Vasculo-Behcet's Disease Developing in a Patient with Ebstein Anomaly / 대한류마티스학회지

ABSTRACT

Behcet's disease (BD) is a chronic inflammatory disease, involving several organs. It is well known that there are the marked regional differences in the disease expression of BD. In case of the vascular involvement in BD, the frequency rate has been described in 25~30% among the patients from the MiddLe East, whereas it has been noted in 5~15% in our country. In general, BD associated with large vessel lesions is named vasculo-BD. On the other hand, the cardiac involvement in BD has rarely been reported in the literature. Ebstein anomaly is a kind of malformation that is characterized by a downward displacement of the tricuspid valve into the right ventricle. We reported a 54-year-old male patient with vasculo-BD who had inferior vena cava obstruction and Ebstein anomaly. The association of Ebstein anomaly with vasculo-BD is considered to be coincidental. To the best of our knowledge, this is the first case of Ebstein anomaly associated with BD.