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% |
| Iritis |
| Vitritis |
| 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
| 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 |
Cause
Unknown. Possibly due to circulating auto-immune complexes causing a
vasculitis (inflammation of blood vessels) all over the body.
Treatment
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 |
Prognosis
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%.
Epidemiology
| USA: 1 person in 300,000 suffers from Behçet's syndrome. |
| Japan: 1/10,000 |
| Turkey: 8-30/10,000 |
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