This week’s medical condition of the week is Behçet’s disease, a rare condition in the UK with a prevalence of about 1-5 in every 100,000 people. It is however more common in the Middle East, Far East and Mediterranean. It was in deed first recorded in 1993 by the Turkish dermatologist Hulusi Behçet, who was the first to realise that a group of symptoms, all previously thought to be unrelated, are caused by a single condition.
Behçet’s disease of Behçet’s syndrome is a chronic condition affecting the immune system in the body, causing uncontrolled and exaggerated inflammation (swelling) of the blood vessels, namely the smaller ones. This causes areas of swelling, responsible for the symptoms of the condition. Two of the main areas affected by inflammation are the mouth – causing severe, painful mouth ulcers, and the genitals – causing genital ulcers, which appear in 75-90% of cases.
However, as Behçet’s disease causes inflammation of the blood vessels, swelling can occur anywhere in the body where there is a blood supply. This can include joints, producing arthritis like symptoms in two-thirds of people with Behçet’s, as well as skin liaisons usually seen on the lower limbs which are present in around 80% of people with the condition.
Another major area effected can be the eyes, which inflammation of the eyes seen in an estimated 30% of people with Behçet’s disease. This often takes the form of uveitis, inflammation of the uveal tract – the group of connected structures inside the eye, including the iris (coloured part of the eye), ciliary body (the ring of muscle behind the iris) and the choroid (the layer of tissue supporting the retina). Symptoms characteristically seen with uveitis are painful red eyes, photophobia (sensitivity to light), dots moving across the field of vision and blurred vision. In severe cases, this can lead to permanent visual impairment.
Behçet’s disease can also lead to an inflammation of the stomach and intestine leading to gastrointestinal disease, with symptoms such as vomiting, diarrhoea, loss of appetite and abdominal pain. It is also estimated that 5-10% of sufferers will develop inflammation of the central nervous system, leading to symptoms such as headaches, double vision, loss of balance, drowsiness and uncontrollable twitching or shaking.
Naturally, as Behçet’s disease causes an inflammation of the blood vessels, blood clots are also more likely in suffers of the condition. However, the treatments greatly reduces these risks.
At the moment there is no specific diagnosis for Behçet’s disease, however, once diagnosed it can be treated by immunosuppressant in order to reduce the inflammation. With medication it is possible for sufferers of the condition to lead a health life. However, relapses of the condition can occur which must then be treated accordingly, often by a specialist in that area.
Sources:
http://www.nhs.uk/Conditions/Behcets-disease/Pages/introduction.aspx
http://behcets.org.uk/
Gracey's medical blog 