Behcet's disease (BD), also known as Behcet's syndrome, is a rare, chronic, autoimmune disorder of unknown origin. Its manifestations are thought to be caused by vasculitis, resulting in damage to blood vessels of all sizes throughout the body. We report a 25-year-old Pakistani male who is sexually active and presents with a one-year history of shortness of breath, cough, exertional dyspnea, and neck and facial swelling. On examination, he had severe anemia, mouth ulcers, distended neck and chest veins, prominent abdominal veins, and a scrotal ulcer. After going through mandatory investigations to evaluate the presenting signs and symptoms, thromboses were found in the major veins, including the superior vena cava (SVC) and external iliac vein, as well as a positive pathergy test. Accordingly, a diagnosis of Behcet's disease with cardiomyopathy and venous thrombosis was made. He was treated with anticoagulants, steroids, and azathioprine for six months and subsequently went into remission.
Keywords: autoimmune disease; behcet's disease; behcet's syndrome; cardiovascular disease; mucocutaneous ulcers; pathergy; superior vena cava (svc) syndrome; thromboembolic disease; thrombotic angiopathy.
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