Home | Contact | Sitemap
Home : Behcet’s Disease : What is Behcets Disease

What is Behcets Disease

Prepared by Dr Wafa Madanat, MD PhD, Amman, Jordan; edited by Dr Colin G Barnes, UK.

IMPORTANT NOTICE: This manuscript is prepared to give some information on Behcet's disease to the patients. Please always follow the suggestions of your physicians.

Facts and overviews
1. What is Behcet`s disease?
2. Who gets Behcet`s disease - and does it occur in certain geographical areas?
3. What causes Behcet`s disease?
4. What are the symptoms of Behcet`s disease?
5. How is Behcet`s disease diagnosed?
6. Course and prognosis.
7. Who is the specialist for Behcet`s disease?
8. Treatment.

Frequently asked questions

9. Where did the name come from?
10. Is it hereditary? 
11. Is it contagious? 
12. Is HLA B51 positivity diagnostic for Behcet`s disease?
13. Can I avoid Behcet`s disease?
14. Can I have children?
15. What triggers Behcet`s disease?
16. If someone has recurrent oral ulcers, does this mean that he or she are going to develop Behcet`s disease? 17. Recommendations.

Facts and overviews

1. What is Behcet`s disease?

Behcet`s disease is a systemic vasculitis (inflammation of large and small blood vessels) of an unknown cause.
It is characterized by recurrent oral and /or genital ulcerations, inflammation of the eyes (uveitis) and skin lesions. It can also affect the joints, all types of vessels, lungs, central nervous system and digestive tract.

2. Who gets Behcet`s disease - and does it occur in certain geographical areas?

The disease occurs most frequently in the Middle East, it is observed more among populations living along the historic Silk Road, which extended from eastern Asia to the Mediterranean basin, that’s why it was given the name silk road disease, but it also occurs in the United States of America, South America and northern Europe.

The prevalence of the disease varies in different countries, epidemiological surveys have shown the following data:
11.9 per 100.000 in Japan (2003), 
2.26 per 100.000 in Germany, 
1.4 per 10.000 in China,
1.67 per 10.000 in Iran
8-37 per 10.000 in Turkey
2.0 per 10.000 in Saudi Arabia
1.7 per 10.000 in Iraq
0.76 per 10.000 in Egypt
0.25 per 10.000 in Europe

Regional differences are a well known fact of Behcet`s disease, for example the frequency of gastrointestinal lesions is higher in cases reported in Japan but are rare in the Mediterranean and European populations. Similarly pulmonary lesions are rare in Japan but more frequent in Europe.

Sex ratio also varies from country to country, men are more affected than women in Turkey and Arab countries, and the ratio is usually between 2-3 men: 1 woman, while in Japan and some European countries women are more affected by the disease. Familial cases are reported, but are uncommon. Behcet`s disease can affect women and men of all ages, but it develops most often between ages 20 and 39.

3. What causes Behcet`s disease?

Scientists do not know the exact cause of Behcet`s disease, but extensive work is ongoing. Most symptoms are caused by inflammation of blood vessels. Inflammation is characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat and pain. Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Usually the immune system protects the body from diseases and infections by attacking germs or viruses, when an autoimmune reaction develops the immune system attacks and harms the body's own tissues.

Doctors believe that two factors are important for a person to get Behcet`s disease. First, it is believed that abnormalities of the immune system make some people susceptible to the disease, scientists believe that this susceptibility may be inherited; that is, it may be due to one or more specific genes (but this does not mean that it is transmitted like inherited disease). Second, something in the environment, possible a bacterium or virus, might trigger or activate the disease in susceptible people.

Behcet`s disease is NOT infectious nor cancerous nor Aids-related. It does NOT spread from one person to another.

4. What are the symptoms of Behcet`s disease?

Behcet`s disease affects people differently. Some patients have only mild symptoms, such as sores or ulcers in the mouth or on the genitals. Others have more severe signs. Symptoms can last for a long time or may go and come in few weeks, the times when a person is having symptoms are called flares. Different symptoms may occur with each flare.

The most common symptoms of Behcet`s disease are:

Oral ulcers 
Almost all patients with Behcet`s disease suffer from mouth ulcers (they are identical to aphthous ulcers occurring without Behcet`s disease in many people). Ulcers are usually painful and make eating difficult; they may reappear occasionally or frequently. These ulcers can affect the tongue or anywhere around the mouth and throat. They are often the first symptom that a person notices and can occur long before other symptoms.
The ulcers usually have a red border and several may appear at same time. They are called minor if they are superficial and heal in less than a week, and major if they are deep and take longer time to heal sometimes with scarring. Multiple small size ulcers are called herpetiform.

Genital ulcers
Genital ulcers affect more than half of all people with Behcet`s disease, in men these most commonly appear on the scrotum and the vulva in women. In both sexes they may appear around the anus and in the groin. The sores look similar to the mouth ulcers and may be painful. They may cause scarring.

