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.
Departments of *Dermatology
∥Hematology, Fundación Jiménez Díaz University Hospital
#Department of Pathology, Fundación Jiménez Díaz University Hospital and CIBERONC, Madrid
Departments of †Pathology
‡Dermatology, Guadalajara University Hospital, Guadalajara
¶Dermatology Hospital, Lozano Blesa Clinical University Hospital, Zaragoza, Spain
Conflicts of Interest and Source of Funding: Supported by grants from the Instituto de Salud Carlos III (ISCIII) of the Spanish Ministry of Economy and Competence (MINECO, RTICC ISCIII, and CIBERONC) (SAF2013-47416-R, RD06/0020/0107-RD012/0036/0060, and Plan Nacional I+D+I: P117/2172, PI16/01294, and PIE15/0081), AECC, and the Madrid Autonomous Community.
Correspondence: Lucía Prieto-Torres, MD, Department of Dermatology, Fundación Jiménez Díaz University Hospital, Madrid 28040, Spain (e-mail: email@example.com).