Published online May 7, 2015. doi: 10.3748/wjg.v21.i17.5417
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
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.
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.