Skin lesions 
These are a common symptom of the disease. Skin lesions often look red or resemble pus-filled bumps or a bruise. The sores are red and raised , and typically appear on the legs. In some people, sores or skin lesions may appear when the skin is scratched or pricked. When doctors suspect that a person has Behcet`s disease they may perform a pathergy test, in which they prick the skin with a sterile needle; 1 to 2 days after the test, people with Behcet`s disease may develop a red bump where the doctor pricked the skin. But it seems that only half of the patients in the middle and far east have this reaction. It occurs very infrequently in the UK and USA, but if this reaction occurs then Behcet`s disease is likely.

Eye inflammation (Uveitis)
Eye involvement involves inflammation of the middle or back part of the eye (uvea) including the iris, and occurs in more than half of all patients with Behcet`s disease. Eye inflammation can cause blurred vision, floaters (dots which appear to float across the field of vision), rarely, it can cause pain and redness. Because loss of vision can result if the inflammation persists, patients should report these symptoms to their doctors immediately, to start treatment as early as possible. In addition to these symptoms Behcet`s disease may also cause painful joints, blood clots, and inflammation in the central nervous system and digestive organs.

Painful joints
Inflammation of the joints occurs in some patients with Behcet`s disease. Patients might experience pain and swelling and stiffness especially in the knees, ankles, wrists and elbows. These symptoms may last for a few weeks, usually come and go, but usually does not cause damage to the joint.

Blood clots (Thrombosis)
Some patients with Behcet`s disease have blood clots resulting from inflammation in the vessels, veins usually are affected more than arteries. If the veins near to the skin are affected, then pain, redness and tenderness may be felt in the affected area, which may become swollen and warm. If deeper veins are involved then the patient will notice pain and swelling. The legs are usually more affected than arms, in some cases thrombosis can occur in the blood vessels of the head, lungs and internal organs.

Central nervous system 
Behcet`s disease can cause inflammation of the brain and the thin membrane that covers and protects the brain and spinal cord. Patients may experience headaches, stiff neck, fever, double vision, difficulty hearing, and difficulty in coordinating movement, faints, numbness or unusual sensations. These symptoms should be reported to the doctor immediately. Sometimes headaches are not serious, but the doctor will decide this after performing the necessary tests.

Digestive tract 
Rarely Behcet`s disease causes inflammation and ulceration throughout the digestive tract that are identical to the aphthous lesions in the mouth, in these cases the patient will complain of abdominal pain, diarrhoea, and or bleeding. These symptoms are similar to symptoms of other diseases like Crohn`s disease and ulcerative colitis, so careful evaluation is needed to rule out other diseases.

Some males with Behcet`s disease might experience pain and swelling of one or both testicles. In such cases doctors will need to perform some laboratory tests to rule out other causes that might be the cause of such condition. Other organs like the heart and kidneys might be involved but rarely.

5. How is Behcet`s disease diagnosed?
Diagnosing Behcet`s disease is not simple because there is no specific laboratory test yet to confirm it. When a patient reports symptoms, the doctor must examine the patient and rule out other conditions with similar symptoms. The diagnosis is especially difficult when symptoms do not appear together, sometimes it may take several months or even years for all the common symptoms to appear. The following symptoms are key to the classification of Behcet`s disease when it is necessary to compare groups of patients.

Recurrent oral ulcers at least three times in 12 months.
Any 2 of the following symptoms (genital ulcers or skin lesions or eye inflammation or positive pathergy test).

The diagnosis in the individual patient depends on the association of various features of the disease to enable the doctor to make the diagnosis

6. Course and prognosis.

Behcet`s disease is a long term problem; there will be times when patient can be free of symptoms (and these times are called remissions), and this does not mean that the disease is cured, usually new symptoms develop later, (central nervous system involvement and major vessel disease could appear late during the course of Behcet`s disease after 5-10 years). The frequency of oral and genital ulcers and joint symptoms might decrease with time. About 25% of patients with eye disease might develop severely impaired vision and this does not always mean an eventual loss of useful vision, but those patients who develop the eye disease at older ages have a better visual prognosis. The right treatment may prevent new symptoms from appearing and control the existing ones. The mortality between patients with Behcet`s disease is about 9%, mainly because of 
large vessel disease and neurological involvement. Male patients who develop the disease at younger ages (before 25 years) might be at greater risk to have a more severe disease.

7. Who is the specialist for Behcet`s disease?

Behcet`s disease is classified in the medical literature as vasculitis which is usually followed by rheumatologists and internists. Because the disease might affect different organs, other doctors like ophthalmologists (doctor who deals with the eyes), neurologists (for problems affecting nervous system), dermatologists (for skin problems), gynecologists (for genital ulcers) and gastroenterologists (for digestive problems) might be involved, and treatment must be collaboration between them to select the best medication for each patient. Patient should tell each of their doctors about all of the medications they are taking so that doctors can coordinate treatment.

