Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report
A 25 year-old man was hospitalized in a public hospital with the following complaints: ascites, prominent venous collaterals on the abdominal wall, swelling in the legs and cutaneous lesions. Two years earlier, he had developed asymmetric recurrent migratory arthritis, aseptic meningitis, moderate and intermittent fever, recurrent lesions in the oral cavity and unilateral blindness. He denied smoking and alcoholism. Physical examination showed jaundice, acneiform eruptions on the abdomen, oral ulcers, reduced vesicular murmur at right lung base, ascites with varicose veins in the abdomen near the skin surface, an enlarged and tender liver and edema of legs (++/4) ( and ). Laboratory tests detected hypochromic and microcytic anemia; nonreactive viral hepatitis serology, HIV and syphilis infection serology; negative autoantibodies; normal rheumatoid factor and serum complement; low levels of protein C and S, and normal levels of antithrombin III; high hemosedimentation velocity and C reactive protein; serum ascites albumin gradient greater than 1.1; and a negative skin pathergy test. Histopathology of papulopustular lesion showed show perifollicular and perivascular mononuclear and neutrophilic infiltration (). Endoscopy showed oesophageal varices. Doppler ultrasound examination and computed tomography showed ascites and absence of flow in the suprahepatic and cava veins ( and ). Doppler ultrasound of lower limbs showed thrombosis. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis of BD in association with BCS. Symptomatic treatment was established with diuretics, oral corticosteroids, azathioprine, colchicine and anticoagulants. After such treatment was begun the patient had significant improvement, but died five months later due to haemorrhage complications.