Introduction: Behcet's Disease (BD) is classified as a vasculitis, and progresses via attacks and remissions. BD is mainly seen around the Silk Road. The picture varies in different reports. For clinical descriptions, the data from the international cohort of patients (27 countries), will be used.
Clinical manifestations: Mucous membrane manifestations were oral aphthosis seen in 98.1%, and genital aphthosis in 76.9% of patients. Skin manifestations were seen in 71.9% (pseudofolliculitis in 53.6% and erythema nodosum in 33.6%). Ocular manifestations were seen in 53.7% (anterior uveitis 38.8%, posterior uveitis 36.9%, retinal vasculitis 23.5%). Joint manifestations were seen in 50.5% (arthralgia, monoarthritis, oligo/polyarthritis, ankylosing spondylitis). Neurological manifestations were seen in 15.5% of patients (central 11.5%, peripheral 4.4%). Gastrointestinal manifestations were seen in 6.3% of patients. Vascular involvement was seen in 18.2% of patients and arterial involvement in 3% (thrombosis, aneurysm, pulse weakness). Deep vein thrombosis was seen in 8%, large vein thrombosis in 6.5%, and superficial phlebitis in 5.8%. Orchitis and epididymitis were seen in 7.2%. Pathergy test was positive in 49.3% and HLA-B51 in 49.1% of patients.
Diagnosis: Diagnosis is based on clinical manifestations. The International Criteria for Behcet's Disease (ICBD) may be helpful.
Treatment: The first line treatment is colchicine (1 mg daily) for mucocutaneous manifestations, non-steroidal anti-inflammatory drugs for joint manifestations, anticoagulation for vascular thrombosis, and cytotoxic drugs for ocular and brain manifestations. If incomplete response or resistance occurs, therapeutic escalation is worthwhile.
Conclusion: Behcet's disease is a systemic disease characterized by mucocutaneous, ocular, vascular and neurologic manifestations, progressing by attacks and remissions.