Behçet syndrome or disease is a disease of unknown causes or origin, though it has been around for some time. It is characterised by ulcers in the mouth, genitals and eyes, and in the vascular system. It is thought to be a multi-system inflammatory disease with a variety of symptoms and manifestations.
It can be complex to diagnose, since it based only on a set of clinical criteria, and these vary by geography. It is thought to occur in between 0.33 and 370 cases per 100,000 people, but there are places where the incidence is much higher than others. It is more common in Turkish, Asian and Middle Eastern people. In Asia, it is more common in females, but in the Middle East, more males develop this disease. It appears to be more severe in males.
- Olmsted County, Minnesota – 5 per 100,000 people
- Turkey – 80-370 per 100,000 people
- Japan, Korea, China, Middle East – 13-20 per 100,000 people
- Northern Spain – 0.66 per 100,000 people
- Germany – 2.26 per 100,000 people
Age of onset
Typically Behçet syndrome appears in the late third and early fourth decades of life and during childhood, with the Turkish average age of onset being 11.7 years, with the disease rarely occurring in kindergarten-aged children.
Signs, symptoms, and pathophysiology of Behçet syndrome
- Recurrent aphthous ulcers (canker sores)
- Genital ulcers
- Uveitis (inflammation of the uvea, middle layer of the eye)
- Retinal vasculitis (inflammation of the retina)
- Gut lesions
- Nervous system problems
- Vascular lesions (aneurysms, thrombosis)
Genital ulcers appear in the vulva and vagina, and are painful, recurrent, and leave scars. These usually occur less than oral ulcers, but genital ulcers are deeper and larger than mouth ulcers. The vagina can harbour undiscovered asymptomatic ulcers.
These include oral ulcers, which are the hallmark of the disease. Next in line are the eyes, skin, arthritis, intestinal disturbances, headache, stiff neck, and vascular complaints.
Cause of Behçet syndrome
There are links with genetic and environmental factors, but no single cause that accompanies every case can be found. The HLA-B51 allele is often found in patients from Asia and the Middle East, but not in Europeans or Americans. Infection with viruses can be singled out, particularly herpes simplex virus (HSV), hepatitis C, and parvovirus B19. Streptococcal antigens have also been suspected – prophylactic penicillin decreased acute arthritis episodes in some patients.
If you have a parent with Behçet syndrome, onset tends to be at a younger age. Children with Behçet syndrome are likely to have a family history of the disease.
What else could it be?
Some suggestions for other conditions that present in this way are:
- Crohn’s disease
- Addison’s disease (adrenal insufficiency)
- Herpes simplex virus (HSV)
- Paediatric sarcoidosis
Each patient will be managed by their physician based on their circumstances. Drugs are used to manage the condition, but there is no cure. Surgery may be used in certain instances.