Behçet's disease

This is a rare disease in the Western world. It is most common in Turkey and Japan, where it is a main cause of uveitis. Outside these countries Behçet's syndrome is rare.

Common symptoms:

Recurrent mouth ulcers - in 100% - usually heal within 10 days without scarring
Genital ulcers (scrotum or vulva) - in 90%
Sticky eyes (conjunctivitis) - typically both eyes at the same time
Patchy red areas on skin (erythema nodosum)

In most cases, ocular symptoms follow the oral and genital ulcers by 3-4 years. However, in one in five sufferers from Behçet's syndrome, ocular symptoms are the symptoms that occur first.

Other symptoms

Red painful eyes (iritis, episcleritis, or keratitis) - typically both eyes
Arthritis of wrists and ankles (50%)
Muscle and sensory weakness
Abdominal pain, blood in stools (50%)

Signs that an ophthalmologist would look for

Hypopyon (a line of white cells at the bottom of the anterior chamber) - pccurs in 30%
Retinal vasculitis - affecting both retinal arteries and veins
Retinal cystoid macular oedema (CMO), disc oedema, retinal oedema

Possible complications outside the eye

Skin lesions (erythema nodosum)
Inflammation of the veins (thrombophlebitis)
Arterial disease, including occlusion and aneurysms
Neurological problems


Diagnosis is based mainly on the clinical features. Blood tests, X rays are routinely done to exclude other causes of the symptoms
Fluoroscein angiography
Amongst sufferers from Behcet's syndrome HLA-B5, a marker found in blood, is particularly common
Cutaneous hypersensitivity, where the skin shows an allergic-type reaction to a minor scratch or injection of salt water, is a particular feature


Unknown. Possibly due to circulating auto-immune complexes causing a vasculitis (inflammation of blood vessels) all over the body.


Treatment is best done by an ophthalmologist with a special interest in iritis/uveitis.

Steroid eyedrops for eye disease
Oral steroids (eg. Prednisolone)
Oral cyclophosphamide
Oral chlorambucil
Oral cyclosporin
Oral colchicine


Eye symptoms can be difficult to control. The disease appears to have a more severe course in Japan and Turkey, where legal blindness occurs in 50% of cases within four years of diagnosis.  In the USA, the prevalence of legal blindness in all cases of Behçet's is only 25%.


USA: 1 person in 300,000 suffers from Behçet's syndrome.
Japan: 1/10,000
Turkey: 8-30/10,000

Back to home