The Spectrum of EBV-Positive Mucocutaneous Ulcer

Abstract

We describe a series of 9 patients with Epstein-Barr virus (EBV)-positive mucocutaneous lymphoproliferative lesions that broadens the concept of EBV-positive mucocutaneous ulcer. We report 5 female and 4 male patients, with an average age of 74 years (range, 55 to 87 y), 2 of whom were HIV-positive. The lesions were located in the oropharynx, skin, and rectal and/or genital mucosa. Histopathologically, 6 cases showed a polymorphic pattern and 3 had a monomorphic and diffuse one, with angiotropism in 4 cases (2 each with the polymorphic and monomorphic patterns). Three of the cases expressed PDL1. In addition to its presence in the neoplastic lymphoid cells, EBV was also detected in adjacent epithelial cells in an oropharyngeal lesion. All cases responded to local therapy or adapted systemic chemotherapy in selected cases. This series extends the spectrum of this disorder to include some HIV-positive cases, patients with multiple lesions confined to a single anatomic area, lesions with an angiocentric pattern, and some cases with monomorphous large-cell cytology. We discuss the differential clinicopathologic diagnosis of this disorder and that of classic EBV large B-cell lymphoma.

Epstein-Barr Virus-related Multi-site Mucocutaneous Ulcer: A Previously Undescribed Clinical Subset of a Rare Disease

“…In contrast to a typical histological and immunohistochemical pattern, the clinical profile of our observation is unprecedented to date, with multiple, rarely ulcerated and merely cutaneous and sub-cutaneous nodules either clinically detectable or identified by PET scan only and disseminated on several anatomical areas on the trunk and the limbs with no associated involvement of mucous membranes. A relatively similar pattern with isolated multiple skin violaceous nodules or infiltrated plaques with no mucous lesions was recently reported, located on the same anatomical area as in previous observation (7). The unusual disease dynamics with rapidly enlarging lesions and the lack of iatrogenic immunosuppression amenable to drug reduction or withdrawal led to the use of monochemotherapy by pegylated liposomal doxorubicin with complete initial response and a good tolerance.…”

Hodgkin Reed–Sternberg-Like Cells in Non-Hodgkin Lymphoma

“…The presence of lymphadenopathy and/or spleen, liver or bone marrow involvement should also lead to a presumptive diagnosis of EBV+ MCU being questioned [99]. EBV+ MCU generally follows an indolent course with spontaneous regression or remission upon reduction of immunosuppressive drugs [99,100].…”

“…Unlike EBV+ MCU, cHL is always a tumor-forming lesion. Additionally, the extreme rarity of cHL presenting as extranodal disease in the absence of nodal involvement must be considered [9,100].…”

The Roles of γδ T Cells in Hematopoietic Stem Cell Transplantation

“…EBV can be found in the skin or mucosa and may be associated with mucositis 70 . gd T cells, with distinct subsets, as important innate immune cells for human, present in both blood and tissues and play multiple roles in mucosal inflammation 71 .…”