Here is more information on Behçet's Syndrome:
In 1937 Behçet described a syndrome characterized by a set of three symptoms:
Aphthous stomatitis which simply means canker sores, genital ulcers, and uveitis, a disease of the eye. Since Behcet's description, there have been additional articles regarding the illness, with clinical descriptions involving almost the entire system. The disease is therefore a chronic multi- system one with spontaneous remissions and relapses similar to those of various other autoimmune diseases.
Characteristically, a course of active disease
attacks lasts a few weeks each, with intervals of months to years between
them. Familial occurrence has been reported and it seems to occur
in patients from eastern Mediterranean countries and Japan. The disease
appears to be linked to HLA-B5 and HLADR5 alleles. The disease's management
may involve rheumatologists, gastroenterologists, neurologists, and immunologists.
It is neccessary that these doctors all work closely together to provide
the best care for the patient with Behçet's
Behçet's disease is not infectious, contagious, nor sexually transmitted. It is a chronic, multisystem autoimmune disease that involves inflammation of blood vessels throughout the body. It is rare, with an occurrence of one case per 20,000 in the United States, but the disease is found worldwide, and is most common in the Eastern Mediterranean countries and in eastern Asia. In those countries, it is a leading cause of blindness. It is not a leading cause of blindness in the Western world.
Onset of the disease is usually between the
ages of 20 and 30, although some patients have been as young as 4 and as
old as 70. Three cases of Behçet's disease were described among
newborns born to mothers with disease. The transmission of the disease
from mother to newborn might be caused by a specific antibody entering
the fetus through the placenta. The literature suggests that the clinical
picture of Behçet's syndrome in children differs from that in adults,
in that there is a lower frequency of eye disease and unusual manifestations
appear to be more
common. Behçet's disease affects twice as many men as women.
Because Behçet's disease is rare and the symptoms of this disease overlap symptoms of other diseases, it can be very difficult to diagnose. Spontaneous remission is common for patients with Behçet's disease; this can add to the difficulty in diagnosis. The most Common Symptoms and Signs of Behcet's Disease are:
Sores in the Mouth
These are painful canker sores that can overlap and take days or weeks to heal. Look for white or yellow centers, with a red edge. They are especially painful on the tongue, but can be found anywhere inside the mouth. Gum lesions are not very common, but do occur. If they predominate, then consider another diagnosis. These sort of ulcers are quite common amongst perfectly healthy people and they have to be linked with some other symptoms to be part of Behcet's Syndrome.
There is usually some painless episodes of vision blurring, seldom with much eye inflammation. Any pain or aching inside the eyes or inflammation or redness round the iris (the coloured circle round the black pupil) should be looked into. Upon diagnosis, it is of particular importance that a prompt referral to an ophthalmologist be given. The doctor should ask for an ocular slit-lamp assessment, as eye involvement can be severe and therapy needs to
be started early in the course of the illness. Ophthalmologists see cells in the anterior or posterior chamber, or signs of retinal vasculitis
These are not herpes, therefore they do not blister and they are virus negative. Genital recurrence is much less frequent than oral. Ulcers can be found on the scrotum, penile shaft or glans. In women, they are found on the labium minor, the cervix, or vaginal wall. Ulcers may be painless in the last two sites.
Skin lesions are often neglected as they are usually painless and relegated to a minor role by the patient and Physician alike. Look for 5-10mm papules and pustules on the upper trunk, thighs, and buttocks. These generally take the form of painful raised red lumps, rashes, septic spots, or patches like acne. They can occur in places where skin gets rubbed or pressed on regularly or as a result of injections, needle pricks or other damage ('pathergy'). Ulcers or sores can occur almost anywhere on the surface of the body. Sometimes there can be painful lumps just under the skin, and poor circulation.
These are either spells of swollen and inflamed joints with aching in many of the joints, or chronic aches and pains in joints. This arthritis (inflammation of joints) or arthalgia (pain in joints) is not crippling or deforming even after years of having it. Joint involvement may reach 65% of the patients with arthralgia, mostly of the knee. These features should not be ignored
These can be recurrent bouts of diarrhea or constipation or bloating, wind or pain in the lower abdomen for no apparent reason or passing small amounts of blood or mucus.
As with any major chronic illness, it is characteristic of chronic diseases to make you feel depressed. You can also feel lethargic and exhausted.
Hearing loss, tinnitus, dizziness, balance problems may be involved.
Also there may be wheezing, breathlessness, pain, coughing up blood.
This is the most controversial criterion. It has not been well studied and has been described variously. Near Eastern clinicians depend heavily on it. Pathergy, by the new rules, must have a red lesion, i.e., a papule or pustule greater than 2mm at the prick site. Its prevalence varies by geographic region, from 17% in Northern Europeans to 70% in Iranians. Pathergy is an exaggerated skin reaction to scratches, needle prick or intradermal saline injection and appears to be typical of the disease.
There may be problems with the veins of some patients usually diagnosed as thrombophlebitis and arterial aneurysms (4%) seen in some patients .Clots (thrombosis); in surface veins (phlebitis) with hot, tender veins, flushing and swelling of the affected limb, or in deep veins ( deep vein thrombosis - DVT ) with deep throbbing pain and swelling in the affected limb. It can also occur in the eyes, lungs and brain. These are not the same sort of clot as the ones that cause heart attacks.
Central Nervous System
In small groups of people several other clinical features were seen. For instance, central nervous system (CNS) involvement was seen in only 10% of the sample of 877 patients whose data was analyzed. The vast majority of patients in this sample were from countries with a high prevalence of Behçet's Disease, i.e., Iran, Turkey, and other Near Eastern or Mediterranean Basin countries. But, CNS involvement approaches 30% in North American patients. The nervous system may be involved and the following list of symptoms should be reported to the doctor if they occur:
A. Any spells of inability to focus or double vision.
B. Any unusual difficulty in walking or balancing.
C. Any unusual dizziness or fainting
D. Any numbness, pins and needles, paralysis or weakness for no apparent reason.
E. Any unusual spells of confusion.
F. Headache especially if with stiff neck.
There are also some Gastrointestinal lesions (10%), and symptoms resembling colic, nausea, vomiting, diarrhea and anorexia. Other patients have ulcerative colitis-like or Crohn's disease-like symptoms. On X-ray using a barium exam, the findings may mimic acute Crohn's disease with aphthous ulcers, collar button ulcers, or a granular appearance to mucosa. The GI involvement can range from a mild inflammation of the rectum to include the total colon and the esophagus. Complications may include perforation, a fistula formation and hemorrhage.
Treatment is symptomatic and empirical. In cases of skin involvement, vasculitis or the eye disease, uveitis, steroids are usually used. However in cases of posterior uveitis, CNS involvement or arteritis, a combination of steroids and chlorambucil is recommended. Indomethacin has been tried and reduced the severity of joint inflammation and pustular skin lesion but controlled studies are needed.
Thalidomide has been reported to be of value in treatment of Behçet's syndrome and in one case it was found to have a dramatic effect on a severe colitis. There are early reports of the beneficial use of cyclosporine A in uveitis.
The American Behçet's Disease Association
has a Home Page listed at: http://pweb.netcom.com/~mharting