8. Treatment

The goal of treatment is to reduce discomfort and prevent serious complications of the disease. The type of medicine and duration of treatment depend on the person's symptoms and their severity; sometimes doctors may use a combination of medications to relieve specific symptoms.
Commonly prescribed medications include:

Corticosteroids- topical for ulcers, eye drops, tablets or injections,
Immunosuppressive drugs,
Interferon alpha,
Anti- TNF agents,

Patients are advised not to use any of the above listed medications without doctors prescription or on their own, since the indication and dosage of each of these drugs is different for each patient and depends on the severity and specific manifestations of the disease. On the other hand the doctor will ask the patient to do regular blood tests to monitor toxicity and side effects.
Finally it is important to note that increased professional awareness and the use of new medications during the last 20 years have contributed to a better outcome of treatments and management of patients.

Frequently asked questions

9. Where did the name come from?

Behcet`s disease owes its name to the Turkish dermatologist Hulusi Behcet, who in 1937, described the classic triple symptom complex of hypopion/ iritis, and orogenital aphtosis and proposed that this may be a separate disease entity.. The Greek physician Adamantiades had reported the disease 6 years earlier, that’s why in some countries it is called Adamantiades-Behcet`s disease. However, the first description probably goes back to the year 500 BC. In his Epidemion, Hippocrates described an endemic disease similar to Behcet`s disease.

10. Is it hereditary?

Behcet`s disease is not hereditary but there are genetic factors that may predispose a person to the condition. The features that point to a genetic aetiology are; the geographical distribution, the close association with the major histocompatibility complex allele HLA B51 and the increased risk of developing the disease in children of affected parents. It is possible for relatives of someone with Behcet`s disease to have mouth ulcers and this does not mean that they will develop Behcet`s disease in the future.

11. Is it contagious?

Some people think that because of a certain localization of symptoms (like oral and genital ulcers) that the disease might be transmitted through contact. But this is not true, it is not contagious and not infectious.

12. Is HLA B51 positivity diagnostic for Behcet`s disease?

HLA B51 is not considered as a diagnostic test for Behcet`s disease.
HLA B51 was found to be associated with Behcet`s disease in some ethnic groups but not in all patients.
Increased HLA B51 frequency was noted in the familial cases and indicates the important role of HLA B51 in the genetic tendency for Behcet`s disease.
Associations of HLA B51 with specific disease manifestations or severe course of the disease has not been confirmed.

13. Can I avoid Behcet`s disease?

There are no ways to tell that a certain person will develop Behcet`s disease, but because we know that there are genetic factors that predispose a person to the disease, the only advice that can be given to people who have somebody in the family with Behcet`s disease, is to avoid marriage between relatives.

14. Can I have children?

Again because of certain localization of the symptoms of Behcet`s disease (genital ulcers and epididimitis in males) some people think that it might affect their sexual life or the ability to have children. Some fears are also raised by patients about the effect of some medications on the fertility issue. 
To answer these questions we will mention some data:

There is no data to indicate that Behcet`s disease affects the reproductive systems of both men and women.

Data on pregnancy in female patients with Behcet`s disease showed some variation, some patients had no effect of pregnancy on the activity of the disease, while others had activity either during first, second or third trimester of pregnancy, while others had activity in the period short after delivery. Women affected with Behcet`s disease are advised to coordinate with their doctors to plan pregnancy, and it is recommended that the patient being in remission at least 6 months before getting pregnant.

Data on children born to mothers with Behcet`s disease show good outcome.

Some of the medications used to treat Behcet`s disease might affect fertility, so patients have to discuss this issue with their doctor.

15. What triggers Behcet`s disease?

There are some observations that trauma and dental extraction might exacerbate or induce symptoms of Behcet`s disease, so if you are going to the dentist or will have surgery for any reason, your doctor will take the necessary measures in order to avoid the development of any new symptoms.

16. If someone have recurrent oral ulcers, does this mean that he or she are going to develop Behcet`s disease?

It is known that recurrent oral ulcers alone can occur in about 20% of the general population. Recent study showed that 2.9% of people having recurrent oral ulcers developed Behcet`s disease within a period between 2 months-15 years after the onset of oral ulcers. It is recommended that patients who suffer from recurrent oral ulcers and who live in areas where the disease is common, should be regularly followed up.

17. Recommendations

In order to enjoy a better health and lead a normal life, patients are advised to follow these rules:

Listen to your doctor carefully and try to inform him about any changes in the way you feel.

Follow your doctor's orders regarding regular visits and regular blood tests, because while using some medications you will need to do these tests to avoid toxicity.

Check your eyes with the ophthalmologist at least every 6 months, even if you don't have eye symptoms.

If you smoke , stop smoking.

Eat healthy food, because fast food is rich with fat and might affect your vessels. If you are taking corticosteroids you will be advised to increase the intake of food rich in calcium to protect your bones, and low in calories to avoid increase in body weight.

Useful Links:

LookUs & Online Makale Home | Contact | Sitemap