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A Challenging Case of Oral Ulcers and Gastrointestinal Bleeding: Crohn's or Behçet's Disease
Marina A S Dantas1, Ana Luiza Graneiro2, Rodrigo Cavalcante3
1Department of Pediatric Hospital Medicine, Medical University of South Carolina, Charleston, SC, USA.
Insights
Differentiating gastrointestinal Crohn's disease (CD) and Behçet's disease (BD) is challenging. This case highlights a rare presentation of Behçet's disease mimicking CD, ultimately diagnosed after neurological complications.
Area of Science:
- Gastroenterology
- Rheumatology
- Immunology
Background:
- Differentiating gastrointestinal Crohn's disease (CD) and Behçet's disease (BD) presents diagnostic challenges due to overlapping symptoms.
- Pediatric cases with initial non-specific systemic and gastrointestinal (GI) symptoms require careful evaluation.
Abstract:
Introduction. Differentiating Crohn's disease (CD) and Behçet's disease (BD) with gastrointestinal (GI) manifestations can be clinically challenging, as current diagnostic criteria are not clear between both conditions and multiple symptoms could overlap. Case Presentation. The patient is an 8-year-old boy of Brazilian descent, who initially presented with a 1-year history of painful oral ulcers. Before presenting to the hospital, he had been treated for periodic fever, aphthous stomatitis, pharyngitis, and adenitis and placed on steroids, with relapsing symptoms on attempts to wean the doses. The initial workup was largely unremarkable. Buccal biopsies showed no granulomas, and the ophthalmologic exam was normal. Infectious and rheumatological tests were negative. Prometheus IBD sgi testing showed a pattern consistent with CD; however, the patient had multiple negative endoscopies, colonoscopies, and capsule endoscopies. He developed intermittent bloody stools and severe malnutrition and did not respond to infliximab, colchicine, or methotrexate. After a large GI bleed, a 4th colonoscopy was performed, which showed large round ulcers in the terminal ileum, and no granulomas. He was started on ustekinumab with clinical improvement. One month later, he developed bilateral hip effusion and meningismus, being diagnosed with aseptic meningitis secondary to COVID-19. He improved, but in one month developed worsening symptoms, and MRV showed extensive venous sinus thrombosis. The patient was started on enoxaparin, methylprednisolone, and colchicine, with resolution of the thrombus on a 3-month follow-up. The patient's overall symptoms remained controlled with clinical and biochemical remission on monthly ustekinumab. Discussion and Conclusion. Our patient had a challenging clinical course, with nonspecific systemic and intestinal manifestations which proved difficult to differentiate between BD and CD. Given endoscopic findings and the worsening of an auto-inflammatory reaction in the central nervous system after COVID-19 in a patient with controlled GI symptoms, the most likely diagnosis is BD.
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