What Is Behçet’s Disease, and Is It Serious?
Key takeaways:
Behçet’s disease causes chronic inflammation of blood vessels throughout the body.
Common symptoms include oral and genital sores, skin lesions, and eye and joint inflammation.
Treatment usually involves corticosteroids or other medications that act on the immune system.
Behçet’s disease (also called Behçet’s syndrome) is a rare inflammatory disorder. It’s named after Dr. Hulusi Behçet, the Turkish dermatologist who first described it in 1937. It is less common in the U.S. than it is in areas located along the Silk Road, which refers to the ancient route that linked the Far East with the Mediterranean Sea.
Since the disease isn’t well-known in the U.S., we’ll walk through how to recognize it, how it’s diagnosed, and what treatment looks like.
What is Behçet’s disease?
Behçet’s is a long-term disease that causes inflammation of large and small blood vessels (arteries and veins) in different parts of the body. The cause is still unknown, but it is thought to be autoimmune. Behçet’s disease affects men and women equally. It typically affects young adults and is commonly diagnosed before age 40.
What are the symptoms of Behçet’s disease?
Behçet’s disease can affect any organ in the body, and different people are affected in different ways. The symptoms can range in severity.
Most symptoms of Behçet’s disease are not life-threatening but can be disabling. The most common symptoms are:
Oral ulcers: Often, the first symptom of Behçet’s disease is painful mouth sores. They can be debilitating, as they make it difficult to eat, drink, and talk. These sores heal in a few days but tend to come back often. Almost all individuals affected by Behçet’s disease get these ulcers.
Genital ulcers: Genital sores affect at least half of people with Behçet’s disease. In men, they usually appear on the penis and scrotum, and in women, they usually appear on the vagina and vulva. They are similar in appearance to the mouth sores and can be painful.
Skin lesions: A variety of skin changes are possible. These include tender red bumps called erythema nodosum and acne-like lesions, such papules, nodules, and pustules.
Gastrointestinal (GI) symptoms: Behçet’s disease can cause ulcers throughout the GI tract. These mainly affect the esophagus and colon, causing GI bleeding and abdominal pain.
Joint symptoms: Joint pain and swelling most commonly affect the knees, ankles, hands, and wrists. While this can be painful, permanent joint damage is rare.
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Occasionally, more severe and dangerous symptoms can occur:
Eye inflammation: The most problematic symptom affects eyes and vision. Inflammation of a part of the eye (uveitis) can develop in more than half of individuals with Behçet’s disease. This is dangerous and can cause permanent vision damage. Both eyes can become damaged in a very short time.
Inflammation in blood vessels: This usually affects veins and can cause blood clots, especially in the legs. Less commonly, large arteries can be affected, which can be life-threatening.
Brain inflammation: This is one of the most serious symptoms of Behçet’s. This can cause headache, strokes, and sometimes, dementia.
Like autoimmune diseases, Behçet’s is a remitting-relapsing condition. This means that the symptoms are sometimes worse (relapsing) and at other times improved or resolved (remitting).
How is Behçet’s disease diagnosed?
Behçet’s disease is difficult to diagnose because the symptoms are highly variable. There is also no specific blood test for this condition. Blood work may show increased inflammatory markers but usually only when symptoms are active. Therefore, the diagnosis is typically based on various clinical criteria.
Diagnosis requires at least 3 episodes of oral ulcers in 12 months. It also requires at least 2 of the following 4 features:
Recurrent genital ulcers
Skin lesions
Eye inflammation (uveitis)
Positive pathergy test
During a pathergy test, a sterile needle is used to make multiple pricks in the skin of the forearm, which is examined after 1 to 2 days. The test is positive if a red bump, pustule, or ulcer forms where the prick was made. While it can be helpful in diagnosis, not all people with Behçet’s disease test positive on a pathergy test. A positive reaction can also be seen in conditions other than Behçet’s disease.
The symptoms of Behçet’s can overlap with many other conditions, which need to be ruled out when making a diagnosis. In particular, the following need to be excluded:
Other autoimmune diseases like as systemic lupus erythematosus (SLE)
Inflammatory bowel disease (IBD)
Sexually transmitted diseases (STDs) such as Herpes virus
Other causes of oral ulcers like vitamin deficiencies
Why is so little known about Behçet’s disease?
Very few large studies on Behçet’s disease exist. (The largest and longest study of people with the disease is almost 20 years old.) This is because diagnosing Behçet’s is difficult. There is often a very long delay between symptom onset and diagnosis for many reasons:
The disease behaves differently in different people.
Symptoms are highly variable and affect many different organs.
The course of the disease is unpredictable, and symptoms tend to come and go.
Symptoms can overlap with other diseases that need to be ruled out.
There is no specific laboratory test or marker for the disease.
Because Behçet’s is very rare, it may not occur to providers when making a diagnosis.
Can Behçet’s disease be treated or cured?
The treatment is directed at controlling symptoms; there is no known cure. The unpredictable nature of the disease’s waxing and waning can be frustrating. The good news is medications can help with symptoms.
Specific treatments depend on the body parts affected. For instance, skin and genital lesions are treated with topical corticosteroid creams or ointments. Mouthwashes containing corticosteroids or other medications can help alleviate mouth sores. If topical medications are not effective, a drug called colchicine may be an option. It can help with oral and genital sores, as well as joint symptoms.
Apremilast is the only FDA-approved treatment for oral ulcers caused by Behçet’s disease. In a study of more than 200 adults, treatment with apremilast improved pain and decreased the number of active oral ulcers caused by Behçet’s.
When symptoms are moderate to severe, treatment can include corticosteroids and other medications that modulate or suppress the immune system:
Corticosteroids: These medications help reduce inflammation caused by Behçet’s disease. They cannot be used long term due to side effects, such as cataracts, glaucoma, bone loss, and high blood pressure, among others.
Immunosuppressants: These medications help to calm down an overactive immune system. This, in turn, decreases the inflammation found in blood vessels. Examples of these medications include azathioprine, cyclosporine, or cyclophosphamide.
Immunomodulators: Tumor necrosis factor (TNF) inhibitors, or immunomodulators, decrease inflammation by targeting TNF, an inflammatory substance in the body. These agents are reserved for more severe cases. Examples of these medications include Infliximab and adalimumab (Humira).
It’s important to note that immunosuppressants and immunomodulators are not approved by the FDA for treatment of Behçet’s. But they are commonly prescribed and recommended by experts.
The bottom line
Behçet’s disease is rare and poorly understood. It commonly causes recurrent oral and genital sores, although it can affect any organ in the body. Some symptoms are mild and require only topical therapies. Others are more serious and warrant more aggressive treatment. Many treatment options are available to help relieve symptoms.
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References
Davatchi, F., et al (2011). Diagnostic value of pathergy test in Behcet's disease according to the change of incidence over the time. Clinical Rheumatology.
Hatemi, G., et al. (2018). 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Annals of the Rheumatic Diseases.
Hatemi, G., et al. (2019). Trial of apremilast for oral ulcers in Behçet’s syndrome. New England Journal of Medicine.
Kural-Seyahi, E., et al. (2003). The long-term mortality and morbidity of Behçet syndrome: A 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine.
Nair, J., et al. (2017). Behcet's disease. Clinical Medicine (London).
National Institutes of Health. (2013). Genetic mystery of Behcet’s disease unfolds along the ancient Silk Road.
Yazici, Y., et al. (2021). Behçet syndrome. Nature Reviews Disease Primers.
