Behcet’s disease is a rare inflammatory condition affecting multiple parts of the body. It causes systemic vasculitis, a widespread inflammation of the blood vessels. Blood vessels are channels that transport blood around the body. These channels include veins, arteries, and capillaries.
Currently, experts do not know the cause of Behcet’s disease. They do, however, believe that it may relate to autoimmune activity in the body.
This article discusses Behcet’s disease and its symptoms, causes, and risk factors. It also explains the treatments for the condition.
Most people with the disease experience periods in which symptoms intensify and periods in which the symptoms alleviate.
Behcet’s disease can cause sores to form on various parts of the body.
The main symptoms of the condition include:
- ulcers, particularly in or around the mouth or genitals
- flushed or red eyes that are painful
- blurred vision
- spots that appear like acne
- stiff, swollen, or painful joints
Affected areas can include:
- Mouth: Sores that resemble canker sores may form on the mouth. They can be painful and recurrent, meaning they can appear repeatedly.
- Skin: Flushed, acne-like sores or spots may appear across the skin. They may have a raised or low-lying appearance.
- Genitals: Painful sores may develop on the genitals, such as on the scrotum, vulva, or surrounding areas. In some cases, the sores may leave scars.
- Eyes: Eye inflammation is another common symptom of Behcet’s disease. Redness, light sensitivity, and blurred vision may also occur.
- Joints: Some people with Behcet’s disease also report painful joints. Joint swelling may affect the ankles, knees, elbows, and hips.
- Blood vessels: Inflammation in the blood vessels may disrupt circulation and cause blood clots. It may also cause swelling in the limbs.
- Gastrointestinal (GI) tract: Lesions may form in the GI tract. They may cause additional symptoms, such as bloody stools, diarrhea, or abdominal pain. In some severe cases, intestinal rupture can also occur.
- Nervous system: Brain and spinal cord inflammation may also occur. Symptoms can include headache, fever, disorientation, and a stiff neck. Stroke, seizure, and difficulty balancing or coordinating movement are also possible.
- Other body parts: In rare cases, the condition can also affect the nerves, lungs, and kidneys.
When to seek medical help
Make an appointment with your doctor promptly for any symptoms of Behcet’s disease, including new sores and other inflammation.
Inflammation of the brain can cause damage to the neurological system, so seek immediate medical care for any symptoms affecting the brain. While these symptoms may resemble Behcet’s, they can also occur due to serious or emergency conditions. A medical evaluation can determine the cause of your symptoms.
Seek emergency medical treatment or call 911 for anyone experiencing even temporary symptoms of:
- disorientation, confusion, or loss of memory
- changes in consciousness
- difficulty moving, balancing, or speaking
- paralysis or numbness
- face drooping or one-sided weakness
- severe headache
Learn more about neurological symptoms and causes.
What causes Behcet’s disease?
Currently, the cause of Behcet’s disease is unknown. However, evidence suggests that the disease may be an autoimmune and autoinflammatory disorder.
Autoimmune disorders occur when the body’s immune system mistakenly attacks healthy tissues in the body. Autoinflammatory refers to when the body cannot effectively regulate or control inflammation. These characteristics may lead Behcet’s disease to mistakenly attack or inflame the blood vessels.
Clinicians do not know the reason for this. However, a combination of genetic and environmental factors may play a role.
Clinicians suggest that Behcet’s syndrome could occur due to a genetic predisposition. This suggests that an inheritable genetic trait can activate an autoinflammatory response after exposure to an environmental trigger. These genetic differences could include having certain genes or increased levels of certain human leukocyte antigens, which play a role in the immune response.
Experts are also not yet able to confirm definite environmental factors that trigger the condition’s onset. However, research suggests that these factors may include bacterial or viral infection.
Researchers are still studying the condition to fully understand it and find the underlying cause.
Is Behcet’s disease a sexually transmitted infection?
Behcet’s disease is not a sexually transmitted infection (STI). It is not infectious or contagious. The condition cannot spread from one person to another, including through physical contact.
Behcet’s disease is more common in some populations than others. This means people from certain groups may have an increased risk of developing the condition.
These factors include:
- Age: Although Behcet’s disease can occur at almost any age, it most commonly affects people from 20-30 years old. The condition is rarer before puberty or in people over age 50.
- Race: People from the Middle East and Asia tend to develop Behcet’s disease more frequently than people from other places.
