Abstract
Hepatic vein thrombosis is a rare occurrence in the clinical course of eosinophilic granulomatosis with polyangiitis (EGPA). The major mechanism of thrombosis has been postulated to involve the release of toxic proteins from eosinophils. A 36-year-old man with EGPA was admitted to our hospital in July 2018 with hematemesis and melena. Findings on physical examination included ascites and pigmentation of the lower extremities. Ultrasonography of the hepatic vein and inferior vena cava showed an obstruction of the hepatic vein. Magnetic resonance imaging showed low enhancement in the right hepatic vein region. At 34 years of age, the patient's EGPA had initially presented as asthma with eosinophilia (white blood cell count of 11.46 × 10(12)/L with 14.6% eosinophils). His skin biopsy showed infiltration of inflammatory cells and eosinophils, especially around medium-sized vessels, which was consistent with EGPA. The patient was thus diagnosed with Budd-Chiari syndrome associated with EGPA.