What is Behçet disease?
Behçet disease is a rare disease characterised by painful mouth ulcers, genital ulcers, eye problems and skin lesions. The disease is named after the Turkish dermatologist Hulusi Behçet who first described the syndrome in 1924. It is also known as Adamantiades–Behçet disease.
What causes Behçet disease?
The cause of Behçet disease is unknown but it is presumed to be an autoimmune disorder. This is where an individual's immune system starts reacting against his or her own tissues. The reason for this remains a mystery but it is possible bacterial or viral infection may have a role in its development.
Who is at risk of Behçet disease?
Behçet disease is most common and more serious in people with Silk Road bloodlines. Silk Road countries include those in the Mediterranean basin, Middle East and Far East; the incidence is around 1 in 10,000 people. However, Behçet disease is seen worldwide even in those with other ethnic heritage; in the US, the incidence is reported to be 1 in 20,000.
In people with Silk Road ancestry, Behçet disease is more common in men than in women. However, the trend is reversed with more women than men being affected in people of other ethnic origins. The disease can develop at any age but is most common as people reach 20–30 years.
What are the signs and symptoms of Behçet disease?
Mouth ulcers (aphthous ulcers) are the most common and earliest sign of Behçet disease. However, before these appear a patient may for a number of years experience a variety of recurrent signs and symptoms that include:
- Sore throats and tonsillitis
- Muscular and joint pain
- Malaise, generalised weakness
- Anorexia, weight loss
- Fluctuations in body temperature.
Painful mouth ulcers are usually the first outward sign of Behçet disease and occur in about 70% of patients. Ulcers can occur anywhere in the mouth including on the tongue and inside the lips and cheeks. They usually last for 1–2 weeks but can remain for as long as 3 weeks.
Other common signs and symptoms indicative of Behçet disease include:
- Genital ulcers: these aphthous ulcers occur less often than mouth ulcers. They are painful and often heal with scarring. They are not genital herpes, which is caused by the herpes virus.
- Eye involvement: uveitis, which is a reddening and swelling of the eye tissue, may occur. The retina may sometimes become involved and if not treated can lead to blindness.
- Skin lesions: painful nodules from erythema nodosum are common. Patients may also have acne-like sores that occur on the arms, legs and trunk.
Less common symptoms include, gastrointestinal problems (eg, abdominal pain, diarrhoea, vomiting), joint pain and swelling, nervous system problems and blood vessel and circulation problems.
How is Behçet disease diagnosed?
Behçet disease can be difficult to diagnose as signs and symptoms come and go and may not be apparent all at one time. If you have a history of recurrent mouth and genital ulcers, uveitis and skin sores this is suggestive of the condition.
The International criteria for classification of Behçet disease defines the conditions as:
At least three episodes of recurrent oral ulcers in a 12-month period plus at least two more of the following:
- Genital ulcers
- Eye involvement (uveitis or retinal damage)
- Skin lesions
- Positive pathergy test (this is a skin prick test that can confirm Behçet disease)
There are no specific tests to confirm the diagnosis, but screening investigations such as blood count, kidney and liver function tests and C-reactive protein may be performed to determine which organs are affected by the condition.
What is the treatment for Behçet disease?
Currently there is no cure for Behçet disease. The main goal is to treat and manage the symptoms so that complications do not develop. Because Behçet disease affects so many different parts of the body, a team of doctors from various specialties will treat most people.
Some medications that may help in controlling symptoms include:
- Oral corticosteroids
- Nonsteroidal anti-inflammatory drugs (eg, aspirin, ibuprofen)
- Immunosuppressants (eg, azathioprine, chlorambucil)
- Experimental and research drugs (eg, colchicine, cyclophosphamide, thalidomide, infliximab and potassium iodide).
What is the prognosis?
Behçet disease is a chronic disease where the acute phases come and go with varying degrees of intensity. In the early stages of the disease attacks may be frequent and last for several weeks. As time progresses the intervals between attacks may become longer and in some cases attacks cease altogether. The disease is then considered to be in remission but may strike up again at any time.
Death occurs in about 4% of Behçet cases. Causes of mortality are attributed to gastrointestinal perforation, central nervous system involvement and vascular aneurysms (ballooning of blood vessels).
Most Behçet patients live out a full life although they will most likely deal with some level of symptoms throughout this time.