What Can You Tell Me About Behcet’s Syndrome?

I am a 37-year-old female and have been diagnosed with Behcet’s syndrome. I had never heard of it before that time. It is very rare and very painful. The doctor I’m seeing now has only had two other Behcet’s patients. Do you know anything about Behcet’s syndrome, or how I can find any information? In three years, I went from a healthy, very active, fun person who was loving life, to now a handicapped, chronically pained person who can’t find anyone to help.

I was surprised recently to get several questions from people who have Behcet’s syndrome, since it is a rare condition — at least in this country. I have not personally seen a case that I was aware of in my own practice, and the information presented below is culled largely from a recent review article in the New England Journal of Medicine.

Behcet’s is a condition of unknown cause that is often considered to be in the category of the autoimmune diseases, diseases where a person’s immune system for some reason starts attacking the person’s tissues. It is usually not a persistent chronic disease, but rather one that comes with recurrent attacks, with periods between when one is comfortable. The disease is most common in Turkey, Saudi Arabia, and along the route of the ancient Silk Road that ran from China to Turkey. It is rare in Europe and the United States.

Behcet’s is strongly associated with a particular antigen on red blood cells called HLA-B51. This antigen is very common among Asians, but not in Westerners. Not every carrier of this antigen develops Behcet’s, however, and it is assumed that there is an infectious or environmental agent which somehow interacts with the antigen to produce the syndrome. This type of genetic/infectious interaction is thought to underlie many autoimmune diseases. Interestingly, in Japan carriers of HLA-B51 are seven times more likely to develop Behcet’s than people without the antigen, but carriers in the United States are only one and a third times more likely to develop it than those without it.

The syndrome has three common manifestations; painful aphthous ulcers of the mouth, ulcers on the genitals, and inflammation of the inner tissues of the eyes. Aphthous ulcers of the mouth, which we commonly call canker sores, are very common, most people have had them at some time, and obviously not everyone who has had a canker sore develops Behcet’s. The criteria for making the diagnosis include recurrent ulcers of the mouth plus at least two of the following: recurrent genital ulcers, inflammation in the eye, skin lesions called erythema nodosum, or a positive skin test.

The diagnosis can be tricky, as many people have oral ulcers, the genital ulcers can be mistaken for genital herpes or syphilis, and the other criteria can also be seen in different conditions. The disease can also produce arthritis, a type of meningitis or brain involvement, and sometimes intestinal symptoms.

Our writer today does not mention which manifestations of Behcet’s she has, but she is clearly in pain a lot, and all of the different components of the disease can be painful. The most worrisome manifestations are the eye inflammation, since repeated attacks can produce blindness, the meningitis, and the less common vascular or gastrointestinal problems which can be fatal.

There are a number of treatments available for Behcet’s syndrome. The oral and genital ulcers will often heal with applications of a cortisone-type cream. Special dental formulations are useful in the mouth. Colchicine, an old drug originally used to treat attacks of gout, often shortens an attack, and there is now evidence that the infamous drug thalidomide, which
was recently approved in the United States for treatment of some complications of HIV infection, may be helpful.

When the eye or brain is threatened by the disease, various drugs usually used in cancer chemotherapy are often given in addition to the steroids and colchicine. Interferon has also recently been reported to be helpful. Despite all these potentially effective treatments, nothing really cures the condition, and the recurrent attacks typically continue.

The information provided on Health Search Online is for educational purposes only and is not a substitute for medical advice, diagnosis or treatment.