study is to raise awareness of the disease and to give a review of the current literature and a recommendation for EBV-MCU management. All EBV-MCU cases of the head and neck region published so far were included. We also report a case of a pharyngeal EBV-MCU in an 89-year-old patient who was immunosuppressed by chronic lymphatic leukaemia/small lymphocytic lymphoma (CLL/SLL). In contrast to all previously described cases, histopathology showed a co-infiltration of EBV-MCU and CLL/SLL. A total of 181 cases were identified on PubMed and summarised. EBV-MCU was predominantly caused by immunosuppressive drug therapy. Complete remission could be achieved in 68% of cases and was mainly attributed to a reduction of the immunosuppressive therapy alone (72%). However, some severe cases require more aggressive treatment. Regarding the various histopathologic similarities to other lymphoproliferative disorders, the diagnosis of EBV-MCU can be misleading, with a great impact on patient care and treatment. This diagnosis must be made with caution and requires a combination of clinical, morphological and immunophenotypic features.

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

Ikeda

Gion

Nishimura

et al. 2021

IJMS

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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.

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EBV-positive mucocutaneous ulcer in a patient with systemic lupus erythematosus

Cited by 3 publications

References 2 publications

Skin-Limited, Methotrexate-Associated Epstein–Barr Virus–Positive Mucocutaneous Ulcer—A Mimicker of High-Grade Lymphoma. A Report of 4 Cases and Review of the Literature

Skin-Limited, Methotrexate-Associated Epstein–Barr Virus–Positive Mucocutaneous Ulcer—A Mimicker of High-Grade Lymphoma. A Report of 4 Cases and Review of the Literature

Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region

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

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