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

Extraordinary Case: Unique Presentation of an Aggressive Epstein-Barr Virus–Positive Mucocutaneous Ulcer

“…The EBV-associated lymphoproliferative disorders (LPDs) are a spectrum of diseases that range from self-limiting localized conditions to aggressive lymphomas. 1,2,4,5 EBV-mucocutaneous ulceration is regarded as a recent entity, included in the WHO database in 2017 1,5,6 and in the WHO-EORTC classification of cutaneous lymphomas as a provisional entity in 2018. 7 It was first described in 2010 by Dojcinov et al 8 in either age-related or iatrogenic immunosuppressed patients, presenting mostly as a solitary demarcated ulcerated lesion involving the skin, oropharyngeal mucosa, or gastrointestinal tract; it manifests when the virus/host homeostasis fail and the infection overcomes immunovigilance.…”

“…They usually regress spontaneously, but an increasing number of reports describe a spectral behavior of the lesion in different mucosal sites, which ranges from a simple ulcer with eosinophilia, to aggressive ulcers with a more complicated outcome. 2,3 In these cases, EBV-related lymphomas are usually considered as differential diagnosis. We report a unique observation of this rare disease with atypical bone involvement and discuss treatment approaches.…”