Behet's disease, orBehet's syndrome,is a rare and poorly understood conditionthat results in inflammation of the blood vessels and tissues.

Confirming a diagnosis ofBehet's disease can be difficult because the symptoms are so wide-ranging and general (they can be shared with a number of other conditions).

This page covers:

Behçet’s disease




Further information

Symptoms ofBehet's disease

Themain symptoms ofBehet's disease include:

  • genital and mouth ulcers
  • red, painful eyes and blurred vision
  • acne -like spots
  • headaches
  • painful, stiff and swollen joints

In severe cases, there's also a risk of serious and potentially life-threatening problems, such as permanent vision loss and strokes .

Most people with the condition experience episodes where their symptoms are severe (flare-ups or relapses), followed by periods where the symptoms disappear (remission).

Over time, some of the symptoms can settle down and become less troublesome, although they may never resolve completely.

Read about the symptoms ofBehet's disease .

DiagnosingBehet's disease

There's nodefinitive test thatcan beused to diagnose Behet's disease.

Severaltests may be necessaryto check for signs of the condition, or to help rule out other causes, including:

  • blood tests
  • urine tests
  • scans, such as X-rays , a computerised tomography (CT) scan or a magnetic resonance imaging (MRI) scan
  • a skin biopsy
  • a pathergy test which involves pricking your skin with a needleto see if a particular red spot appears within the next day or two; people withBehet's disease often have particularly sensitive skin

Current guidelines state a diagnosis of Behet's disease can usually be confidently made if you've experienced at least three episodes of mouth ulcers over the past 12 months and you have at least two of the following symptoms:

  • genital ulcers
  • eye inflammation
  • skin lesions (any unusual growths or abnormalities that develop onthe skin)
  • pathergy (hypersensitive skin)

Other potential causes also need to be ruled out before the diagnosis is made.

Causesof Behet's disease

The cause of Behet's disease is unknown, although most experts believe it's an autoinflammatory condition.

An autoinflammatory condition is where the immune system the body's natural defence against infection and illness mistakenly attacks healthy tissue.

In cases of Behet's disease, it's thought the immune system mistakenly attacks the blood vessels.

It's not clear what triggers this problem with the immune system, but two things are thought to play a role:

  • genesBehet's disease tends to be much more common in certain ethnic groups where the genes that are linked to the condition may be more common
  • environmental factorsalthough a specific environmental factor hasn't been identified,rates of Behet's disease are lower in people from an at-risk ethnic group who live outside their native country

Behet's disease is more common inthe Far East, the Middle East and Mediterranean countries such as Turkey, Iran and Israel.

People of Mediterranean, Middle Eastern and Asian origin are thought to be most likely to develop the condition, although it can affect all ethnic groups.

Treating Behet's disease

There's no cure for Behet's disease, but it's often possible to control the symptoms with medicines that reduce inflammation in the affected parts of the body.

These medications include:

  • corticosteroids powerful anti-inflammatory medications
  • immunosuppressants medications that reduce the activity of the immune system
  • biological therapies medications that target the biological processes involved in the process of inflammation

Your healthcare team will createa specific treatment plan for you depending on your symptoms.

Read about treatingBehet's disease .

SpecialistBehet's disease centres

There are three NHS Centres of Excellencethat have been set up to help diagnose and treat people with Behet's disease in England.

These are located in London, Birmingham and Liverpool.

You may be referred to one of these centres so a diagnosiscan be confirmed. Staff at these centres may also liaise with specialists at other centres to help with a person's management and treatment, even if they're not seen directly.

You can find out more about these centres on the Behet's Syndrome Centres of Excellence website .

If you haveBehet's disease, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register .

Further information aboutBehet's disease

A natural response to receiving a diagnosis of a complex condition such as Behet's disease is to find out as much as possible about the condition.

However, in the UK this may be difficult because Behet's disease is so rare that even most healthcare professionals know little or nothing about it.

A good place to start to learn more about Behet's disease is the Behet's Syndrome Society(BSS) . The BSS is the UK's main patient support group for people with Behet's disease.

The BSS website has a range of information about different aspects of Behet's disease, a member's forum, blogs, and links to other useful resources.

Content supplied by the NHS Website

Medically Reviewed by a doctor on 2 Dec 2016