- Behçet's Syndrome Center
- Rheumatoid Arthritis Slideshow Pictures
- Joint-Friendly Exercises to Reduce RA Pain Slideshow
- Take the RA Quiz
- Patient Comments: Behcet's Syndrome - Symptoms
- Patient Comments: Behcet's Syndrome - Effective Treatments
- Patient Comments: Behcet's Syndrome - Experience
- Patient Comments: Behcet's Syndrome - Diagnosis
Behçet's syndrome facts
- Behçet's syndrome is associated with inflammation of various areas of the body.
- Symptoms of Behçet's syndrome depend on the body areas affected including:
- Recurrent mouth ulcers are characteristic of Behçet's syndrome.
- Treatment of Behçet's syndrome depends on the severity and the location of its manifestations.
What is Behçet's syndrome?
Behçet's syndrome is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (aphthous ulcers, canker sores), genital ulcers, and inflammation of a specialized area around the pupil of the eye termed the uvea. The inflammation of the area of the eye that is around the pupil is called uveitis. Behçet's syndrome is also sometimes referred to as Behçet's disease.
What causes and contagious of Behçet's syndrome?
The cause of Behçet's syndrome is not known. The disease is relatively rare but is more frequent and severe in patients from the Eastern Mediterranean and Asia than those of European descent. Both inherited (genetic) and environmental factors, such as microbial infections, are suspected to be factors that contribute to the development of Behçet's. Behçet's is not known to be contagious.
What are symptoms of Behçet's syndrome?
The symptoms of Behçet's syndrome depend on the area of the body affected. Behçet's syndrome can involve inflammation of many areas of the body. These areas include the arteries that supply blood to the body's tissues. Behçet's syndrome can also affect the veins that take the blood back to the lungs to replenish oxygen. Other areas of body that can be affected by the inflammation of Behçet's syndrome include the back of the eyes (retina), brain, joints, skin, and bowels.
The mouth and genital ulcers of Behçet's syndrome are generally painful and tend to recur in crops (many shallow ulcers occur at the same time). They range in size from a few millimeters to 20 millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner lining of the mouth. The genital ulcers occur on the scrotum and penis of males and vulva of women and can leavescars.
Inflammation of the eye, which can involve the front of the eye (uvea) causing uveitis, or the back of the eye (retina) causing retinitis, can lead to blindness. Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights. It is very important for patients with Behçet's syndrome to have this sensitive area monitored by an eye specialist (ophthalmologist).
If the arteries become inflamed (arteritis) from Behçet's syndrome, it can lead to death of the tissues whose oxygen supply depends on these vessels. This could cause a stroke if it is affecting the brain vessels, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis), the inflammation can involve large veins that develop blood clots which can loosen and migrate to cause pulmonary embolisms.
Symptoms of inflammation of the brain or tissue that covers the brain (meninges) include headaches, neck stiffness, and is often associated with fever. Inflammation of the brain (encephalitis) and/or the meninges (meningitis) can cause damage to nervous tissue and lead to weakness or impaired function of portions of the body. This can result in confusion and coma. Typically these features occur later in the disease course, years after the diagnosis.
Joint inflammation (arthritis) can lead to swelling, stiffness, warmth, pain, and tenderness of joints with Behçet's syndrome. This occurs in about half of patients with Behçet's syndrome at sometime during their lives. Knees, wrists, ankles, and elbows are the most common joints affected. The skin of patients with Behçet's syndrome can develop areas of inflammation that spontaneously appear as raised, tender, reddish nodules (erythema nodosum), typically on the front of the legs. Some patients with Behçet's syndrome develop a peculiar red or blistery skin reaction in places where they have been pierced by blood-drawing needles (see pathergy test in diagnosis section). Research has found that acne occurs more frequently in patients with Behçet's syndrome that also have arthritis as a manifestation.
Ulcerations can occur at any location in the stomach, large or small bowel in patients with Behçet's disease.
How is Behçet's syndrome diagnosed?
Behçet's syndrome is diagnosed based on the finding of recurrent mouth ulcerations combined with any two of the following: eye inflammation, genital ulcerations, or skin abnormalities mentioned above.
A special skin test called a pathergy test can also suggest Behçet's syndrome. (The other criteria above are still required for ultimate diagnosis.) This test consists of pricking the skin of the forearm with a sterile needle. The test is called positive and suggests Behçet's syndrome when the puncture causes a sterile red nodule or pustule that is greater than two millimeters in diameter at 24 to 48 hours after the test.
What is the treatment of Behçet's syndrome?
The treatment of Behçet's syndrome depends on the severity and the location of its manifestations in an individual patient.
Steroid (cortisone) gels, pastes (such as Kenolog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine (Colcrys) can also minimize recurrent ulcerations. Trental (pentoxifylline) has also been used in the treatment of oral and genital ulcers.
Joint inflammation can require nonsteroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine (Azulfidine) has been effective in some patients for arthritis. Bowel disease is treated with oral steroids and sulfasalazine.
Diligent treatment of eye inflammation is essential. Patients with eye symptoms or a history of eye inflammation should be monitored by an ophthalmologist. Resistant eye inflammation can often respond to new biologic medications that block a protein that plays a major role in initiating inflammation, called TNF. These TNF-blocking medications, including infliximab (Remicade) and adalimumab (Humira), can also be helpful for severe mouth ulcerations.
