Behçet’s disease: what is it?

Behçet’s disease is a chronic vasculitis (inflammation blood vessels) manifested by ulcerations of the mouth, skin, eyepieces and genitals. It progresses through acute and recurrent inflammatory attacks. Behçet’s disease is neither hereditary nor contagious. Its frequency differs greatly depending on the country, the most affected being Turkey and Japan. It is much rarer in Europe.

Behçet’s disease: symptoms

Behçet’s disease is generally defined by a typical triad:

• Oral and genital ulcers: canker sores are very frequent and recurrent. The typical lesion is round (between 1 and 3 cm in diameter), with a well-defined erythematous border, and covered with a yellowish-white film. In the mouth, the canker sores may affect the tongue, gums, mucous membranes labial and buccal. At the genital level, the lesions appear on the scrotum and the penis in men, and on the vulva in women. They are often very painful and can leave scars.
• Skin ulcerations: erythema nodosum (painful subcutaneous nodules), pseudofolliculitis (pustule not centered by a follicle hairy), type pimples acne, thrombophlebitis superficial migratory. The skin becomes hyperreactive to all aggressions (injections, scratches, allergen testing).
• Eye damage: this is the symptom the most serious form of Behçet’s disease. Inflammation of theeye concerns about 60% of patients with a blindness rate of 10 to 15%. It is manifested by uveitis, hypopion (intraocular suppuration), photophobia, tearing or red eye. These repeated inflammations can lead to alterations in theiris and some retinacausing a decrease or even loss of vision.

Other symptoms can be found:

• Arthralgia affecting especially large joints (knees, wrists, pegs, elbows). They are recurrent and most often asymmetrical.
• Digestive disorders : anorexianausea and vomiting, diarrhea, pains abdominal. The symptoms are quite similar to those of Crohn’s disease.
• Venous involvement: thrombosis venous most often in the lower limbs, stomach and head. Arterial thrombosis is rarer (3 to 5% of cases), with a significant risk ofaneurysm.
• Neurological impairment: this is the main cause of death linked to Behçet’s disease. It affects 20% of patients within one to ten years of the appearance of the first signs of the disease. We then speak of “neuro-Behçet”. It takes different forms: meningitis or meningoencephalitis, paralysis of the nerves cranial, cerebral venous thrombosis. These attacks are responsible for headaches, impaired consciousness, confusion (disorientation), paralysis of the muscles of the face (disorders of eye movement and swallowing, etc.), epilepsy, personality changes, dementia.

Behçet’s disease: causes and origin

The causes of Behçet’s disease remain unknown to this day. The most common hypothesis is that of a inflammation triggered by a viral or bacterial infection. The disease is characterized by an infiltration of lymphocytes and of neutrophils in the affected organs, which would be activated by an infectious agent. We also know that this infiltration pathological depends on a predisposition genetic. antigen HLA B51 is thus found in 50% to 70% of patients.

Behçet’s disease: diagnosis and treatment

There is no specific test for Behçet’s disease. the diagnostic is based on the presence of the classic triad mentioned above, and by eliminating other possible causes of inflammation. Treatment mainly consists of relieving the symptoms. It depends a lot on the clinical manifestations and must be individualized. Colchicine, for example, is used to reduce the frequency of mouth and genital ulcers. For severe forms, anti-inflammatory drugs are prescribed corticosteroids during pushes. Recent treatments also include anti-TNF alphas antibody which inhibit the action of TNF alpha, a protein involved in inflammation and immune response.

Without treatment, eye damage is irreversible and leads to blindness. Arterial rupture presents a life-threatening risk and neurological disorders lead to loss ofautonomy.