Behcet's Disease - An Overview

What Is Behcet's Disease?

Human arm and hand with blood veins
Behcet's disease.. Mint Images - Frans Lanting / Getty Images

Behcet's disease was first described in 1937 by Dr. Hulusi Behcet, a dermatologist in Turkey. Behcet's disease is now recognized as a chronic condition that causes:

  • canker sores or ulcers in the mouth and on the genitals
  • inflammation in parts of the eye

In some people, the disease also results in:

  • arthritis
  • skin problems
  • inflammation of the digestive tract, brain, and spinal cord


Behcet's disease is common in the Middle East, Asia, and Japan. It is rare in the U.S. In Middle Eastern and Asian countries, the disease affects more men than women. In the U.S., the opposite is true. Behcet's disease tends to develop in people in their 20's or 30's, but people of all ages can develop this disease.


The exact cause of Behcet's disease is unknown. Most symptoms of the disease are caused by vasculitis (inflammation of the blood vessels)

Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs:

  • swelling
  • redness
  • heat
  • pain

Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues. Behcet's disease is not contagious. It is not spread from one person to another.

What Are the Symptoms of Behcet's Disease?

Two Factors

Researchers think 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 think that this susceptibility may be inherited; that is, it may be due to one or more specific genes.

  • Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people.

Symptoms Vary

Behcet's disease affects each person differently. Some people have only mild symptoms, such as canker sores or ulcers in the mouth or on the genitals. Others have more severe signs, such as meningitis (inflammation of the membranes that cover the brain and spinal cord). Meningitis can cause:

  • fever
  • a stiff neck
  • headaches

More severe symptoms usually appear months or years after a person notices the first signs of Behcet's disease. Symptoms can last for a long time or may come and go in a few weeks.


Typically, symptoms appear, disappear, and then reappear. The times when a person is having symptoms are called flares. Different symptoms may occur with each flare; the problems of the disease often do not occur together. To help the doctor diagnose Behcet's disease and monitor its course, patients may want to keep a record of which symptoms occur and when.


Since many conditions mimic Behcet's disease, doctors must observe the lesions (injuries) caused by the disorder in order to make an accurate diagnosis.

What Are the 5 Most Common Symptoms of Behcet's Disease?

5 Common Symptoms

The 5 most common symptoms of Behcet's disease are:

  • Mouth sores affect almost all patients with Behcet's disease. Individual sores or ulcers are usually identical to canker sores. They are often the first symptom that a person notices and may occur long before any other symptoms appear. The sores usually have a red border and several may appear at the same time. They may be painful and can make eating difficult. Mouth sores go away in 10 to 14 days but often come back.

  • Genital sores affect more than half with Behcet's disease and most commonly appear on the scrotum in men and vulva in women. They look similar to the mouth sores and may be painful. After several outbreaks, they may cause scarring.

  • Skin problems are a common symptom of Behcet's disease. Skin sores often look red or resemble pus-filled bumps or a bruise. The sores are red and raised and typically appear on the legs and on the upper torso.

  • Uveitis involves inflammation of the middle or back part of the eye including the iris, and occurs in more than half of all people with Behcet's disease. Uveitis is more common among men than women and typically begins within 2 years of the first symptoms.

  • Arthritis occurs in more than half of all patients with Behcet's disease. Arthritis which causes pain, swelling, and stiffness in the joints, especially in the:

  • knees
  • ankles
  • wrists
  • elbows

Arthritis that results from Behcet's disease usually lasts a few weeks and does not cause permanent damage to the joints.

What Are the Less Common Symptoms of Behcet's Disease?

Less Common Behcet's Disease Symptoms

Behcet's disease may also cause less common symptoms including:

  • Blood Clots About 16% of patients with Behcet's disease have blood clots resulting from thrombophlebitis, usually in the legs. Symptoms include pain and tenderness in the affected area. The area may also be swollen and warm. Because this can have severe complications, people should report symptoms to their doctor immediately. A few people may experience artery problems such as aneurysms.

  • Central Nervous System is affected in about 23% of all patients with the disease in the U.S. The central nervous system includes the brain and spinal cord. Behcet's disease can cause inflammation of the brain and the thin membrane that covers and protects the brain and spinal cord. This condition is called meningoencephalitis. People with meningoencephalitis may have:

  • fever
  • headache
  • stiff neck
  • difficulty coordinating movement

Patients should report any of these symptoms to their doctor immediately. If this condition is left untreated, a stroke can result.

