Behcet's disease (BD) is a rare multisystem chronic vasculitis of variable clinical presentation and unknown origin. Pulmonary involvement in BD is uncommon, with pleural effusion being an even rarer and difficult to diagnose manifestation. Herein, we report a challenging case of a young man who presented with recurrent pleural effusion and hepatic vein thrombosis and a recent history of papilledema with idiopathic intracranial hypertension. The patient was hospitalized for diagnostic and therapeutic thoracocentesis. Biochemistry and cytological analysis showed an exudative aspect of the collected pleural fluid with lymphocyte-dominated cytology. A multidisciplinary discussion was held, and thorough investigation was carried out to rule out malignant and infectious etiologies, among other differentials. During the second hospitalization, the patient complained of genital ulcers, which were verified to be recurrent along with oral ulcers. The diagnosis of BD was established based on the International Classification Criteria for BD, and the symptoms improved significantly upon using colchicine and immune-suppressive drugs.
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