EBV-positive mucocutaneous ulcer affecting the bowel

EBV-positive mucocutaneous ulcer affecting the bowel

Daniela Kemper, Christine Beham - Schmid, Marion Pollheimer, Cord Langner


Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) was included in the latest edition of the WHO classification of tumors of hematopoietic and lymphoid tissues as a distinct clinicopathological entity. The disease is characterized by well demarcated indurated ulcers occurring in cutaneous and mucosal sites including the gastrointestinal tract. Herein, we report two new cases of this peculiar disease. The first case presents as large bowel obstruction mimicking malignancy, the second as acute abdomen due to small bowel perforation. Histology discloses characteristic B cell blasts with Hodgkin/Reed-Sternberg cell like-features, which are positive for EBV on EBER in situ-hybridization. Clinical implications and differential diagnosis, including distinction from classical Hodgkin lymphoma and lymphomatoid granulomatosis are discussed.


Epstein-Barr virus; EBV-positive mucocutaneous ulcer; immunosuppression; Hodgkin lymphoma; lymphomatoid granulomatosis.

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