Background: Behçet's disease is a rare systemic vasculitis of unknown etiology. The typical symptoms include recurrent oral and/or genital aphthous lesions, iridocyclitis (historically with hypopyon) and various skin lessions. The number of young adults and children which are diagnosed with Behçet-syndrome is increasing in recent years and the mean age of manifestation has decreased to 25 years in German patients. The disease is generally diagnosed later in German (48.5 months) than in Turkish patients (25.5 months). Ocular manifestation has been confirmed as a marker of severe prognosis. In 15-25% of affected patients it leads to blindness.
Patient: We describe a 19-year-old Caucasian woman who has suffered from the typical symptoms (oral aphthous lesions, recurrent uveitis posterior, various skin lesions) since the 16th year of age.
Results: After three years the patient was finally be diagnosed with Behçet's disease (using the criteria of the "International Study Group for Behçet's Disease"). Visual acuity was stabilized with an immunosuppressive therapy, although there was no complete remission in disease activity. In addition a cerebral vasculitis was manifested.
Conclusions: Ophthalmologists should be familiar with Behçet's disease. In cases of recurrent uveitis Behçet's disease should be included in the differential diagnosis because timely immunosuppressive therapy can prevent irrevocable changes in the corpus vitreum and retina and preserve complete visual acuity.