Superior Vena Cava Syndrome in Behçet's Disease

Abstract

Behget's disease was first defined by a Turkish dermatologist of the same name, as a trisymptom syndrome which manifested itself with aphthae in the mouth, genital exul cerations and iridocyclitis with hypopyon. ¹ Later, some other features have been attributed to this syndrome, namely skin changes, joint involvement, obstruction of arteries or veins, and infection of sterile injection sites.^2-16 Some other rare findings have been also noted in Behqet's disease. Aphthae, genital exulcerations, eye lesions and skin changes are the major lesions. If a patient has three of the major lesions or two major + two minor lesions; the disease is considered to be complete in his case. According to the information we now have, the basic pathological disorder in Behget's disease is vasculitis. Thrombosis in the great veins and arteries, and aneurysms, are frequently noted. The majority of the cases, when they first come to the doctor, suffer from superior vena cava obstruction.

Superior vena cava obstruction is usually diagnosed easily. This causes the appear ance of many collateral veins over the anterior chest wall, the dilation of neck veins, and the growth of edema of the chest, neck and face. In the majority of superior vena cava syndromes noted in the literature, malignancy is pinpointed as the cause, while, occa sionally, benign lesions are also winnowed out to be the culprit.^27,31

The aim of this work was to present the review of the last fourteen years' etiology of the superior vena cava syndromes in our hospital, and to attempt to find out how many of them were caused by Behçet's disease. As a result, it was found that Behget's disease is the second cause of superior vena cava syndrome, after malignancy. It is known that Behçet's disease is most frequently diagnosed in Turkey, Japan and Greece, so perhaps this result is not surprising for Turkey. ^7,26 What's more, it is also probable that Behçet's disease might be the underlying cause in most of the cases dubbed "due to unknown reasons." To ascertain this properly, a careful case history and complete physical examination are compulsory.