TY - JOUR
T1 - Epstein–barr virus-positive mucocutaneous ulcer
T2 - A unique and curious disease entity
AU - Ikeda, Tomoka
AU - Gion, Yuka
AU - Nishimura, Yoshito
AU - Nishimura, Midori Filiz
AU - Yoshino, Tadashi
AU - Sato, Yasuharu
N1 - Funding Information:
Funding: This work was partially supported by the Grant-in-Aid for Young Scientists [JSPS KA-KENHI grant number 19K16586] and Scientific Research [C] [JSPS KAKENHI Grant Number JP 20K07407], from the Japan Society for the Promotion of Science.
Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2021/2/1
Y1 - 2021/2/1
N2 - 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-todate clinical, pathological, and genetic aspects.
AB - 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-todate clinical, pathological, and genetic aspects.
KW - Clinical features
KW - EBV-positive mucocutaneous ulcer
KW - Immunosuppression
KW - Pathological features
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U2 - 10.3390/ijms22031053
DO - 10.3390/ijms22031053
M3 - Article
C2 - 33494358
AN - SCOPUS:85099598994
SN - 1661-6596
VL - 22
SP - 1
EP - 14
JO - International journal of molecular sciences
JF - International journal of molecular sciences
IS - 3
M1 - 1053
ER -