Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 21, 2015; 21(19): 6072-6076
Published online May 21, 2015. doi: 10.3748/wjg.v21.i19.6072
Epstein Barr virus-positive mucocutaneous ulcer of the colon associated Hodgkin lymphoma in Crohn’s disease
Neil R Moran, Bradley Webster, Kenneth M Lee, Judith Trotman, Yiu-Lam Kwan, John Napoli, Rupert W Leong
Neil R Moran, Rupert gW Leong, Gastroenterology and Liver Services, Concord Repatriation General Hospital, Sydney, NSW 2137, Australia
Bradley Webster, Kenneth M Lee, Department of Pathology, Concord Repatriation General Hospital, Sydney, NSW 2137, Australia
Judith Trotman, Yiu-Lam Kwan, Department of Haematology, Concord Repatriation General Hospital, Sydney, NSW 2137, Australia
John Napoli, Pennant Hills Endoscopy, Pennant Hills, Sydney, NSW 2120, Australia
Author contributions: Moran NR contributed to data acquisition, analysis and interpretation of data, drafted the manuscript; Webster B drafted the manuscript; Lee KM analyzed and interpretation of data, drafted the manuscript; Leong RW contributed to study concept and design; Webster B, Lee KM, Kwan YL, Trotman J, Napoli J, Leong RW contributed to critical revision of the manuscript for important intellectual content.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Neil R Moran, MB, BCh, BAO, Gastroenterology and Liver Services, Concord Repatriation General Hospital, Hospital Road, Concord, Sydney, NSW 2137, Australia. neilmoran@gmail.com
Telephone: +61-2-97676111 Fax: +61-2-97676767
Received: September 19, 2014
Peer-review started: September 21, 2014
First decision: November 14, 2014
Revised: December 11, 2014
Accepted: January 8, 2015
Article in press: January 8, 2015
Published online: May 21, 2015
Processing time: 243 Days and 7 Hours
Abstract

Epstein Barr virus (EBV) positive mucocutaneous ulcers (EBVMCU) form part of a spectrum of EBV-associated lymphoproliferative disease. They have been reported in the setting of immunosenescence and iatrogenic immunosuppression, affecting the oropharyngeal mucosa, skin and gastrointestinal tract (GIT). Case reports and series to date suggest a benign natural history responding to conservative management, particularly in the GIT. We report an unusual case of EBVMCU in the colon, arising in the setting of immunosuppression in the treatment of Crohn’s disease, with progression to Hodgkin lymphoma 18 mo after cessation of infliximab. The patient presented with multiple areas of segmental colonic ulceration, histologically showing a polymorphous infiltrate with EBV positive Reed-Sternberg-like cells. A diagnosis of EBVMCU was made. The ulcers failed to regress upon cessation of infliximab and methotrexate for 18 mo. Following commencement of prednisolone for her Crohn’s disease, the patient developed widespread Hodgkin lymphoma which ultimately presented as a life-threatening lower GIT bleed requiring emergency colectomy. This is the first report of progression of EBVMCU to Hodgkin lymphoma, in the setting of ongoing iatrogenic immunosuppression and inflammatory bowel disease.

Keywords: Epstein Barr virus; Mucocutaneous ulcer; Hodgkin lymphoma; Inflammatory bowel disease; Crohn’s disease

Core tip: This is the first reported case of Epstein Bar virus mucocutaneous ulcer (EBVMCU) affecting the gastrointestinal tract progressing to widespread Hodgkin lymphoma in the context of iatrogenic immunosuppression in the treatment of Crohns disease. EBVMCU is a newly recognised clinico-pathological condition that was previously thought to have a benign natural history. This case highlights the malignant potential of this disease entity even after withdrawal of immunosuppression.