- Sex assigned at birth: All sexes can develop Behcet’s disease, and the distribution rate is equal globally. However, the distribution of the disease across sexes varies within certain groups. The disease more commonly affects males in Turkey and the Middle East. In contrast, females may be more likely to experience the condition in Korea, China, the United States, and northern Europe.
Certain factors can increase the risk of the condition being severe. These factors include being male and being younger at the time of the condition’s onset.
No one test can diagnose Behcet’s disease. Doctors will analyze a variety of variables to arrive at a conclusion.
They may start by examining your symptoms, including the severity and frequency with which they occur.
They may also order some laboratory tests to rule out other conditions with similar symptoms. These tests can include:
- Blood test: This looks for evidence of inflammation or other underlying conditions in your blood. It also evaluates your overall health.
- Urine test: This examines your urine’s visual, chemical, and microscopic features for signs of a disorder.
- Imaging tests: Imaging tests can check internal organs and structures for indications of a disease. Examples of imaging tests include X-ray, CT scan, and MRI.
- Skin biopsy: This is when a doctor takes a small sample of your skin for testing. The test can confirm the presence of a skin disorder, especially when examining sores and other skin symptoms.
- Pathergy test: This is when a doctor pricks your forearm with a sterile needle to see how your body responds. Clinicians can use a pathergy test to help positively diagnose Behcet’s disease.
Currently, there is no specific treatment for Behcet’s disease. However, medications can help you manage symptoms and reduce the risk or impact of complications.
These medications include:
- Corticosteroids: Corticosteroids, such as prednisone, can reduce inflammation and autoimmune activity.
- Immunosuppressant drugs: Immunosuppressants, such as methotrexate (Trexall), can treat sores and joint pain by reducing the immune response. Unlike steroids, which do not protect from the damage of the condition when used alone, immunosuppressants can help protect from damage due to inflammation.
- Other medications: A doctor may recommend both over-the-counter and prescription products or medication to alleviate various symptoms. For example, they might recommend eye drops for eye inflammation and mouthwashes for mouth sores.
Your doctor will recommend a custom treatment plan based on your condition to support your individual experience.
Follow your specific treatment plan even if your symptoms go away or temporarily improve.
Living with Behcet’s disease
Behcet’s disease can have serious physical, mental, and social implications for some people.
If you have the disease, you may find certain practices useful in helping you cope with your symptoms. These can include:
- getting sufficient rest
- staying active and exercising regularly
- sharing your experience with family, friends, or a support group
- regularly meeting with a mental health professional or therapist
It may also help to connect with other people living with the disease. You can do so by joining a support group. The American Behcet’s Disease Association offers in-person and virtual events for people with the condition.
In some cases, Behcet’s disease can lead to various complications, especially if the condition does not receive effective or appropriate treatment.
The type of complication may depend on the area of the body that the condition affects. Complications of severe or untreated Behcet’s disease can include permanent damage or loss of function of a body part. For example, eye inflammation may gradually progress into decreased vision or blindness. Similarly, brain inflammation may result in a stroke.
In pregnant people, the condition may increase the risk of miscarriage as vasculitis can occur in the placenta.
However, prompt treatment can effectively reduce the risk of complications or damage. Contact your doctor if you experience any new or worsening symptoms to optimize your treatment.
Outlook and life expectancy
Mortality from the condition can be significant. Mortality rates increase among male populations or those with a younger age of onset for the condition. The condition may cause ruptures, aneurysms, or neurological or GI effects. However, effective treatment can relieve many symptoms and help prevent complications.
Around 60% of people with the disease experience remission, or a temporary recovery, after the years when the condition is most active. Additionally, most people who experience remission also experience an improvement in their prognosis.
If you have Behcet’s disease, schedule regular appointments to help you monitor the status or progression of your symptoms.
Behcet’s disease, also Behcet’s syndrome or silk road disease, is a chronic condition that causes inflammation of multiple blood vessels in the body. Symptoms can include sores, pain when moving joints, and eye inflammation.
The exact cause of the condition is unclear. However, experts believe it may result from an autoimmune response to genetic differences and environmental triggers, such as infection.
Currently, there is no known cure. However, some medications can help alleviate symptoms and complications. These include corticosteroids and immunosuppressant drugs.
Contact your doctor promptly if you have symptoms of Behcet’s disease. Seek emergency medical care for neurological symptoms, such as difficulty speaking, loss of consciousness, weakness, or paralysis.