Behcet Syndrome

Behcet’s syndrome is a unique disease that is identified by the presence of the classic three symptoms, i.e., aphthous ulcers or mouth ulcers which occur in little clusters, genital ulcers, and uveitisor inflammation of a particular area around the eye’s pupil. Pathergy test, skin biopsy, and MRI scan can also confirm this syndrome.

So far, the actual cause for Behcet’s syndrome has not been identified. This syndrome is more prevalent in people from the East Mediterranean and the Asian regions as compared to those from other areas like Europe. It is believed that the disease is not contagious. It is suspected that genetic causes as well as environmental factors like infection by pathogens can lead to the development of Behcet’ssyndrome.

Symptoms of Behcet’s syndrome

The following are some of the signs and symptoms of Behcet’s syndrome:

• The symptoms are dependent on the specific section of body that gets affected. It causes inflammation on many parts of the body.
• Inflammation of arteries which carries blood to the tissues present all over the body.
• Inflammation of veins that take blood to lungs to get oxygen
• Inflammation of retina, brain,skin,joints, and bowels
• Painful shallow cluster of ulcers occur in the mouth; it can affect the gums, tongue, and inner lining of the mouth.
• Genital ulcers that occur on penis and the scrotum in males and on vulva in women can lead to scarring.

Inflammation of the eye: The inflammation may occur on back side of the eye (retina)and cause retinitis which may result in blindness. It can also affect the front portion of the eye (uvea) causing uveitis. The following are some of the symptoms of ocular inflammation:

• Blurred vision
• Pain in the eyes
• Redness of the eyes
• Excess tearing from the eyes
• Pain in eyes whenever bright lights are directed towards them

It is essential to monitor the affected eye by an ophthalmologist.

The signs of inflammation of brain or the covering tissue, i.e., meninges,are fever, stiffness of neck, and headaches. Encephalitisor meningitis is caused due to brain inflammation.It may damage the nervous system resulting in weakness and failure of portions of the body. After about a period of one year from diagnosis, it may cause disorientation and even coma.

Patients with Behcet’s syndrome can suffer from skin inflammation that leads to raised skin, tender skin, and reddish nodules or erythema nodosum, mostly on the front part of legs. Some patients may experience redness and blisters on areas where the skin has been punctured with a needle for drawingblood. Research has shown that the occurrence of acne is common in Behcet’s syndrome patients who also suffer from arthritis.

If inflammation of arteries or arteritis occurs, it may cause the decay of tissues to which the affected arteries carry the required oxygen. If it affects the brain, then it may result in stroke. If it affects the belly it may cause stomach pain. The inflammation of veins or phlebitis leads to blood clots and can result in pulmonary embolism.

The signs of joint inflammation due to Behcet’s syndromeare swelling of joints, pain, feeling warmth on joints, stiffness and tenderness in joints. Wrists, ankles, elbows and knees are the joints commonly affected by Behcet’s syndrome.

Ulcers in stomach can occur anywhere in the small or large intestinal portions for persons suffering from Behcet’s syndrome.

Treatment of Behcet’s syndrome

The treatment of Behcet’s syndrome depends upon its type, location on body, and its level of manifestation. The following are some of the treatments provided as per the accompanying symptoms:

• For oral and genital ulcers:Cortisone steroid gels, orabase kenolog pastes, and creams are used. Colchicine minimizes the recurrence of ulcers. Trental helps maintain the healed wounds for about 29 months. It was observed that a combination of Trental with colchicines enhance the effectiveness on some patients.
• Eye inflammation: Biologic medications such as infliximab and etanercept are used to treat resistant ocular inflammation. These are helpful in controlling severe mouth ulcers as well. Patients with a history of eye inflammations need to be monitored by an eye specialist.
• Joint inflammation:Non-steroidal anti-inflammatory drugs such as ibuprofen or oral steroids are prescribed. For eye, joints, brain and skin inflammation, oral and intravenous cortisone as well as colchicines are given. In some arthritic patients, sulfasalazine/ azulfidine was found to be quite effective.
• Severe abnormalities of the eyes, brain, and arteries:    They need stronger medicines which can suppress the immune system, i.e., immunosuppressive agents. For severe Behcet’s syndrome, immunosuppressants such as, chlorambucil, cyclophosphamide, and azathioprine are used. For resistant diseases, Cyclosporine is administered.
• Bowel diseases:Sulfasalazine and oral steroids are recommended

Recent research indicates that Behcet’s syndromepatients who have had heart valve replacement, can benefit from the use of immune suppressive prednisone and azathioprine, post operation.

Recent research has also shown that use of thalidomide has benefited some patients in controlling ulcers of the mouth and genitals. But support of abnormal fetal growth, hypersedation, and nerve injury or neuropathy are some of the side effects of this medicine.

 Behcet Syndrome – Pictures