Miscellaneous manifestations of behçet syndrome

Abstract

Behçet syndrome (BS) may rarely present with rapidly progressive hearing loss, epididymitis, or orchitis. Fever can occur in BS with neurologic, vascular, or arthritic involvement, but is not a typical finding in their absence. Data for a direct association of BS with malignancy remain difficult to interpret, but several, especially hematologic malignancies and hematinic deficiencies can mimic BS. There is general consensus that manifestations such as hearing loss and epididymitis are part of the BS disease spectrum, but there remains controversy regarding others, such as renal disease. Within those areas of controversy, it is debatable whether a proposed manifestation represents a true association, is part of the BS disease spectrum, or is an independent primary disease mimicking BS. Thorough familiarity with the clinical phenotypes of BS, including its rarer manifestations, is a prerequisite for the successful evaluation of patients with suspected BS, especially if their presentation includes manifestations that lie at the far ends or outside of the recognized Behçet disease spectrum.