Behcets Syndrome

Behçet's disease

Behcet's syndrome is an uncommon disease that affects the blood vessels throughout the body causing chronic inflammation. [1] The disease is a classical triad of recurring mouth ulcers, inflammation of an area around the pupil called uvea (uveitis) and ulcers in the sex organ. [2] This triad is first described by Hulusi Behçet, a Turkish dermatologist to whom the disease is named after. [3]

Epidemiology

The disease is common in the Middle East, Asia and Japan where more males develop the disease than females. In United States, Behcet's syndrome is rare and affects females more. [4] Individuals commonly affected are those in their 20s and 30s. [5]

Causes

The exact cause of Behcet's syndrome is still unidentified. It is proposed to be an autoimmune disease in which the body's immune system mistakenly attacks its own. [1] The consequence is damage to blood vessels. [4] Other factors such as genetics and environment like infections are thought to play a role in its development. [2]

Signs and Symptoms

Most commonly found aside from the triad of uveitis, mouth and genital ulcers are skin sores and pain, swelling and stiffness of joints. [5]

Both mouth and genital ulcers may range from few millimetres to 20 millimetres in diameter.

Mouth ulcers are commonly found on the gums, tongue and mouth's inner lining. Genital ulcers are usually seen in the scrotum and penis of males and in vulva among females. [2]

Uveitis may occur anteriorly or posteriorly. Anterior uveitis is associated with pain, blurred vision, watery eyes, eye redness and sensitivity to light. Posterior uveitis, although causes less symptoms, is more dangerous with potential loss of sight because of retinal damage.[4]

Lesions can also be found in the gastrointestinal tract and the central nervous system may also be involved. [3] When the brain is affected, the patient may have headache, personality changes and dementia. In some cases, the lungs may be involved which could lead to haemorrhage. [4]

Diagnosis

A finding of recurrent mouth ulcers coupled with eye inflammation, genital ulcers or skin lesions is used to diagnose the Behcet's syndrome. A special skin test termed pathergy test may be performed. Other laboratory tests are requested when there are other symptoms present. [2]

 Treatment

There is no cure. Treatment is aimed at decreasing discomfort and preventing complications.

Some measures can be done to deter involvement of other organs such as exercise to keep the joints strong. [4]

References:

 1. http://www.mayoclinic.com/health/behcets-disease/DS00822

 2. http://www.medicinenet.com/behcets_syndrome/article.htm

 3. http://emedicine.medscape.com/article/1006358-overview

 4. http://www.behcets.com/site/pp.asp?c=bhJIJSOCJrH&b=260548

 5. http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html

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