Hughes stovin syndrome
(John Patterson Hughes, 20th century, British physician and Peter George Ingle Stovin, 20th century, British physician), a variant of Behcets disease. It is even more rare than Behcet's disease, most patients being young adult males in the second to fourth decades. The major features are:
systemic venous thrombi involving the vena cava, cerebral
sinuses and limb veins; pulmonary artery
occlusions due to emboli or thrombi; and
one or more segmental pulmonary artery aneurysms
Common clinical presentations include venous thrombosis, fever and haemoptysis. Radiological features are similar to those of Behcets disease (Fig.1).
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Contrast-enhanced CT scan of the chest in a patient with thrombosis of the inferior vena cava and Bechet's disease showing bilateral aneurysms of the interlobar pulmonary arteries. Mural thrombosis is present in both aneurysms.
Hughes stovin syndrome, Fig.1