The incidence of EBV positive mucocutaneous ulcer is likely highly underestimated, given its self-limiting course, in addition to its rarity amongst the spectrum of EBV positive lymphoproliferative disorders with only a limited number of reported cases in the literature. It was first identified in 2010 and is associated with immunosuppression, iatrogenic, primary and age related. A slight female predominance has been reported with a median patient age of 66.4 years. Disruptions in the balance of this interaction are believed to result in the lymph proliferation of various cell derivatives. EBV positive Mucocutaneous Ulcer (EBVMCU) is an indolent condition on this spectrum of LPDs, which localizes to the skin and mucosal surfaces. It is a rare lymph proliferation that gained recognition as a new entity in the 2016 revisions to the World Health Organization classifications.
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