br Acknowledgments br Introduction Beh

Acknowledgments
Introduction Behçet\'s disease (BD) is an inflammatory disease characterized by recurrent oral aphthous ulcers and numerous potential systemic manifestations such as genital ulcers, dextromethorphan hydrobromide lesions, and ocular, neurologic, vascular, articular, and gastrointestinal diseases. The most commonly involved site in the gastrointestinal tract is the ileocecal area. Hemorrhage, fistula formation, penetration, and perforation occur in up to 50% of patients with intestinal BD. In most reports, these complications are indications for surgery. There are, however, no definitive laboratory tests for BD; as a result, the diagnosis is made on the basis of clinical findings. Because it may take several months or even years for all of the common symptoms to appear, the diagnosis may not be made for a long time and often retrospectively.
Case Report Abdominal computed tomography (CT) was performed to rule out intra-abdominal abscess formation (Fig. 1A and B). He was then admitted under the impression of wound infection and inflammatory process of the ileocecal region. The wound was treated with a wet dressing, and the patient was discharged 5 days later under stable condition. During an Outpatient Department follow-up 1 month later, pus was found to be coming from the operative wound, and an abdominal CT revealed an abscess with suspicion of fistula formation (Fig. 1C). Therefore, he was treated for a localized appendicocutaneous fistula at the outpatient clinic for the next 2 months. The patient was then admitted again because of poor digestion. An abdominal CT scan prior to admission revealed focal wall thickening of the cecum, without fat stranding of the surrounding tissue, but no intra-abdominal free air or fluid was noted (Fig. 1D). During admission, lower gastrointestinal bleeding with hemorrhagic shock occurred suddenly, and an emergency laparotomy was performed following resuscitation. A right hemicolectomy was performed immediately. A huge ulcer was located on the antimesenteric side of the cecum, with nodular margins and converging folds, which measured 7 cm × 4 cm in size and 1.5 cm in depth. Several discrete perforations with sharp margins were present at the terminal ileum (Fig. 2). Another deep ulcer with a volcano-like shape was noted 32 cm proximal to the ileocecal valve, and it was also resected. The pathology report showed nonspecific inflammatory cell infiltration and mucosal inflammation without granuloma formation or venulitis (Fig. 3). We collected samples for laboratory testing following the operation, which included human leukocyte antigen-B51 (HLA-B51), human leukocyte antigen-B27 (HLA-B27), anti-hepatitis C virus (anti-HCV), Extractable nuclear antigen (anti-ENA) (SSA/SSB/Jo-1 Ab), anti-human immunodeficiency virus (anti-HIV), anti-dsDNA, antinuclear antibody (ANA), Anti-neutrophil cytoplasmic antibody (ANCA), QuantiFERON®-TB (QFT) test for tuberculosis, HCV immunoglobulin G (IgG), Epstein-Barr virus (EBV) IgG and EBV IgM; they were all negative. Blood cultures were performed seven times during the course of treatment, and the reports were again all negative. The pus culture which was obtained from the subcutaneous wound revealed Escherichia coli and Enterococcus faecalis.
Discussion According to the International Study Group criteria (ISG, 1990), for a patient to be diagnosed with BD, the patient must have aphthous ulcers along with two of the following four “hallmark” symptoms: (1) genital ulcers; (2) skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids); (3) eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous); and (4) pathergic reaction (papule > 2 mm diameter, 24–48 hours or more after needle-prick). According to the International Criteria for Behçet\'s disease (ICBD, 2006), a total score from ocular lesions (2 points), genital aphthosis (2 points), oral aphthosis (2 points), skin lesions (1 point), neurological manifestations (1 point), vascular manifestations (1 point), and the positive pathergy test (1 point) is used for the diagnosis of BD.