EBV-positive mucocutaneous ulcers: a presentation of two cases and a brief literature review

Surgical and Experimental Pathology

Tullia Cuzzi¹  José Carlos Morais²  Danielle Carvalho Quintella²  Cristiane Bedran Milito²  Denize D’Azambuja Ramos²  Mário Romañach³  Karolinne Correia Wanderlei⁴ 

[1] 0000 0001 0723 0931, grid.418068.3, Serviço de Anatomia Patológica, Instituto Nacional de Infectologia, FIOCRUZ, Rio de Janeiro, Brazil;0000 0001 2294 473X, grid.8536.8, Departamento de Patologia, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil;0000 0001 2294 473X, grid.8536.8, Patologia Oral da Faculdade de Odontologia da, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil;0000 0001 2294 473X, grid.8536.8, Serviço de Anatomia Patológica, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil;

关键词: Epstein Barr Virus;    EBV positive mucocutaneous ulcer;    Immunosuppression;    Lymphoproliferative;   

DOI  :  10.1186/s42047-019-0037-8

来源: publisher

【 摘 要 】

Mucocutaneous ulcers associated with the Epstein Barr virus constitute an EBV-induced B-cell lymphoproliferative disorder first described in 2010 by Stefan D. Dojcinov et al. These lesions can occur in association with a spectrum of immunosuppressive conditions, including primary immune deficiency, Human Immunodeficiency Virus (HIV) infection, post-transplantation and the use of methotrexate or tumor necrosis factor-alpha (TNF-a) antagonists. Patients clinically present with slowly developing indurated cutaneous and/or mucosal ulcers, especially in the oropharynx. Histopathology reveals circumscribed ulcers containing a mixture of lymphocytes, plasma cells, histiocytes, eosinophils and large transformed cells resembling Hodgkin and Reed-Sternberg cells. The adjacent squamous epithelium presents reactive nuclear atypia and pseudoepitheliomatous hyperplasia. The large transformed cells show positivity for CD20, CD30, Oct-2, PAX5 and EBV. These cells are also positive for MUM1, yet lack CD10 expression, with absent or focal positivity for BCL6. Despite the presence of highly atypical cells, the clinical course is indolent, without progression to disseminated disease. We report herein two cases of diagnosed EBV-positive mucocutaneous ulcers to add to the relatively few cases previously described in the literature.

【 授权许可】

CC BY