An Unusual Faint Halo Around Colonic Ulcers

Question: A 47-year-old woman presented with a 1-month history of abdominal pain, fever for 10 days, and tarry stool. She reported recurrent mouth and genital ulcers since childhood. The patient had no family history of mouth and genital ulcers, and never consulted a doctor. On examination, she had tenderness in the right iliac fossa with no mass. Blood examinations revealed elevated C-reactive protein of 225 mg/L (normal range, 0–12 mg/L) and decreased hemoglobin of 111 g/L (normal range, 130–175 g/L). She had a low serum albumin level of 31 g/L (normal range, 40–55 g/L). Interferon gamma release assay for Mycobacterium tuberculosis was negative. Fecal calprotectin was elevated at 360 μg/g (normal range, 0–50 μg/g). Abdominal computed tomography showed bowel wall thickening in the cecum and ascending colon (Figure A). Esophagogastroduodenoscopy and chest computed tomography were normal. Colonoscopy revealed massive ulcers of different sizes throughout the colon (Figures B–G). The ileocecal valve was pulled open, and a large and deep terminal ileum ulcer was shown (Figure H). Linked color imaging views represented circumferential circular and deep colonic ulcers with heaped up edges, with a faint halo around them (Figures I–K). Colonic and ileocecal biopsy specimens (H&E staining) revealed the findings shown in Figures L–N. What is the most likely diagnosis? Look on page 352 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. The differential diagnosis includes intestinal tuberculosis, inflammatory bowel disease, and gastrointestinal lymphoma. Granulomas was not found. Tuberculosis culture and polymerase chain reaction were negative. We did not find any histologic evidence of Crohn’s disease and gastrointestinal lymphoma. Histologic evaluation of biopsy specimens showed ulcerated mucosa with deeply inflamed granulation tissue containing thrombosed venules (Figure O, asterisk), which was consistent with intestinal Behçet’s disease. Screening was positive for the following autoantibodies: anti-nuclear antibody, anti-SSA/Ro52, and anti-SSA/Ro60. Behçet’s disease comprises deposition of immune complexes in veins and arteries of all sizes, characterized by recurrent painful oral and genital ulcers, which is a multisystemic immune-mediated inflammatory disorder.1Yen H.H. Soon M.S. Electronic image of the month. Fever and upper gastrointestinal bleeding: Behçet's disease.Clin Gastroenterol Hepatol. 2010; 8: e35-e36Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Genetic predisposition, environmental factors, and immunologic abnormalities have been implicated in the etiopathogenesis of the disease.2Nieto I.G. Alabau J.L.C. Immunopathogenesis of Behçet disease.Curr Rheumatol Rev. 2020; 16: 12-20Crossref Scopus (8) Google Scholar Behçet’s disease may affect the gastrointestinal, neurologic, and musculoskeletal systems.3Davatchi F. Behçet's disease.Int J Rheum Dis. 2018; 21: 2057-2058Crossref Scopus (12) Google Scholar Gastrointestinal Behçet’s disease may involve all areas from the lips to the anus, which is less frequently in the colon.4Suzuki Kurokawa M. Suzuki N. Behcet's disease.Clin Exp Med. 2004; 4: 10-20Crossref PubMed Scopus (223) Google Scholar Symptoms of intestinal Behçet’s disease may include hematochezia, abdominal pain, and weight loss, which share several overlapping intestinal and extraintestinal manifestations with Crohn’s disease.5Skef W. Hamilton M.J. Arayssi T. Gastrointestinal Behçet's disease: a review.World J Gastroenterol. 2015; 21: 3801-3812Crossref PubMed Scopus (125) Google Scholar Oral ulcers generally precede the onset of gastrointestinal ulcers, which are larger in size than Crohn’s disease and are typically rounded rather than longitudinal, occurring usually in the ileocecal region.6Davatchi F. Chams-Davatchi C. Shams H. et al.Behcet's disease: epidemiology, clinical manifestations, and diagnosis.Expert Rev Clin Immunol. 2017; 13: 57-65Crossref PubMed Scopus (191) Google Scholar Genital ulcers commonly occur on the vulva in women and on the scrotum and penis in men.7Marshall S.E. Behçet's disease.Best Pract Res Clin Rheumatol. 2004; 18: 291-311Crossref PubMed Scopus (153) Google Scholar No clinical guideline for intestinal Behçet’s disease is well established based on the lack of rigorous clinical trial data. Establishing the diagnosis remains a great challenge with no universally accepted criteria. Gastroenterologists should be aware of the relevant clinical features and diagnostic considerations. Therapeutic options may comprise steroids, immunomodulators, and biologic therapies.8Hisamatsu T. Naganuma M. Matsuoka K. Kanai T. Diagnosis and management of intestinal Behçet's disease.Clin J Gastroenterol. 2014; 7: 205-212Crossref PubMed Scopus (39) Google Scholar Surgical resection of the involved segment is suggested for patients with bowel perforation and persistent bleeding or for those who are resistant to medical therapy.9Cheon J.H. Kim W.H. An update on the diagnosis, treatment, and prognosis of intestinal Behçet's disease.Curr Opin Rheumatol. 2015; 27: 24-31Crossref PubMed Scopus (59) Google Scholar She was treated with prednisolone at a dose of 0.5 mg/kg body weight per day for 4 weeks, tapered over a period of 3 months to 5.0 mg/d. Besides prednisone, use of conventional immunosuppressants was recommended. Sulfasalazine 1000 mg/d and thalidomide 25 mg/d were commenced at the same time. Her abdominal pain and mouth and genital ulcers gradually improved within 2 months. A repeat lower gastrointestinal endoscopy confirmed endoscopic ulcer healing (Figures P and Q). Fecal calprotectin (30 μg/g) and C-reactive protein (10 mg/L) levels returned to normal. Subsequent therapeutic schedule depended on outpatient follow-up assessment using blood test and endoscopy. We plan to repeat endoscopic surveillance in 6 months for the patient.