About Behçet Disease
Behçet Disease is a chronic, recurrent systemic vasculitis affecting small, medium, and large vessels of multiple organ systems. The pathophysiology involves abnormal T cell activation, increased Th1/Th17 responses, and immune complex deposition leading to vessel inflammation. The disease is characterized by recurrent oral and genital ulceration, which occurs in nearly all patients.
Recurrent painful oral ulcers are the hallmark feature, typically appearing as well-demarcated shallow ulcers with erythematous borders. Genital ulcers occur in 75% of patients and can be more painful and scarring than oral ulcers. Ocular involvement (uveitis) occurs in 50-90% of patients, ranging from mild anterior uveitis to severe posterior uveitis with retinal vasculitis, optic neuritis, and vision-threatening complications.
Skin manifestations include erythema nodosum, pseudofolliculitis, papulopustules, and acne-like lesions. Vascular involvement includes superficial and deep venous thrombosis (thrombophlebitis), arterial aneurysms, and large vessel vasculitis. Neurologic manifestations (neuro-Behçet) occur in 5-30% and include meningoencephalitis, optic neuritis, and myelitis. Gastrointestinal involvement with ulceration occurs in 5-10%.
Common Symptoms of Behçet Disease
Recognizing the signs of Behçet Disease early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.
- Recurrent painful oral ulcers (aphthous stomatitis)
- Recurrent genital ulcers
- Ocular involvement with eye pain, redness, and vision loss (uveitis)
- Skin lesions including erythema nodosum and pseudofolliculitis
- Vascular complications including thrombosis
- Neurologic symptoms from central nervous system involvement
Who Behçet Disease Affects
Behçet Disease typically manifests in the second and third decades of life, though it can present at any age. It affects males more severely than females in some populations, though prevalence is roughly equal.
The disease predominantly affects people from Turkey, Iran, Japan, Korea, China, Middle East, and Mediterranean regions. It is rare in Northern European and North American populations. HLA-B51 positive patients tend to have more severe disease.
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FDA-Approved Treatments for Behçet Disease
There are currently 2 FDA-approved medications for Behçet Disease. These therapies represent the current standard of care and may be used alongside or compared against investigational treatments in active clinical trials.
Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.
Help Paying for Behçet Disease Treatment
Charity funds and drugmaker programs for Behçet Disease, checked at the source. Pick your insurance to see what fits.
Side Effect Explorer
Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.
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Genetic Testing
Genetic testing can confirm a diagnosis, guide treatment decisions, and identify family members who may be at risk.
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Trusted Behçet Disease Resources
Reputable organizations and medical references for learning more about Behçet Disease, including disease registries, foundation resources, and clinical guidelines.