Ebv-positive mucocutaneous ulcer

EBV-positive mucocutaneous ulcer is a new provisional category in the current WHO classification for lymphoid neoplasms. It has been distinguished from EBV+ DLBCL, NOS due to its self-limiting course and good response to conservative measures. This EBV-associated lymphoproliferative disorder occurs preferentially in elderly patients with varying degrees of (e.g., therapeutically induced) or infectious (HIV+) immunodeficiency (Bunn B et al. 2015).

In a series of 15 Japanese patients (11w/4m) with a median age of 74 years (35-84 years) with autoimmune diseases, 8 individuals developed EBVMCU after chemotherapeutic treatment for diffuse large B-cell lymphoma. Ulcers occurred in the oral mucosa (n=11), gastrointestinal tract (N=2) and 2 x on the skin.

A mixed proliferation of EBV-positive large, anaplastic B cells and Hodgkin/Reed-Sternberg-like cells is detectable.

Favourable; apart from the need for treatment of the underlying disease, the ulcers should be conservatively treated locally.

1. Bunn B et al (2015) EBV-positive mucocutaneous ulcers of the oral cavity associated with HIV/AIDS. Oral Surg Oral Med Oral Pathol Oral Radiol 120:725-732.
2. Daroontum T et al (2018) Comparison of Epstein-Barr virus-positive mucocutaneous ulcer associated with treated lymphoma ormethotrexate in Japan. Histopathology doi: 10.1111/his.13464.
3. Dojcinov SD et al (2010) EBV positive mucocutaneous ulcer--a study of 26 cases associated with various sources of immunosuppression. On J Surg Pathol 34:405-417.
4. Lamos C. et al (2017) Aggressive primary cutaneous B-cell lymphomas and new EBV-positive entities. The dermatologist 68: 727-739