Behçet’s disease is a disease related to inflammation of the blood vessels. It is mainly manifested by canker sores in the mouth or on the genitals, but also by damage to the eyes, skin or joints. More serious manifestations consist of neurological or digestive damage, venous thrombosis and arterial aneurysms as well as certain ophthalmological damage which can cause blindness. Treatment is primarily symptomatic and may include colchicine and corticosteroids with or without immunosuppressants for more severe manifestations.

This disease was first described by the dermatologist Behçet in 1934. It designates an inflammatory disorder which may include vasculitis, that is to say inflammation, of the arteries and / or veins of small or large caliber. , as well as thromboses, that is to say clots also forming in the arteries and / or veins.

Behçet’s disease predominates in the Mediterranean Basin and in Japan. It affects both men and women but tends to be more severe in men. It usually occurs between the ages of 18 and 40 and can be seen in children. 

It evolves in spurts, interspersed with periods of remission. It can sometimes be fatal, following neurological complications, vascular (ruptured aneurysm) or gastrointestinal disorders. A large number of patients eventually go into remission.

The cause of Behçet’s disease is unknown. 

Immunological triggers, including autoimmune triggers, and viral (eg the herpes virus) or bacterial (eg streptococci) could be involved. The HLA-B51 allele is a major risk factor. Indeed, carriers of this allele have a risk of developing the disease 1,5 to 16 times higher compared to non-carriers.

The clinical manifestations of Behçet’s disease are diverse and can be disabling in activities of daily living. These include:

• skin damage such as mouth ulcers present in 98% of cases, genital canker sores present in 60% of cases and preferentially located in men on the scrotum, pseudo-folliculitis, dermo-hypodermic nodules present in 30 to 40% of cases case ;
• joint damage, such as arthralgia and inflammatory oligoarthritis of the large joints (knees, ankles), present in 50% of cases;
• muscle damage, rather rare;
• eye damage, such as uveitis, hypopyon or choroiditis, present in 60% of cases, and causing serious complications such as cataracts, glaucoma, blindness;
• neurological damage present in 20% of cases. Flare-ups often start with fever and headache. They include meningoencephalitis, damage to the cranial nerves, thrombophlebitis of the cerebral sinuses;
• vascular damage: venous thrombosis, often superficial, present in 30 to 40% of cases; arterial damage, rare, such as inflammatory arteritis or aneurysms;
• cardiac disorders, rare, such as myocarditis, endocarditis or pericarditis; 
• gastrointestinal disorders, rare in Europe, they are manifested by abdominal discomfort, abdominal pain and diarrhea with intestinal ulcers, similar to the outbreaks of Crohn’s disease or ulcerative colitis;
• other rare disorders are possible, in particular renal and testicular.

There is no cure for Behçet’s disease. The treatments available aim to control the disease by reducing inflammation.

The management of Behçet’s disease is multidisciplinary (general practitioner, ophthalmologist, internist, etc.). Treatment depends on clinical manifestations:

• colchicine (1 to 2 milligrams per day) remains the basis of treatment, especially skin and joint damage. It may be sufficient in mild forms;
• neurological, ocular and vascular damage requires treatment with corticosteroids or immunosuppressants (cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate) by the system;
• in certain severe ocular forms, alpha interferon can be used by subcutaneous injections;
• anti-TNF alpha antibodies are increasingly used in severe forms of the disease or forms resistant to previous treatments;
• local treatments, in particular ocular forms, may be useful (eye drops based on corticosteroids combined with eye drops to dilate the pupil in order to prevent complications of uveitis);
• oral anticoagulants intended to thin the blood are used to treat thrombosis.

At the same time, it is recommended to stop smoking, tobacco being a risk factor for worsening vascular disorders. Taking corticosteroids, especially in high doses, must be accompanied by a diet low in sugars and salts. In the event of joint pain, practicing moderate intensity exercises, apart from the thrusts, can help to maintain the flexibility of the joints and the muscular strength.

Finally, since Behçet’s disease can cause anxiety and an alteration of one’s self-image, psychological support can help to better accept one’s disease and to cope with it as well as possible on a daily basis.