Behcet's (beh-CHETS) disease,
also called Behcet's syndrome, is a rare disorder that causes chronic
inflammation in blood vessels throughout your body. The exact cause of
Behcet's is unknown, but it may be an autoimmune disorder, which means
the body's immune system mistakenly attacks some of its own healthy
cells. Both genetic and environmental factors may be responsible for
The inflammation of Behcet's disease leads to numerous symptoms that may
initially seem unrelated. The signs and symptoms of Behcet's disease —
which may include mouth sores, eye inflammation, skin rashes and
lesions, and genital sores — vary from person to person and may come and
go on their own.
Treatment aims to reduce the signs and symptoms of Behcet's disease and to prevent serious complications, such as blindness.
Behcet's disease symptoms vary from person to person. Behcet's disease
may disappear and recur on its own. The signs and symptoms that you may
experience depend on which parts of your body are affected by the
inflammation of Behcet's disease. Areas commonly affected by Behcet's
* Mouth. Painful mouth sores, identical to canker sores, are the
most common sign of Behcet's disease. Sores begin as raised, round
lesions in the mouth that quickly turn into painful ulcers. The sores
heal usually in seven to 21 days, though they do recur.
* Skin. Skin lesions may occur in people with Behcet's disease. Skin
problems can vary. Some people may develop acne-like sores on their
bodies. Others may develop red, raised and tender nodules on their skin,
especially on the lower legs.
* Genitals. People with Behcet's disease may develop sores on their
genitals. The sores commonly occur on the scrotum or the vulva. Sores
appear as red, ulcerated lesions. The genital sores are usually painful
and may leave scars.
* Eyes. Behcet's disease may cause inflammation in the eye — a
condition called uveitis (u-ve-I-tis). In people with Behcet's disease,
uveitis causes redness, pain and blurred vision in one or both eyes and
may come and go. Inflammation that occurs in the blood vessels of the
retina is a serious complication of the disorder.
* Joints. Joint swelling and pain often affect the knees in people
with Behcet's disease. The ankles, elbows or wrists also may be
involved. Signs and symptoms may last one to three weeks and go away on
* Vascular system. Inflammation in veins and large arteries may
occur in Behcet's disease, causing redness, pain and swelling in the
arms or legs when a blood clot results. In fact, many of the signs and
symptoms of Behcet's are believed to be caused by inflammation of the
blood vessels (vasculitis). Inflammation in the large arteries can lead
to complications, such as aneurysms and narrowing or blockage of the
* Digestive system. Behcet's disease may cause a variety of signs
and symptoms that affect the digestive system, including abdominal pain,
diarrhea or bleeding.
* Brain. Behcet's disease may cause inflammation in the brain and
nervous system that leads to headache, fever, disorientation, poor
balance or stroke.
When to see a doctor
Make an appointment with your doctor if you notice any unusual signs and
symptoms that might indicate Behcet's disease. If you've been diagnosed
with Behcet's disease, see your doctor if you notice any new signs and
Doctors don't know what causes Behcet's disease. However, a combination
of genetic and environmental factors likely plays a role. Several genes
have been found to be associated with the disease. Some researchers
believe a virus or bacterium may trigger Behcet's disease in people who
have certain genes that make them susceptible to Behcet's.
Factors that may increase your risk of Behcet's include:
* Age. Behcet's disease commonly affects men and women in their 20s
and 30s, though children and older adults also can develop the
condition. When the condition occurs at an earlier age, it tends to be
* Location. Although the disease occurs worldwide, people from
countries in the Middle East and Asia, including Turkey, Iran, Iraq,
Japan and China, are more likely to develop Behcet's.
* Sex. While Behcet's disease occurs in both men and women, the disease is usually more severe in men.
* Genes. Having certain genes is associated with a higher risk of developing Behcet's.
* Smoking. Chemicals in cigarette smoke can damage your DNA, which may increase your risk of Behcet's.
Behcet's disease typically comes and goes in unpredictable cycles.
