Behcet's disease - relapsing disease belonging to the group of systemic vasculitis and manifests erosive ulceration of the mucous membranes of genitals, eyes, mouth, with involvement in the pathological process sutavov and internal organs.(Vasculitis - damage of the vascular wall).
the first time, in 1937, the characteristic triad of this disease (uveitis with hypopyon ulcer + + genital ulceration of the oral mucosa) described the Turkish dermatologist Behçet.Mostly the disease is observed in the Far / Middle East, the Mediterranean and in Tunisia, Turkey, Iran, Korea, Morocco and Japan.The incidence in different countries much retail, so, for example, in Turkey it is 2 per 1000 population in Japan, 1 in 10,000 and 1 in northern Europe at the 300,000 available scientific evidence on individual cases, Behcet's disease in Australia and the United States.
Behcet's disease in children, even in countries with high rates of morbidity, is very rare.The average age of onset of the
Causes Behçet modern science is not completely known.A role in the development of the disease is removed genetic conditions.It is also assumed that the precipitating factor may be bacterial or viral infection.The only thing that is proven is that Behcet's disease - an autoimmune condition, ie,a condition where the body's own immune system is not responding to his cell the same organism, namely killing them.The most probable picture of the development of this disease is the simultaneous combination of autoimmune mechanisms with toxic, bacterial and viral agents
Behcet's disease - symptoms
Symptoms of Behcet's disease are diverse, their defining features are inflammatory eye disease and aphthous ulceration of the mouth and genitals.
- Changes in the oral cavity.For buccal mucosa, gums, tongue, palate and lips appear clear content filled small vesicles, which are then opened to form aphthae bright pink color with a diameter of two to twenty millimeters.If Behcet's disease ulcerative stomatitis is healing for one month, but then three or four times a year recurs.It is also possible the emergence of diseases such as pharyngitis, glossitis and gingivitis
- Changes in the reproductive organs.Women on the mucosa of the vagina and vulva, and men in the scrotum and / or head of the penis form of genital ulcers.As to the oral mucosa and mucous genital sores are painful and often merging together, turn into solid erosive area.After healing of genital sores on mucous scars usually remain
- Changes in the organs of vision.Bodies of Behcet's disease affects several weeks after which developed ulcerative stomatitis and manifests itself in the form of iridocyclitis (inflammation of the ciliary body and the iris of the eyeball) and recurrent iritis, keratitis (inflammation of the cornea), conjunctivitis (changes in mucosal inflammatory), uveitis (inflammation of thechoroid).There is pain in the eyes, watery eyes, increased sensitivity to light and blurred vision
- Changes in the skin and the musculoskeletal system.Skin symptoms include hyponychial felon, folliculitis, ulcers, papules, rash, erythema nodosum.In about 50% of patients with monoarthritis or oligoarthritis with the defeat of the wrist, elbow, knee and ankle joints.Arthritis in Behcet's disease occurs without joint destruction
- changes in the nervous system.If Behcet's disease neurological symptoms is the development of stem and spinal disorders, meningoencephalitis, papilledema, hemiparesis, quadriplegia
In addition to all the above, with Behcet's disease is marked development of inflammatory intravascular changes that lead to a narrowing of the arteries and veins, which then flowsthe development of necrosis of their walls.Disorders of the cardiovascular system appear lower limb venous thrombophlebitis, vasculitis, pulmonary embolism, thrombosis, hepatic vein thrombosis, and aneurysms of the aorta, femoral, popliteal and ulnar arteries.
With gastrointestinal symptoms are observed such as colitis, enteritis, diarrhea, nausea;by light - diffuse infiltration of lung tissue, pleural effusion, hemoptysis;from the heart - endocarditis, myocarditis, pericarditis.
Home Behcet's disease is a gradual and sharp.In childhood, from the time the first symptoms (recurrent stomatitis, headache, unmotivated fever) to the expanded picture of the disease often takes several years (sometimes up to ten years)
diagnosis of Behcet's disease
based on diagnostic criteria that are diagnosed Behcet's disease: aphthous stomatitis, aphthae external genitalia, synovitis, uveitis, meningoencephalitis, cutaneous vasculitis.If two of the above symptoms + Aphthous mucous membranes, the diagnosis is considered to be reliably established.Compulsory shown holding differential diagnosis of diseases such as the Reiter's disease, systemic lupus erythematosus, herpes mucous membranes, other systemic vasculitis
Behcet's disease - treatment
Treatment of Behcet's disease requires long-term complex system therapy, a course which includes antibacterialand antiviral drugs, multivitamins, protivopodagricakih and anti-inflammatory drugs, immunosuppressants.
Driving treatment of Behcet's disease developed by the Institute of Rheumatology:
1. Acyclovir or bonafton - 1 hour after meal 1 tablet.3 p.a day, a course of 20 days.
2. Rifampitsilin - 2 caps.2 p.a day, or tarivid - 1 tab.2 p.a day, a course of 20 days.
3. Aevitum - intramuscularly 1 ml of 1 p.a day, a course of 20 days.
4. Oligovit or Supradin - while eating jelly beans 1 1 p.a day, a course of 3-4 months.
5. delagil - after dinner, 1 tab.1 p.a day, a course of 3-4 months.(sometimes up to 10 months.)
6. Colchicine - after eating 0.5 mg 3.a day, a course of a few months to many years
symptomatic local therapy for Behcet's disease involves the use of indomethacin and gapatrombinovoy ointments, mouthwash, and so on. In the period of acute uveitis shown subconjunctival dexamethasone with metazonom, parabulbarno injections of heparin and dexamethasone Kenalogum
Behcet's disease - complications and prognosis
Ocular Behcet's disease can lead to atrophy of the optic nerve, secondary glaucoma, and as a result - a complete or partial loss of vision.Over the past two to five years from the date of onset of the disease is sharply reduced vision or blindness develops.
thrombosis of peripheral arteries often leads to gangrene of the extremities.Meningoencephalitis Behcet's disease leads to mental retardation, blindness, hearing loss and paralysis.
disease in fact is incurable and is a chronic recurrent nature.The period of time the well-being are replaced by relapses, and early in the disease relapses occur frequently, whereas with increasing age the number of disease is reduced, and sometimes even stops completely.
If a patient diagnosed with isolated form of Behcet's disease, which only leads to the defeat of the external mucous membranes, forecast future life satisfactory.In contrast, with the involvement of central nervous system, internal organs, blood vessels and eyes - the prognosis is very unfavorable.About 5% of patients die from the following reasons: aneurysm rupture vasculitis the affected vessels, central nervous system, intestinal perforation.