  • Digestive Tract Rarely, Behcet's disease causes inflammation and ulceration throughout the digestive tract that are identical to the aphthous lesions in the mouth and genital area. This leads to:

  • abdominal pain
  • diarrhea and/or bleeding

Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as ulcerative colitis and Crohn's disease, careful evaluation is essential to rule out these other diseases.

How Is Behcet's Disease Diagnosed?

Difficult to Diagnose

Diagnosing Behcet's disease is difficult because no specific test can confirm it. Less than half of patients initially thought to have Behcet's disease actually have it. When a patient reports symptoms, the doctor must examine the patient and rule out other conditions with similar symptoms.

Since it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time. A patient may even visit several different kinds of doctors before the diagnosis is made.

Key Symptoms

These symptoms are key to diagnosis:

  • Mouth sores at least three times in 12 months
  • Any 2 of the following symptoms:

  • recurring genital sores
  • eye inflammation with loss of vision
  • characteristic skin lesions
  • positive pathergy (skin prick test)

In this test, the skin is pricked with a small needle; 1 to 2 days after the test, people with Behcet's disease may develop a red bump where the skin was pricked. However, only 50% of patients in Middle Eastern countries and Japan have this reaction. This reaction is even less common in United States patients.

Other Factors

Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as:

Doctors also may recommend that patients see an eye specialist to identify possible complications related to eye inflammation. To help distinguish Behcet's from other disorders, doctors may perform biopsies for lesions of the:

  • mouth
  • genitals
  • skin

How Is Behcet's Disease Treated?

Complete Treatment Plan

Since the disease affects different parts of the body, a patient probably will see several different doctors. It may be helpful for one doctor to manage the complete treatment plan. This doctor can:

  • coordinate the treatments
  • monitor any side effects from the various drugs the patient takes

Patients should tell each of their doctors about all of the drugs they are taking.

A rheumatologist often manages a patient's treatment and treats joint disease. Specialists may also treat other symptoms:

  • Gynecologist treats genital sores in women
  • Urologist treats genital sores in men
  • Dermatologist treats genital sores in men and women, and skin and mucous membrane problems
  • Ophthalmologist treats eye inflammation
  • Gastroenterologist treats digestive tract symptoms
  • Hematologist treats disorders of the blood
  • Neurologist treats central nervous system symptoms


Although there is no cure, people usually can control symptoms with:

  • proper medication
  • rest
  • exercise
  • healthy lifestyle

The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on a patient's symptoms and their severity.

Treatment Combinations

It is likely that a combination of treatments will be needed to relieve specific symptoms.

Although rest is important during flares, when the symptoms have improved, doctors usually recommend moderate exercise, such as:

  • walking
  • yoga
  • water exercise

What Medications Are Used to Treat Behcet's Disease?

Topical Drugs

Topical medications are applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe:

  • rinses
  • gels
  • ointments

Creams are used to treat skin and genital sores. These medicines may include;

  • topical corticosteroids
  • topical anti-inflammatory drugs
  • topical anesthetics

Oral Medications

Doctors also prescribe medicines taken by mouth to:

  • reduce inflammation throughout the body
  • suppress the overactive immune system
  • relieve symptoms

Doctors may prescribe one or more of the drugs described below to treat the various symptoms of Behcet's disease.

  • Corticosteroids - Prednisone is one of the corticosteroids prescribed to reduce pain and inflammation throughout the body for people with severe joint pain, skin sores, eye disease, or central nervous system symptoms.

  • Immunosuppressive Drugs (also called DMARDs (disease-modifying anti-rheumatic drugs) - These drugs (in addition to corticosteriods) help control an overactive immune system (which occurs in Behcet's), reduce inflammation, and can lessen the number of disease flares. Doctors may also use these drugs when a person has eye disease or central nervous system involvement. Depending on symptoms, these include:

Combination Therapy

In addition, a combination drug therapy can be used (sometimes cyclosporine is used with azathioprine when one alone fails or prednisone along with an immunosuppressive is used in combination).

What Is the Prognosis for a Person With Behcet's Disease?

Behcet's Disease Prognosis

Most people with Behcet's disease can lead productive lives and control symptoms with:

  • proper medicine
  • rest
  • exercise

Doctors can use many medicines to:

  • relieve pain
  • treat symptoms
  • prevent complications

When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms).

In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behcet's disease.

Behcet's Disease Research

Researchers are exploring possible genetic, bacterial, and viral causes of Behcet's disease as well as improved drug treatment.

Researchers are also investigating factors in the environment, such as bacteria or viruses, that may trigger Behcet's disease. In addition, researchers are identifying other medicines to better treat Behcet's disease.


NIH Publication No. 04-5026

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