Symptoms of the disease may become less severe after about 20 years.
Though treatment can't cure Behcet's disease, it often can control signs
and symptoms and reduce the risk of complications. For instance,
untreated uveitis can lead to decreased vision or even blindness. People
with eye signs and symptoms of Behcet's disease should be carefully
monitored by an eye doctor because treatment can help prevent this
complication. Other complications of Behcet's disease depend on the
specific set of signs and symptoms you're experiencing.
Preparing for your appointment
You're likely to start by seeing your primary care doctor if you have
signs and symptoms of Behcet's disease. But, because Behcet's disease is
rare, and some of the signs and symptoms are similar to those of other
disorders, you may need to see a specialist, such as a rheumatologist,
before getting the diagnosis of Behcet's disease.
If a rheumatologist wasn't involved in your diagnosis, you may be
referred to a rheumatologist for the management of Behcet's. Depending
on your signs and symptoms, you may also need to see an eye doctor
(ophthalmologist) for eye problems, a gynecologist or a urologist for
genital sores, a dermatologist for skin problems, a gastroenterologist
for digestive difficulties, or a neurologist for symptoms that involve
the brain or central nervous system.
Because appointments can be brief and there's often a lot of ground to
cover, it's a good idea to be prepared. Here's some information to help
you get ready, and what to expect from your doctor.
What you can do
* Write down any symptoms you're experiencing, including any that
may seem unrelated to the reason for which you scheduled the
appointment. Also, write down key personal information, including any
major stresses or recent life changes.
* Make a list of all medications, vitamins and supplements that you're taking.
* If possible, bring a family member or friend with you. Sometimes
it can be difficult to absorb all the information provided during an
appointment. Someone who accompanies you may remember something that you
missed or forgot.
* Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions
can help you make the most of your visit. List your questions from most
important to least important in case time runs out. For Behcet's, some
basic questions to ask your doctor include:
* What do you think is causing my symptoms?
* Are there any other possible causes for my symptoms?
* Do I need any special tests?
* Is this condition temporary?
* What treatments are available? Which do you recommend?
* Are there alternatives to the primary approach that you're suggesting?
* I have another health condition. How can I best manage these conditions together?
* Are there any brochures or other printed material that I can take
with me? What websites or organizations do you recommend?
In addition to the questions that you've prepared, don't hesitate to ask
questions during your appointment any time you don't understand
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to
answer them may reserve time to discuss points you want to spend more
time on. Your doctor may ask:
* What types of signs and symptoms have you been having?
* When did you begin experiencing symptoms?
* Have your symptoms been continuous or occasional?
* How severe are your symptoms?
* Does anything seem to improve your symptoms?
* What, if anything, appears to worsen your symptoms?
* Is there anyone in your family who has a similar illness?
Tests and diagnosis
No tests can determine definitively whether or not you have Behcet's
disease. Instead, your doctor relies primarily on your signs and
symptoms to diagnose Behcet's disease. Your doctor may conduct blood
tests or other laboratory tests to rule out other diseases and
Criteria have been established for the diagnosis of Behcet's disease,
but these aren't always essential for the diagnosis of the disease. Your
doctor may use other factors for your diagnosis. The classification
* Mouth sores. Because nearly everyone with Behcet's will have mouth
sores at some point, this sign is generally necessary for a diagnosis.
The diagnostic criteria require mouth sores that have recurred at least
three times in 12 months.
In addition, you must have at least two additional signs, such as:
* Genital sores. Sores that recur may indicate Behcet's disease.
* Eye problems. An ophthalmologist can identify signs of inflammation in your eyes.
* Skin sores. A variety of rashes or acne-like sores may be caused by Behcet's disease.
* Positive pathergy test. In a pathergy test, your doctor inserts a
sterile needle into your skin and then examines the area two days later.
If the pathergy test is positive, a small red bump forms under your
skin where the needle was inserted. This indicates your immune system is
overreacting to a minor injury.
