Behcet's disease is a rare, chronic disorder that involves inflammation of blood vessels throughout the body, affecting mainly small to medium sized arteries and veins.
Recurrent oral ulcers are usually the first manifestation and develop in most patients with Behcet's Disease. They appear as a single lesion or as crops, involving the lips, tongue, gingival or mouth cavity, but seldom the nose or upper airways.
The disorder may also cause recurrent genital ulcers, and eye inflammation plus various types of skin lesions, arthritis, bowel inflammation, and meningitis (inflammation of the membranes of the brain and spinal cord).
Behcet's disease is a multi-system disease; it may involve all organs and affect the central nervous system, causing memory loss, impaired speech, and balance and movement disorders. A physical finding that is very characteristic of Bechet's is the appearance of an inflamed skin area at the site of a needle puncture. This is called “pathergy”.
The problem arises as a consequence of inflammation of blood vessels – arteries and veins. This is called a vasculitis.
Organ involvement includes heart, lungs, kidneys and gastrointestinal tract.
There is a genetic link. There is a strong geographical link as well. The condition is common in the Mediterranean region, especially in Turkey. It is rare in South Africa.
Behcet's disease generally begins when patients are in their 20's or 30's, although all age groups may be affected. Males are more commonly affected.
The disease course of Behcet's is very variable and a combination of features may be present at any given time. The skin, mouth and genital lesions usually appear with each attack.
Symptom-free intervals may be measured in weeks, months or even years. Death rate is the highest in those with central nervous involvement, e.g. meningitis or nerve palsies.
Behcet's disease is a lifelong disorder which proceeds over a long period of time in a series of remissions (lack of disease activity) and exacerbations (periods of active disease). The disease is serious and painful but seldom fatal.
- Treatment for Behcet's disease is symptomatic and supportive. Medication may be prescribed to reduce inflammation and/or regulate the immune system.
- These include high dose cortisone and immune suppression drugs, including cyclophosphamide or azathioprine.
- Anticlotting therapy may be required, including aspirin in low dose or warfarin.
- It is a serious disease. About 20% of people with Behcet’s Disease get severe vision loss.
- If a patient goes into remission, treatment is still required for a prolonged period.
Previously reviewed by Dr David Gotlieb, rheumatologist
Reviewed by the Arthritis Foundation, September 2011
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