EBV-positive Mucocutaneous Ulcer With Small Lymphocytic Infiltration Mimicking Nonspecific Ulceration

Abstract

Epstein-Barr virus (EBV)-associated lymphoproliferative disorder may resemble nonspecific inflammation. We report 3 cases of immunosuppressed adult patients with small lymphocytic EBV ulcers in the skin and oral mucosa, characterized by a lack of atypical lymphocytic infiltration. All 3 cases were diagnosed in routine practice. For comparisons, cases of conventional Epstein-Barr virus–positive mucocutaneous ulcer (EBVMCU) were reviewed which were extracted from our pathology archives (n=11). The present patients were 2 females and 1 male, aged above 70 years. The primary disease was rheumatoid arthritis (n=2) and dermatitis herpetiformis (n=1). The main source of immunosuppression was prednisolone (n=2) and methotrexate (n=1). The ulcers were located in the oral cavity, buttock, and/or external genitalia. Histology evaluation revealed nonspecific lymphocytic infiltration. Epstein-Barr virus–encoded small RNA (EBER)-positive cells were small and coexpressed CD20. The number of EBER-positive cells ranged from 52 to 132/HPF, which was within the range of that observed in the reviewed conventional EBVMCUs (range, 48 to 1328; median, 121). All 3 cases regressed spontaneously or by the reduction of immunosuppressants. Although the present cases lacked cytologic atypia, those clinical course and loads of EBER-positive cells (>50/HPF) suggested EBV involvement. Current cases of EBVMCU with small lymphocytic infiltration underscore the need for EBER in situ hybridization when an etiology of ulcer with predominant lymphocytes in an immunosuppressed patient is unclear.

Epstein-Barr virus-positive mucocutaneous ulcer, plasmablastic type, associated with nodal CD4+ angioimmunoblastic T-cell lymphoma and generalised pruritus: a self-limiting lymphoproliferative disorder resembling cutaneous plasmablastic lymphoma

“…As was so in the current case, EBV-MCU with plasmacytic/plasmablastic differentiation microscopically mimics plasmablastic lymphoma 19 21. It has been emphasised that the histopathological distinction between EBV-MCU and aggressive EBV-positive cutaneous lymphoma may be impossible without careful clinical evaluations 22 23…”

“…EBV-MCU, a self-limiting LPD involving both the mucosa and skin, is observed in patients with iatrogenic immunosuppression after administration of steroid or methotrexate or in the elderly people 18–20. The prototypic histopathology of EBV-MCU represents benign-looking polymorphous type18–20 or small lymphocytic infiltration-mimicking non-specific ulceration 22. Based on its wide histological variations; however, differential diagnosis of EBV-MCU includes such malignant cutaneous lymphomas as DLBCL, classic Hodgkin’s lymphoma and mucosa-associated lymphoid tissue lymphoma 18 19.…”

“…There are significant histological variations in EBV-MCU, including prototypic polymorphous type, large cell-rich type resembling DLBCL, classic Hodgkin’s lymphoma-like type and mucosa-associated lymphoid tissue lymphoma-like type 18 19. EBV-MCU with plasmacytic/plasmablastic differentiation19 21 mimicking plasmablastic lymphoma, as well as EBV-MCU with small lymphocytic infiltration mimicking non-specific ulceration,22 have also been described. The distinction between aggressive EBV-positive cutaneous lymphoma and EBV-MUC is often difficult by histopathologic evaluation alone 22 23.…”

“…EBV-MCU with plasmacytic/plasmablastic differentiation19 21 mimicking plasmablastic lymphoma, as well as EBV-MCU with small lymphocytic infiltration mimicking non-specific ulceration,22 have also been described. The distinction between aggressive EBV-positive cutaneous lymphoma and EBV-MUC is often difficult by histopathologic evaluation alone 22 23. It is noteworthy that plasmablastic lymphoma in non-AIDS patients showed a good prognosis 24–28.…”

Epstein-Barr Virus-positive Mucocutanous Ulcer of the Uterine Cervix: Report of a Rare and Evolving Entity

“…Commonly involved sites included the oropharyngeal tract, skin and gastrointestinal tract. Since then, emerging publications have expanded our understanding of the clinical, morphologic and molecular spectrum of this entity (2)(3)(4)(5)(6). It now includes human immunodeficiency virus (HIV) infection as etiology, multiple lesions within an anatomic region, and a widened spectrum of morphologic patterns.…”