Behçet's Disease (BD) is a rare type of vasculitis that affects arteries and veins of all diameters (see "Vasculitis" section). Oral and genital ulcers are the most common BD symptoms, but other organ systems such as the eyes, central nervous system, gastrointestinal tract, and skin may also be affected. Male patients and younger individuals often experience more severe disease activity and higher complication rates.
This disease shows a clear geographic pattern. In North America, only 1 in 300,000 people are affected, making it very rare. Along the historic “Silk Route,” including Turkey, Iran, and Japan, Behçet’s Disease is hundreds of times more common. Populations in the Middle East are especially affected, and pathergy (an exaggerated skin reaction after minor injury) is often observed.
The exact cause of BD remains unknown, but both genetics and environmental triggers may play a role. Patients from Japan and the Eastern Mediterranean more frequently carry the HLA-B51 genetic marker, while North American and European patients do not consistently show this marker. Infectious agents, such as viruses (herpes simplex) and bacteria (streptococcus, staphylococcus, E. coli), have been suggested as possible triggers. Immune system abnormalities, such as proteins that activate white blood cells, are also found in BD patients, though it is unclear whether these are inherited.
Behçet’s often follows a pattern of flare-ups and remissions, with disease severity usually declining over time.
Diagnosing BD can be challenging due to its varied presentation. Physicians rely on clinical criteria rather than a single test. The 1990 International Criteria include:
Recurrent oral ulcers plus two of the following:
Supporting tests, biopsies, or imaging may be used to rule out similar conditions such as lupus.
BD treatment is personalized, depending on organ involvement.
Mild Disease (mucous membranes & skin):
Moderate Disease:
Severe Organ Involvement (eyes, lungs, CNS, heart):
Regular follow-up is essential. Missing treatments or stopping medications suddenly may worsen symptoms.
If you are looking for a Behçet’s disease specialist near you, consider:
Early treatment with an experienced Behçet’s specialist can significantly improve long-term outcomes.
The HLA-B51 genetic marker is strongly associated with Behçet’s in Eastern populations. Current research explores how genetics and environment play a role in disease development and whether targeted therapies may reduce future flare-ups.
Behçet’s Disease is a complex autoimmune vasculitis requiring ongoing monitoring and tailored treatment. If you suspect symptoms, book an appointment with a Behçet’s specialist in Scottsdale, AZ. Regular check-ups, appropriate medications, and lifestyle adjustments can help manage flare-ups and prevent serious complications.
If you need help finding a Behçet’s syndrome doctor near you, local vasculitis experts in Scottsdale can provide guidance and long-term care.