Severe disease of the arteries, eyes, and brain can be difficult to treat and require powerful medications that suppress the immune system called immunosuppressive agents. Immunosuppressive agents used for severe Behçet's syndrome include chlorambucil (Leukeran), azathioprine (Imuran), and cyclophosphamide (Cytoxan). Cyclosporine has been used for resistant disease.
Studies suggest that thalidomide (Thalomid) may be of benefit for certain patients with Behçet's syndrome in treating and preventing ulcerations of the mouth and genitals. Side effects of thalidomide include promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation. Trials are currently underway evaluating interferon alpha for the treatment of eye disease in patients with Behçet's syndrome.
Latest Arthritis News
Health Solutions From Our Sponsors
American College of Rheumatology, Annual Scientific Meeting; November, 2006, 2007.
Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Behcet's Syndrome - Symptoms
What were the symptoms of your Behcet's syndrome?Post View 25 Comments
Behcet's Syndrome - Effective Treatments
What kinds of treatment, including medication, have you found effective for Behcet's syndrome?Post View 6 Comments
Behcet's Syndrome - Experience
Please discuss your experience with Behcet's syndrome.Post View 10 Comments
Behcet's Syndrome - Diagnosis
How was the diagnosis of your Behcet's syndrome made?Post View 2 Comments
Top Behcet's Syndrome Related Articles
AcneAcne is a localized skin inflammation as a result of overactivity of oil glands at the base of hair follicles. This inflammation, depending on its location, can take the form of a superficial pustule (contains pus), a pimple, a deeper cyst, congested pores, whiteheads, or blackheads. Treatments vary depending on the severity of the acne.
Canker SoresCanker sores are a common complaint, and are small ulcers on the inside of the mouth. Canker sores aren't contagious (as opposed to cold sores), and typically last for 10-14 days usually healing without scarring. A variety of things cause canker sores, for example, medications (aspirin, beta-blockers, NSAIDs, high blood pressure medication, and antibiotics); injury to the mouth from dental work, braces, or sports accidents; acidic foods; allergies; and diseases or conditions like celiac disease, Crohn's disease, and lupus. Canker sores can be cure with home remedies, and prescription and OTC topical and oral medication.
IV Drug Infusion FAQsMany diseases may require IV drug infusion, including:
- Ankylosing spondylitis,
- Behcet's disease,
- Crohn's disease,
- juvenile rheumatoid arthritis,
- multiple sclerosis,
- cancer, and
Juvenile ArthritisJuvenile idiopathic arthritis (juvenile rheumatoid arthritis or JRA) annually affects one child in every thousand. There are six types of JIA. Treatment of juvenile arthritis depends upon the type the child has and should focus on treating the symptoms that manifest.
Lumbar PunctureA lumbar puncture or "LP" is a procedure whereby spinal fluid is removed from the spinal canal for the purpose of diagnostic testing. It is particularly helpful in the diagnosis of inflammatory diseases of the central nervous system, especially infections, such as meningitis. A lumbar puncture is also known as a spinal tap.
MRI ScanMRI (or magnetic resonance imaging) scan is a radiology technique which uses magnetism, radio waves, and a computer to produce images of body structures. MRI scanning is painless and does not involve X-ray radiation. Patients with heart pacemakers, metal implants, or metal chips or clips in or around the eyes cannot be scanned with MRI because of the effect of the magnet.
Sexually Transmitted Diseases (STDs In Women)
Sexually transmitted diseases (STDs) are among the most common infectious diseases in the United States. STDs can be spread through any type of sexual activity involving the sex organs, the anus or mouth, or through contact with blood during sexual activity. Examples of STDs include, chancroid, chlamydia, gonorrhea, granuloma inguinale, lymphogranuloma venereum, syphilis, genital herpes, genital warts, hepatitis B, trichomoniasis, pubic lice (crabs), and scabies.Treatment is generally with antibiotics; however, some STDs that go untreated can lead to death.
Skin BiopsyDuring a skin biopsy, a piece of skin is removed under a local anesthesia and examined using a microscope. There are different types of skin biopsy:
- shave biopsy,
- punch biopsy,
- and excisional biopsy.
Stroke Symptoms and Treatment
A stroke is an interruption of the blood supply to part of the brain caused by either a blood clot (ischemic) or bleeding (hemorrhagic).
Symptoms of a stroke may include:
- double vision or vision loss,
- difficulty speaking or understanding speech.
A physical exam, imaging tests, neurological exam, and blood tests may be used to diagnose a stroke. Treatment may include administration of clot-busting drugs, supportive care, and in some instances, neurosurgery. The risk of stroke can be reduced by controlling high blood pressure, high cholesterol, diabetes, and stopping smoking.
Uveitis PictureUveitis (pronounced you-vee-EYE-tis) involves all inflammatory processes of the middle layers of the eye, also called the uveal tract or uvea. See a picture of Uveitis and learn more about the health topic.
VasculitisVasculitis (arteritis, angiitis) is a general term for a group of uncommon diseases which feature inflammation of the blood vessels. Each form of vasculitis has its own characteristic pattern of symptoms. The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.