Treatments and drugs
No cure exists for Behcet's disease. If your signs and symptoms of
Behcet's disease are mild, your doctor may offer medications to control
temporary flares in pain and inflammation. You may not need to take
medication between flares. But if your signs and symptoms are more
severe, your doctor may advise systemic medications to control the signs
and symptoms of Behcet's disease throughout your body, in addition to
medications for the temporary flares. Several factors, including your
age and sex, may influence the specific treatment your doctor
Treatments for individual signs and symptoms of Behcet's disease
Behcet's disease may come and go on its own in periods of flares and
remissions. Your doctor works to control any signs and symptoms you
experience during flares with medications such as:
* Skin creams, gels and ointments. Topical medicines are applied
directly to skin and genital sores in order to reduce inflammation and
pain. These types of medications usually contain a corticosteroid drug
that reduces inflammation or an anesthetic to relieve pain.
* Mouth rinses. Special mouthwashes that contain corticosteroids and
other agents to reduce the pain of mouth sores may ease your
* Eyedrops. Eyedrops containing corticosteroids or other
anti-inflammatory medicines can relieve pain and redness in your eyes if
inflammation is mild.
Systemic treatments for Behcet's disease
Severe cases of Behcet's disease require treatments to control damage
from the disease between flares. If you have moderate to severe Behcet's
disease, your doctor may prescribe:
* Corticosteroids to control inflammation. Corticosteroids, such as
prednisone, in combination with other medications may reduce the
inflammation caused by Behcet's disease. The signs and symptoms of
Behcet's disease tend to recur when corticosteroids are used alone, so
doctors often prescribe them with another medication to suppress the
activity of your immune system (immunosuppressives). Side effects of
corticosteroids include weight gain, persistent heartburn, high blood
pressure and bone thinning (osteoporosis).
* Medications that suppress your immune system. Immunosuppressive
drugs suppress your immune system, which overreacts in Behcet's disease.
By stopping your immune system from attacking normal, healthy tissues
in your body, immunosuppressive drugs reduce the inflammation that your
immune system causes. Immunosuppressive drugs that may play a role in
controlling Behcet's disease include azathioprine (Imuran, Azasan),
cyclosporine (Sandimmune) and cyclophosphamide (Cytoxan). Since these
medications suppress the actions of your immune system, they may
increase your risk of infection. Other possible side effects include
liver and kidney problems, low blood counts and high blood pressure.
* Medication that regulates your immune system. Interferon alfa-2b
(Intron A) regulates the activity of your immune system to control
inflammation. It may help control skin sores, joint pain and eye
inflammation in people with Behcet's disease. Side effects include
flu-like signs and symptoms, such as muscle pain and fatigue. Interferon
alfa-2b may be combined with other medications. More recently, small
studies have suggested that medications that block a substance called
tumor necrosis factor (TNF), such as infliximab (Remicade) and
etanercept (Enbrel), may be effective in treating some of the
manifestations of Behcet's.
Other drugs that have been used to treat Behcet's disease include colchicine and methotrexate.
Coping and support
The unpredictability of Behcet's disease can make it particularly
frustrating. Taking good care of yourself may help you better cope with
the ups and downs of Behcet's disease. Your approach to caring for
yourself will depend on what signs and symptoms you're feeling on a
particular day. In general, try to:
* Rest during flares. When signs and symptoms such as mouth sores
and joint pain appear, take time for yourself. Be flexible and adjust
your schedule when possible so that you can rest when you need it.
* Be active when you have the energy. Moderate exercise, such as
walking or swimming, can make you feel better between Behcet's disease
flares. Exercise strengthens your body and can improve your mood.
* Connect with others. Because Behcet's is a rare disorder, it may
be difficult to find others with the disease nearby. However, ask your
doctor about support groups in your area. If it's not possible to
connect with someone close, the American Behcet's Disease Association
offers message boards and chat rooms where you can connect with other
people who have Behcet's.