What is Behcet's Disease ?

Behcet’s disease is a condition characterized by sores in the mouth and on the genitals and by inflammation in parts of the eye. The disease could also cause inflammation of joints, digestive tract, brain, and spinal cord. It is a chronic condition affecting the blood vessels because of the abnormal behavior of the body’s immune system. The immune system is typically responsible for protecting the body from diseases and infections by identifying and killing foreign substances that enter the body. In autoimmune disease, the immune system mistakenly attacks and harms the body's own tissues. Behçet's disease is not contagious and affects each person differently. There is no known cure for it and it is a lifelong recurrent condition that can be controlled through medication preventing serious complications. It is a chronic disease where the acute phases come and go with varying degrees of intensity. The disease gets its name from Hulusi Behçet (1889-1948), the Turkish dermatologist and scientist, who first recognized the syndrome in one of his patients in 1924. Because Hulusi Behçet was Turkish, the correct pronunciation of the word is "Beh-chet".

Behcet’s disease is also known as the “silk road disease” since it is commonly found along the “Old Silk Route” spanning the region from Japan and China in the Far East to the Mediterranean Sea. The disease is rare in the United States though sporadic cases have been reported. In Japan, Behcet’s disease ranks as a leading cause of blindness. It occurs most often in men between the ages of 20 and 35.

 


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Causes
The exact cause for this rare disease is not known. The presence of the gene HLA–B51 may be a likely predisposition for getting this disease. It is believed that more than one factor, including infections and other environmental exposures, play a role.

 

Symptoms
The most common symptoms are:
• Mouth sores affect almost all patients with Behçet's disease. They are often the first symptom of the disease visible to the eye. The lesions are numerous, large, painful and can make eating difficult.
• Genital sores affect more than half of the patients with the disease. They are similar to mouth sores and may be painful. In men, painful genital lesions form on the scrotum and in women, painful genital ulcers develop on the vulva.

• Inflammation inside of the eye occurs in more that half of Behçet's disease patients. It can cause blurred vision, pain, redness and can lead to blindness.
• Skin problems are common in Behcet’s disease. Painful red nodules or acne-like lesions typically appear on the legs and upper torso.
• Inflammation of the joints or arthritis occurs in more than half of Behcet’s disease patients.
• Inflammation in the central nervous system called meningoencephalitis can occur in this disease. If untreated, this can lead to stroke.
• Inflammation in the digestive organs can occur as a result of this disease. Sores identical to mouth and genital sores can occur throughout the digestive tract.

 

Diagnosis
It is not easy to diagnose Behcet’s disease. There no one single comprehensive test that can identify the disease. It is usually a case of reviewing the symptoms that a patient reports and ruling out other diseases. To diagnose Behcet’s disease, a patient must have experienced mouth sores at least three times in 12 months with any two of the following symptoms:

• Recurring genital sores
• Eye inflammation with loss of vision
• Characteristic skin lesions
• A positive pathergy test.
Pathergy test is a skin test in which the forearm is pricked with a small, sterile needle. After one or two days, if there is a small red bump at the site of the needle insertion, then the test is considered positive and the patient is likely to have the disease. But the pathergy test is not 100% accurate, not all patients have the same reaction to the test and certain other conditions can cause positive pathergy results.

Treatment
There is no cure for this disease but patients can control symptoms and prevent serious complications arising out of it. The treatment is targeted to reduce the patient’s discomfort and prevent serious complications such as blindness. An effective treatment is based on an individual’s symptoms and its severity. Usually, a combination of treatments is prescribed to relieve specific symptoms. Some of the medication used in controlling the disease includes Corticosteroids such as prednisone, Nonsteroidal anti-inflammatory drugs, and Immunosuppressant drugs. Exercise can help patients keep their joints strong and flexible. Lifestyle changes that benefit Behcet's include a nutrient-rich diet, moderate exercise, proper rest, and an avoidance of environmental chemicals.

 

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Article Contributed By: Jaya Suresh

 

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