Esophageal stenosis as an initial presentation of Behçet’s disease: A case report and literature review

Introduction

Behçet’s disease (BD) is a multiorgan inflammatory vascular disease of unknown etiology, affecting vessels of any size and type. While, gastrointestinal involvement in BD is uncommon, esophageal Behçet’s disease (EBD) is rarely reported, especially in the Mediterranean region.

Aim of the work

To report a case with esophageal stenosis as an initial presentation of BD and compare to reported cases worldwide.

Case presentation

A 30-year-old Syrian male presented with dysphagia, odynophagia with weight loss 20 kg. He had a history of recurrent oral ulcers and was otherwise healthy. On examination he appeared pale, dehydrated with normal systemic examination. Blood tests revealed anemia (hemoglobin 10.5 mg/dl), elevated erythrocyte sedimentation rate (ESR) (90 mm/1^sthr) and C-reactive protein (CRP)(98 mg/dl)levels. Upper gastrointestinal endoscopy showed mild ulceration with edema and mucosal thickness at the upper sphincter of the esophagus, leading to stenosis. Biopsy showed nonspecific inflammation, without granulomas or signs of malignancy. Percutaneous endoscopic gastrostomy (PEG) tube was placed and during hospitalization he developed genital ulcers and hemoptysis, with chest computed tomography revealing pulmonary vasculitis. The patient was diagnosed with BD, and treated with methylprednisolone and cyclophosphamide, leading to significant improvement. After two months he could swallow normally, and the PEG tube was removed with no recurrence after one year follow-up. He was maintained on azathioprine 100 g/day and prednisolone 7.5 mg/day.

Conclusion

BD is a diagnostic challenge due to its diverse manifestations and geographic variations. EBD is rare and can cause disability. Early diagnosis of EBD and appropriate treatment are crucial for improving outcomes.