P-026 Startling Case of Behcet Syndrome with Atypical Presentation and Initial EGD and Microscopic Features Overlapping with Crohn's Disease

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A 9 year-old female presented with 1-month history of epigastric abdominal pain exacerbated by eating, nausea, non-bilious vomiting with occasional hematemesis and failure to thrive. Physical examination revealed malnourishment, BMI 16, abdominal examination revealed soft, and non-tender abdomen without masses. Laboratory analysis revealed WBCs 9.5 bil/l, hemoglobin 10.4 g/dL and platelets 595 bil/l. Serum parameters of liver and kidney function were normal, electrolytes were within normal level, C-reactive protein (CRP) 4.8 mg/dL and iron profile showed evidence of iron deficiency anemia.


Esophagogastroduodenoscopy (EGD) revealed normal esophagus, stomach and duodenum. Colonoscopy revealed patchy, congested, inflamed, ulcerated, and decreased vascular pattern mucosa in the terminal ileum, cecum and ascending colon with normal remaining colon and rectum. Biopsy results showed marked chronic active ileitis with ulceration and patchy chronic severe active colitis with foci of neutrophilic crypt abscesses. Magnetic resonance enterography showed bowel wall thickening and engorgement of the vasa recta at terminal ileum. Infliximab was given for treatment of Crohn's disease and the patient symptoms improved.


After 7 months, the patient presented to the emergency department with fever 107, sore throat, innumerable small aphthous ulcers in palate, gums and buccal mucosa with abdominal pain and decrease oral intake, which was thought to be due to Crohn's flare up. Palatal ulcers were swabbed with negative viral cultures and PCR for herpes simplex, entero-viruses, varicella zoster, and cytomegalo-virues ruling out herpangina, hand-foot-mouth disease and other viral infection and histoplasma urine antigen was negative. Autoimmune workup including; anti-neutrophil cytoplasmic antibodies and anti-double stranded antibodies were negative and total complement, C3 and C4 were normal. Despite Infliximab infusion, the patient had fever spikes with persistence of oral ulcers. During the hospital course, the patient was found to have parotitis, ulcerative esophagitis, hepatitis and pancreatitis that were attributed to an epiphenomenon. Steroids and broad-spectrum antibiotics were given empirically but they were stopped, as there was no clinical improvement. Repeat EGD showed healed esophageal ulcers with abnormal mucosa and repeat colonoscopy showed severely inflamed, congested, and ulcerated mucosa in the terminal ileum. The patient underwent laparoscopic ileo-cecectomy given the severity of the ulcers with high risk of perforation, and the fact that terminal ileum resection might help to control the disease. Unilateral necrotizing pneumonia was found on chest x-ray, started treatment with carbapenem, linezolid and fluconazole that showed no improvement. After initiation of high dose steroid, her fever subsided, symptoms improved and pneumonia resolved. Microscopic pathology revealed large foci of deep ulceration involving the terminal ileum and cecum with features suggestive of Behcet's disease with focal perforation of the ileum. Patient is doing well for 2 years, without steroids or immunosuppressive medication, which is rare with Behcet syndrome with normalization of CRP and hemoglobin.


Case report.




Behcet syndrome can overlap with Crohn's disease and sometimes it is difficult to differentiate them. Resolution of necrotizing pneumonia with steroids is a clue that the underlying pathology is Behcet syndrome. Steroids are the main line of treatment for behcet syndrome flares.

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