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What is Behçet's disease?
Medically reviewed by Tina Patel on 04 January 2023
Behçet's disease or Behçet's syndrome is a disease affecting blood vessels and tissues of the body causing inflammation across different parts of the body. Mostly, the skin, the mouth and genitals are affected by inflammation which can lead to rashes or ulcers.
The condition is rare in the UK and there are treatments available to ease the discomfort caused by this syndrome. In very rare cases, Behçet’s can cause vision loss or permanent damage of joints.
What are the signs and symptoms of Behçet's syndrome?
Symptoms can vary from person to person. Moreover, the symptoms are quite common and wide-ranging. They can be related to a variety of conditions. The main symptoms include:
Ulcers in mouth and genital areas
Ulcers in the mouth are quite a common symptom of this condition and they will be recurring; they look like normal ulcers but may be numerous and quite painful, affecting your tongue, gums, lips and the insides of the cheeks.
Genital ulcers are very similar to mouth ulcers in appearance, but they affect men in the groin area, including the scrotum and penis and in women, they usually appear on the cervix, vulva or vagina.
Inflammation in eyes
7 out of 10 people will experience inflammation in eyes as a symptom of this disease. It can affect one eye at a time or both eyes at the same time. Inflammation affects a group of connected structures inside the eye called the uveal tract. The inflammation of uveal tract causes uveitis and can cause red eyes with pain, blurred vision and eye floaters (various shaped particles in your vision that move). Without any treatment or proper care, inflammation in the eye can lead to vision loss, which is very rare.
Painful and swollen joints
Causing arthritis-like symptoms, this disease affects knees, ankles, wrists and small joints in the hands. It causes pain, swelling, stiffness and tenderness in the joints. These symptoms can be easily controlled, but if untreated, it can lead to permanent joint damage, which is rare.
One of the common symptoms of this syndrome; any unusual growth or abnormality that develops on your skin, such as a bump or discoloured area of a skin is a lesion. This can include widespread acne-like spots and red, tender swelling on the legs. Similarly, to the other symptoms, skin lesions are also recurring.
Inflammation in the nervous system can cause headaches, double vision and seizures.
According to the NHS, although these symptoms can be controlled, in severe cases, this condition can cause life threatening problems and lead to permanent vision loss and strokes. Experiencing all the above symptoms together only happens in very rare cases.
These symptoms can act temperamentally, starting with severe symptoms, often getting worse, followed by slowing down/improvement (remission) of the symptoms and relapses. Again, the frequency and intensity of the symptoms will vary from person to person. These symptoms might settle down with time, giving you less discomfort, although, they may never resolve completely.
What causes Behçet's syndrome?
The exact cause of this syndrome is unknown. However, it is believed to be an autoinflammatory/ autoimmune condition, meaning that the body’s immune system mistakenly attacks the healthy cells/ tissues.
As a feature of this syndrome, blood vessels are mistakenly attacked by the immune system, causing inflammation in different parts of the body. The below risk factors are believed to play a role in acquiring this rare syndrome.
Risk factors for this condition:
Age – although this condition can be seen in children, the syndrome generally affects both men and women in their 20’s and 30’s.
Demography – ethnicity plays an important role in this condition. People from Middle East and East Asia, including Turkey, Japan, China and Iran are most likely to develop this disease. The NHS quotes, ‘chances and rates of this disease are lower in people from an at-risk ethnic group who live outside their native country.’
Genes – gene HLA B-51 is said to be associated with this condition as this gene is present in people affected by this disease and plays a role in immunity attacking the healthy cells. As this syndrome is not present from birth, this disease may or may not develop at the later stage.
How can Behçet's syndrome be diagnosed?
As this condition displays a wide range of symptoms, no definitive test can be used for diagnosis. However, to rule out other conditions several tests can be conducted:
- Blood test
- Urine test
- A skin biopsy
Current guidelines for this condition state that a person can be declared to be suffering with this syndrome if a person has experienced at least 3 episodes of mouth ulcers over the past 12 months and have at least 2 of the previously mentioned symptoms simultaneously.
Treatment for Behçet's syndrome
There’s no cure for Behçet's syndrome, but the symptoms can be controlled to relieve the sufferer from the discomfort of the inflammation. Additionally, these measures help in reducing potential risk of more serious complications.
Steroids – steroids are anti-inflammatory medicines; they can be consumed in different forms such as tablets, syrups, inhalers, injections (for joints and muscles), creams and lotions
Immunosuppressants – these medicines will help reduce the activity of the immune system, reducing the attacks on healthy tissues and cells
Biological therapies – this is a new treatment and includes medicines that target the biological processes involved in the process of inflammation more selectively. Although effective, biological therapy can be expensive
All the above treatment methods may cause side effects. Be sure to consult with your doctor before commencing any medical treatment options mentioned.
If you suspect that you’re suffering from any symptoms associated with this disease, the NHS offers three centres in the UK that have been set up for the specific diagnosis and treatment of this disease. For any additional help or information, the NHS recommends that you refer to behcetsuk.uk.