Behcet'S Disease

1 What is Behcet's Disease?

Behcet's (beh-CHETS) disease, also called Behcet's syndrome, is a rare but chronic disorder characterized by a symptom triad of recurring oral aphthous ulcers, genital ulcers, and uveitis.

It also causes vasculitis, which is inflammation of the blood vessels, and causes problems in various parts of your body.

This disease can give rise to numerous signs and symptoms that may seem unrelated initially.

In some cases, it may cause inflammation in eyes, digestive tract, brain and spinal cord, skin rashes and lesions, and arthritis.

The symptoms of Behcet's disease may differ from one person to another and these may resolve on their own.

The treatment includes medications to manage the symptoms of Behcet's disease and to prevent serious complications such as blindness.

2 Symptoms

The symptoms of Behcet's disease vary from one individual to the other. These may disappear and recur on their own.

Over a period of time, the severity of symptoms may reduce. Your signs and symptoms depend on the parts of your body that have been affected.

The parts commonly affected by Behcet's disease include:

Mouth: Painful oral ulcers that appear similar to canker sores are one of the most common signs of Behcet's disease.

These are often the first symptoms to be noticed, and these sores initially appear as raised, round lesions with a red border.

Several lesions may occur at the same time, which rapidly turn into painful ulcers. These sores can heal within one to three weeks, though they recur after some time.

Skin: Skin problems may vary. While some people may develop acne-like sores, others develop red, raised, pus-filled, tender bumps on their skin, especially on the lower legs and on the upper torso.

In few people, sores or lesions may occur when the skin is either scratched or pricked.

Genital area: Red, open sores most commonly appear on the scrotum or the vulva.

These sores are similar to oral sore and are usually painful. These may leave scars after several outbreaks.

Eyes: Behcet's disease may cause inflammation in the middle or back portion of the eye (the uvea) including the iris, a condition called uveitis (u-vee-I-is).

Uveitis can result in blurred vision, and rarely it may cause pain and redness in one or both eyes.

Joints: Inflammation of the joints is called arthritis. This condition causes joint swelling, pain, and stiffness in the joints such as the knees, ankles, wrists, and elbows.

These symptoms last for a few weeks and resolve on their own without causing any permanent damage to the joints.

Vascular system: Inflammation in the veins (thrombophlebitis)of the legs may result in the formation of blood clots.

This causes symptoms such as pain, redness, swelling in the arms or legs.

Inflammation in the large arteries can lead to complications such as aneurysms (balloon-like swelling of the artery wall) and narrowing or blockage of the vessel.

Digestive system: Behcet's disease may cause inflammation and ulceration (sores) similar to oral aphthous lesions throughout the digestive tract.

This causes a variety of signs and symptoms such as abdominal pain, diarrhea, and/or bleeding.

Central nervous system: Behcet's disease may cause meningoencephalitis, an inflammation of the brain and the thin membrane covering the brain and spinal cord.

This results in symptoms such as fever, headache, difficulty in coordinating movement, stiffness in the neck, and disorientation.

Consult your doctor immediately if you note any unusual signs and symptoms that might indicate Behcet's disease.

If you have already been diagnosed with Behcet's disease, seek medical care if you notice any new signs and symptoms.

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3 Causes

The exact cause of Behcet’s disease is still not known. Most of the symptoms of this disease occur due to inflammation of the blood vessels.

Doctors believe that the blood vessels become inflamed because of an autoinflammatory reaction, but the trigger for this reaction is not known.

In such reaction, the immune system attacks and harms the body’s own tissues.

Behçet’s disease is not a contagious disease, which means it does not spread from one person to the other.

Two factors have been identified to play an important role in causing Behçet’s disease, which includes:

Abnormalities of the immune system: This makes you susceptible to Behcet's disease, and this susceptibility may be inherited due to one or more specific genes.

Environmental factors: A bacterium or virus may trigger or activate Behcet's disease in susceptible people.

4 Making a Diagnosis

Making a diagnosis of Behcet's disease is done after your primary doctor's evaluation.

After evaluation, you may be referred to a doctor who treats arthritis and other rheumatic illnesses (rheumatologist) to confirm a diagnosis of Behcet's disease.

Depending on your signs and symptoms, you may also need to consult an eye doctor (ophthalmologist) if you have eye problems, a gynecologist or a urologist for genital sores, a dermatologist for skin problems, a gastroenterologist for digestive problems, or a neurologist for symptoms that involve the central nervous system.

As appointments can be brief and there is often a lot of information to discuss, it is better to be prepared. Here is some information that can help you get ready for your appointment.

What you can make a list of the following:

  • Symptoms you are experiencing, including those that seem unrelated to the reason for your appointment
  • Key personal information, including major stresses and recent life, changes
  • All your regular medications, vitamins, and supplements, including their doses

Questions to ask your doctor. For Behcet's disease, some basic questions you may ask your doctor include:

  • What is the most likely cause of my symptoms?
  • What are other possible causes of my symptoms?
  • Which tests do I need to undergo? Do these require any special preparation?
  • Is my condition temporary or chronic?
  • What treatment options are available, and which one do you recommend?
  • How can I best manage my other medical conditions?

