Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. May 7, 2015; 21(17): 5417-5420
Published online May 7, 2015. doi: 10.3748/wjg.v21.i17.5417
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab
José Miranda-Bautista, Alejandro Fernández-Simón, Isabel Pérez-Sánchez, Luis Menchén
José Miranda-Bautista, Alejandro Fernández-Simón, Luis Menchén, Department of Gastroenterology, “Gregorio Marañón” General Hospital-IiSGM, 28003 Madrid, Spain
Isabel Pérez-Sánchez, Department of Hematology, “Gregorio Marañón” General Hospital-IiSGM, 28003 Madrid, Spain
Luis Menchén, Centro de Investigación en Red de Enfermedades Hepáticas y Digestivas, Instituto de Salud Carlos III, 28003 Madrid, Spain
Luis Menchén, Medicine Department, Universidad Complutense, 28003 Madrid, Spain
Author contributions: Miranda-Bautista J wrote and corrected the manuscript; Fernández-Simón A reviewed patient chart and contributed writing the article; Pérez-Sánchez I contributed with discussion and review of the literature; Menchén L designed, wrote and corrected the manuscript.
Ethics approval: The case report has been submitted to the Ethics Committee of “Gregorio Marañón” General Hospital-IiSGM.
Informed consent: Verbal and written informed consent was performed and signed by investigator and patient before inclusion into the study.
Conflict-of-interest: The authors declare to have no conflict of interest, financial or otherwise, with respect to this work.
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: Luis Menchén, MD, PhD, Department of Gastroenterology, “Gregorio Marañón” General Hospital-IiSGM, IiSGM.C/Dr. Esquerdo 46, 28003 Madrid, Spain. lamenchen.hgugm@salud.madrid.org
Telephone: +34-915-868300 Fax: +34-914-265024
Received: September 12, 2014
Peer-review started: September 12, 2014
First decision: October 14, 2014
Revised: November 12, 2014
Accepted: December 22, 2014
Article in press: December 22, 2014
Published online: May 7, 2015
Processing time: 243 Days and 8.8 Hours
Abstract

Weber-Christian disease (WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation, being the subcutaneous adipose tissue the most frequent one, followed by liver, spleen, bone marrow and mesenteric adipose tissue. Systemic corticosteroids are first line treatment, but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation. We report herein a case with WCD showing orbital, mesenteric and ileocolonic involvement, which required surgical treatment and also developed postoperative recurrence. Symptoms were resolved by administration of thalidomide and, afterwards, infliximab. To our knowledge, this is the first report of Weber-Christian disease with luminal ileocolonic involvement, treated with infliximab.

Keywords: Weber-Christian disease; Idiopathic lobular panniculitis; Crohn’s disease; Infliximab; Ileocecal resection

Core tip: Weber-Christian disease is an autoimmune panniculitis affecting most frequently subcutaneous adipose tissue; liver, spleen, bone marrow and mesenteric adipose tissue might be also involved. We report a case of this uncommon disease with luminal ileocolonic involvement, mimicking Crohn’s Disease, which required hemicolectomy and distal ileum resection. As the intestinal inflammation and symptoms recurred after surgery infliximab was started, leading to an improvement in clinical and radiological signs.