What is Behcet's disease?
Behcet's disease is a chronic condition in which the bodys immune system, which normally protects the body against infections, becomes over-active.
Behcets disease causes inflammation of different parts of the body. These may flare up without warning and can occur wherever there is a blood supply. Small blood vessels are particularly affected, and inflammation can appear in patches in areas where there are clusters of small blood vessels. Where this occurs in the body dictates the symptoms a person experiences.
Behcet's disease generally begins when a person is in their 20s or 30s. Behcet's disease is rare in the UK. The condition is more common in Mediterranean countries, Turkey, the Middle East, Japan and South-East Asia.
People with Behcet's disease will have several of the following symptoms:
- Mouth ulcers. These affect almost everyone with Behcets disease. They occur around the mouth, tongue and throat, and are often painful.
- Genital ulcers. These affect more than half of all people with Behcets disease. Women may get ulcers either in the vagina or on the vulva. In men ulcers may affect the scrotum and the penis. Ulcers may also occur around the anus and in the groin. Men may have swollen testicles.
- Skin sores, including spots, boils, red patches, ulcers, and lumps under or in the skin.
- Eye problems. Inflammation of the middle part of the eye (uveitis) occurs in more than half of all people with Behcets disease. The back of the eye, next to the retina (retinal vasculitis) can also be affected. Symptoms include 'floaters', haziness or loss of vision, pain and redness in the eye.
- Inflammation of the joints causing pain and swelling. This occurs in more than half of all patients with Behcets disease.
- Inflammation and ulceration (sores) in the digestive system. This sometimes leads to stomach pain, diarrhoea, constipation, and vomiting.
- Severe headaches due to inflammation.
- Inflammation of blood vessels, which increases the risk of thrombosis (blood clots). Thrombosis in veins near to the skin surface can become painful, hot and red. Thrombosis in deeper veins or arteries leads to pain and swelling of the affected limb.
- Extreme tiredness.
Causes of Behcet's Disease
The cause of Behcets disease is unknown. It is not hereditary but there may be genetic factors that make some people more likely to develop the condition.
It is possible that a virus or bacteria may be the cause of the syndrome, but a specific infection has not yet been identified. It is also possible that Behcet's disease is an 'autoimmune' disease in which the immune system attacks the body's own tissues.
Behcets disease is not infectious: it cannot be spread from person to person.
Diagnosis of Behcet's Disease:
There is no specific test for Behcets disease. The diagnosis is based on the clinical signs and symptoms that are associated with the disease. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time.
Behcet's disease is diagnosed by specific patterns of symptoms and repeated outbreaks of them. These include recurrent mouth ulcers (more than three in a twelve month period) plus any two of the following symptoms:
- genital ulcers,
- skin sores such as lumps or boils,
- eye inflammation, and
- a positive pathergy test (skin prick test). This measures increased sensitivity of the skin, which occurs in Behcet's syndrome.
These symptoms are also associated with Crohns disease and Reiters syndrome, and your GP will need to rule these out before diagnosing Behcets disease. Your GP may also refer you to an eye specialist to identify possible complications related to eye inflammation.
Behcet's Disease Treatment
There is currently no cure for Behcet's disease. Treatment is designed to control symptoms and reduce inflammation. The aim is to reduce pain and prevent serious complications such as blindness and severe arthritis.
Behcet's disease is generally chronic (long-term) but usually manageable, with periods of remission and relapse. Since it can affect many different parts of the body, someone with Behcet's disease may need to be seen by several different specialists. It is likely that a combination of treatments will be needed to relieve specific symptoms. These may include:
- Creams, ointments or gels applied directly to the inflammation, sore or ulcer. These will usually contain corticosteroids to reduce inflammation or an anaesthetic to relieve pain or antibiotics.
- Oral drugs:
1. Corticosteroids reduce pain and swelling in the body. They are used to treat severe joint pain and inflammation, skin sores, eye disease, or central nervous system symptoms.
2. Immunosuppressive drugs (containing corticosteroids) help to control an overactive immune system, reduce inflammation throughout the body and reduce the frequency of flare-ups.
- Moderate exercise, such as swimming or walking, is usually recommended during periods of remission to keep up general levels of fitness.
- A healthy well-balanced diet. Behcets disease is not caused by poor diet, but good general health can help to improve symptoms. Most people with Behcets disease will have a normal life span
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