Your doctor may ask you a number of questions, such as:

  • Which type of signs and symptoms have you been having and when did you notice these first?
  • Are your symptoms long-lasting or do they come and go?
  • How severe are your symptoms?
  • Is there anything that seems to improve or worsen your symptoms?
  • Does anyone in your family have this illness?

Diagnosis:

There are no definitive diagnostic tests available to determine whether you have Behcet's disease or not.

Your doctor makes a diagnosis based on your signs and symptoms. Certain blood tests or other laboratory tests may be performed to rule out other conditions.

Diagnostic criteria have been established for Behcet's disease, but these are not always essential for the diagnosis of the disease.

The following symptoms are a key to the diagnosis of Behcet's disease:

Mouth sores: The diagnostic criteria requires mouth sores to have recurred at least three times in a year.
In addition, to derive a diagnosis of Behcet's disease there must be any two additional signs, such as:

  • Recurrent genital sores
  • Inflammation of eyes with loss of vision
  • Skin sores, rashes or acne-like sores
  • Positive pathergy test (skin prick test): In a pathergy test, your doctor will insert a sterile needle into your skin and then examine the area after one to two days. If the pathergy test is positive, a small red bump develops under your skin at the site of needle insertion. This indicates that your immune system is overreacting to a minor injury.

5 Treatment

Although there is no specific treatment for Behcet's disease, proper medication, rest, exercise, and a healthy lifestyle can control your symptoms.

The objective of treatment is relief of discomfort and prevention of complications. The type of medications and the duration of treatment depends on your symptoms and their severity.

If you have a mild form of the condition, your doctor may prescribe medicines to control temporary flares in pain and inflammation. You may not take medications in between your flares.

If your signs and symptoms are more severe, your doctor may advise medications to control the signs and symptoms of Behcet's disease throughout your body, in addition to medications for the temporary flares.

Treatment for individual signs and symptoms of Behcet's disease:

Your doctor prescribes medications to control any signs and symptoms that occur during flares. These includes:

Skin creams, gels, and ointments: Topical corticosteroid medicines are applied directly to the skin and genital sores in order to relieve pain and reduce inflammation.

Mouth rinses: Special mouthwashes containing corticosteroids and other agents decrease the pain caused by mouth sores.

Eyedrops: Eyedrops containing corticosteroids or other anti-inflammatory medicines relieve pain and redness in a case of mild inflammation in your eyes.

Systemic treatment for Behcet's disease:

If topical medications are not effective, your doctor may recommend a drug called colchicine (Colcrys). Symptoms of arthritis symptoms may also improve with colchicine.

Severe cases of Behcet's disease require treatments to manage the symptoms that occur in between flares.

If you have moderate to severe Behcet's disease, your doctor may prescribe:

Corticosteroids to control inflammation: Corticosteroids such as prednisone may control the inflammation caused by Behcet's disease.

The signs and symptoms of Behcet's disease may recur when corticosteroids are used alone, therefore, doctors often prescribe corticosteroids with another medication that suppresses the activity of your immune system (immunosuppressive drugs).

Medications that suppress your immune system: Immunosuppressive drugs reduce the inflammation by preventing an attack on healthy tissues by your immune system.

Immunosuppressive drugs that are important in controlling Behcet's disease include azathioprine (Imuran, Azasan), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide (Cytoxan).

As these medications suppress the actions of your immune system, the risk of infection may be high.

Medications that alter your immune system's response: Interferon alfa-2b (Intron A) controls inflammation by regulating the activity of your immune system.

It may be used alone or with other drugs to help control skin sores, joint pain, and eye inflammation in people with Behcet's disease.

Medications that block a substance called tumor necrosis factor (TNF) are effective in treating more severe or resistant signs and symptoms of Behcet's disease.

Examples include infliximab (Remicade) and etanercept (Enbrel).

6 Lifestyle and Coping

The unpredictable nature of Behcet's disease can make it very frustrating, but taking good care of yourself may help you cope up with its symptoms better.

Your approach to self-care depends on the symptoms you are feeling on a particular day. In general, try to:

Take rest during flares: When symptoms appear, take time for yourself. Be flexible and adjust your schedule when possible so that you have time to rest when you need to.

Avoid getting stressed as stress can further aggravate your symptoms.

Try to be active when you have the energy: Moderate exercises such as walking or swimming can make you feel better between Behcet's disease flares.

Exercise improves the flexibility of your joints, strengthens your body, and enhances your mood.

Connect with others: As Behcet's disease is a rare disorder, you may find it difficult to connect with other people with Behcet's disease in your neighborhood. Ask your doctor about support groups in your area.

If it is 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 disease.

7 Risks and Complications

The factors that can increase your risk of developing Behcet's disease include:

Age: Behcet's disease equally affects men and women in their 20s and 30s. Children and older adults may also develop the condition.

Place or region where you live: People residing in the Middle East and the Far East, including Turkey, Iran, Japan and China, are more likely to develop Behcet's disease.

Gender: While Behcet's disease affects both men and women, the disease is more severe in men.

Genes: Presence of certain genes increases your susceptibility to Behcet's disease.

Complications of Behcet's disease depend on your signs and symptoms. This include:

  • Vision disturbances or even blindness in cases of untreated uveitis
  • Aneurysm
  • Vasculitis may lead to ischemia
  • Neurological involvement leads to progressive disease or permanent deficits or even death
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