Behcet's disease

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at May 17, 2016
StartBehcet's disease

Behçet's disease or Behçet's syndrome has an unknown origin and is a very rare condition. It is an autoinflammatory disease that affects the blood vessels in the body and is associated with vasculitis. It is not commonly seen in the US, primarily affecting those in the Middle East and Asia.

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Definition & Facts

This condition is named after the Turkish doctor, Dr. Hulusi Behçet, who discovered the syndrome. It is referred to both as a syndrome and a disease, and Behçet's syndrome and Behçet's disease are often used interchangeably. This condition typically affects young adults between the ages of 20-30 with the highest number of cases occurring in Turkey. It is recognized worldwide, but the prevalence is higher in Middle Eastern countries.

Though its exact causes aren't fully understand, it is believed that viruses, bacteria, genetic factors, and environmental factors all play a role in this disease. It is estimated that there is about 16,000-20,000 case in the United States, or about 1 in 700,000. This disease is not contagious and there should be no fear of spreading it from one person to another.

Symptoms & Complaints

Behçet's disease affects the body with ulcers both in the mouth and genitals, as well as uveitis which is inflammation of the uvea or middle layer of the eye. The most common manifestation of Behçet's is oral ulcers. These small ulcers are painful and often have a yellow color. They do not leave any scars, but they will last anywhere from 10 to 20 days. They resemble canker sores.

Sometimes the ulcers do not appear in the mouth area but they are found in the genital area instead. These are not as common as mouth ulcers, but about 25 percent of patients will develop these painful lesions. A male will see ulcers on their scrotum but not typically on the penis. The affected female will see lesions on the vulva, vagina, and also the cervix. Genital ulcers from Behçet's typically do leave scars.

Arthritis is another condition associated with Behçet's. It can affect one or multiple joints. The joint pain and joint stiffness can be felt in the knees, wrists, elbows, and ankles. The arthritis from this syndrome will not cause permanent damage, and will usually only last until the condition's flare up subsides.

Another common issue associated with Behçet's disease syndrome is gastrointestinal lesions. About one-fourth of patients will develop these issues. Some patients who have extreme gastrointestinal issues will go on to further develop Budd-Chiari syndrome. It is important to evaluate and rule out other issues in the gastrointestinal tract before beginning treatment. Neurological symptoms may arise due to inflammation of the brain caused by Behçet's. Stroke and difficulty balancing may ensue.

Causes

Though there are many theories as to the cause of Behçet's disease, there is no exact known cause. It is understood that this disease is closely linked to inflammation in the blood vessels. An auto-inflammatory reaction can cause the blood vessels to become inflamed, creating symptoms such as swelling, redness, warmth and pain.

When the body is healthy, the immune system protects the body by killing anything that enters the body that should not. However, in Behçet's syndrome, the body attacks its own tissues. It is suspected that inherited genetic traits may play a role in these abnormalities of the immune system. It is also believed that exposure to certain types of bacteria or virus may activate the disease.

Diagnosis & Tests

Diagnosing Behçet's is often difficult. There is no definitive test that proves or denies the presence of the condition. A person suffering with this condition can battle for years before receiving a diagnosis. A doctor will begin testing to rule out other conditions. Since Crohn's disease is so common, it is one of the first to be ruled out.

Some people seek medical help thinking they have a blood clot, but the culprit is inflammation caused by a flare-up of Behçet's. Symptoms can come and go with periods of remission; they may be difficult to track. A doctor typically needs to see mouth sores for at least the past 12 months before rendering a diagnosis. During a flare up, diagnosis is easier to render than when the condition is in a dormant state.

Treatment & Therapy

Because Behçet's is an autoimmune disease that has no cure, only the symptoms can be treated. Pain relievers can help with arthritis, as well as general pain associated with ulcers. If the pain becomes too great, other medications with long-lasting pain control may be used. The goal is to reduce discomfort to allow a person to get through the painful flare-up. Medications that doctors may prescribe to treat Behçet's include corticosteroids, immunosuppressive drugs, and biological therapies. These drugs aim to reduce inflammation.

There can be complications that affect various organ systems, so each case is analyzed and proper treatments given. It is often the case that many doctors need to be involved, as this condition affects so many different parts of the body. When there are skin issues, a dermatologist is needed. A female with ulcers in the vaginal area will require the expertise of a gynecologist. If the kidneys should become affected, a nephrologist may be consulted. There is no cure for this condition, but a team of doctors working together will bring about the best outcome for their patient.

Prevention & Prophylaxis

Due to the fact that Behçet's disease is autoimmune based, there is really no way to prevent it. If there is an area on the body that is causing warm spots and redness that resembles that of a blood clot, it is important to have it checked. What may appear as a blood clot may be Behçet's, and the earlier it can be caught, the better the prognosis.