Bekhchet's disease – a disease of group of system vaskulit with damage of small and average arteries and veins, recidivous erosive ulcerations of a mucous mouth, genitals, eyes, skin, involvement of internals and joints. Displays of a disease of Bekhchet are diverse and can include aftozny stomatitis, ulcers of genitals, a skin and hypodermic knotty eritema, uveit, arthritis, vaskulit, dysfunction of lungs, kidneys, a stomach, an encephalomeningitis. Diagnosis of a disease Bekhcheta is based on clinical and laboratory data; treatment includes local and system immunosupressivny therapy. Bekhchet's disease proceeds with the periods of a recurrence and temporary remission, complications from the interested bodies.
The disease etiology Bekhcheta is not clear. A part in its development is played by genetic conditionality. It is known that prevalence of a disease is higher among men of 20-40 years inhabiting Middle East and Asian regions. Value of an infection and toxic factors in development of a disease of Bekhchet is taken into account. Influence of autoimmune mechanisms is not excluded: at patients the CEC, antibodies to cells of a mucous membrane of an oral cavity decide on Bekhchet's disease, oppression of specific and nonspecific immune protection is noted. The simultaneous combination several factors when virus, bacterial, toxic and so forth agents provoke the beginning of a disease with the subsequent connection of autoimmune mechanisms is represented to the most probable.
Symptoms of a disease of Bekhchet
Bekhchet's disease proceeds polisimptomno; the defining signs are aftozny ulcerations of a mucous mouth and genitals, an inflammation of eyes. On lips and in an oral cavity (in the sky, language, gums, mucous cheeks) there are small vesicles filled with opaque contents which are opened with formation of bright pink sores () with a diameter from 2 to 10-20 mm. Occupies healing of ulcer stomatitis at Bekhchet's disease to 1 month, however its recurrence arises 3-4 times a year.
Genital sores are localized at men in a penis and a scrotum, at women – on mucous vulvas and vaginas. Sores on a mucous mouth and genitals are painful, merging among themselves, form continuous erosive surfaces. After healing of ulcers on mucous there can be hems.
Symptoms from organs of vision appear several weeks later after ulcer stomatitis. Recidivous Irit's development and an iridotsiklita (an inflammation of an iris and a tsiliarny body of an eyeball), a uveita (an inflammation of a vascular cover of an eye), conjunctivitis (inflammatory changes of a mucous membrane), a keratit (a cornea inflammation) is characteristic of Bekhchet's disease. Patients with Bekhchet's disease note dacryagogue and gripes in eyes, a sight zatumanennost, the raised photosensitivity.
Skin symptoms include a nodal eritema, rash, papules, ulcers, follikulita, subnail felons. From musculoskeletal system at 50-60% of patients develop oligo-or monoarthritises with damage of ankle, knee, elbow, luchezapyastny joints. Arthritises at Bekhchet's disease proceed without destruction of joints. The neurologic symptomatology at Bekhchet's disease consists in development of an encephalomeningitis, spinal and stem frustration, a tetraplegiya, a hemiparesis, hypostasis of a disk of an optic nerve owing to increase in VChD.
Bekhchet's disease is characterized by development of the intra vascular inflammatory changes causing narrowing of a gleam of veins and arteries, and then a necrosis of their walls. Vascular frustration are shown vaskulity, thrombophlebitis of veins of the lower extremities, thrombosis of hepatic veins and a thrombembolia of a pulmonary artery, aneurisms and thromboses of an aorta, elbow, popliteal femoral arteries. At patients with Bekhchet's disease the GIT (nausea, a diarrhea, enteritis, colitis), heart (perikardit, myocarditis, an endocarditis), lungs (a blood spitting, pleurisy, diffusion infiltration of pulmonary fabric) is surprised.
Diagnosis of a disease of Bekhchet
Kliniko-diagnostichesky criteria of a disease of Bekhchet is the recidivous course of stomatitis, aftozny damage of genitals, arthritis, a uveit, vaskulit, an encephalomeningitis. For suspicion of Bekhchet's disease there is enough presence of any 3rd from the listed symptoms at obligatory existence of aftozny stomatitis. Consultation of the rheumatologist is necessary for specification of the diagnosis.
From diagnostic tests patergichesky test has the greatest value: at patients with Bekhchet's disease 24-48 hours later after performance of a subcutaneous injection on the place of a prick develops a jet inflammation. Clinical blood test at Bekhchet's disease shows moderate anemia, neytrofilny , increase in SOE. In biochemical blood test increase in values of fibrinogen, a gaptoglobin, α-2-globulin, a seromukoida is noted. The research of a koagulogramma shows increase in activity of factors of coagulability of blood.
Immunoassays reveal decrease in quantity T-helperov and T-lymphocytes, increase of the CEC, level of components of a complement, immunoglobulins (IgA), etc. For diagnostics of changes of joints at Bekhchet's disease carry out a X-ray analysis, ultrasonography of a joint and an arthroscopy. According to indications carry out a diagnostic puncture of a joint. In sinovialny liquid the neutrophils testifying to the inflammatory nature of process prevail.
Treatment of a disease of Bekhchet
Bekhchet's disease demands complex and long system therapy. The medicamentous course includes purpose of antiviral and antibacterial means, polyvitamins, immunosupressor (), medicines of anti-inflammatory and antigouty action (colchicine).
In 1-2 months of therapy in the absence of dynamics add Prednisolonum; in a month – cytostatics (hlorambutsit inside or intravenously). Therapy by cytostatics is carried out under control of dynamics of the general blood test, appointing if necessary transfusion of trombotsitarny or leykotsitarny weight.
At a disease Bekhcheta is shown extracorporal therapyto - plasma exchange, a gemosobrtion, UFOK and VLOK having immunomodulatory effect. Local symptomatic therapy at Bekhchet's disease includes rinsings of a mouth, use of gepatrombinovy and indometatsinovy ointments etc. During an aggravation of a uveit appoint subconjunctival introduction of a mezaton with dexamethasone, parabulbarny injections of dexamethasone and heparin, methylprednisolonum, a triamtsinolon.
Complications and forecast of a disease of Bekhchet
Danger of damage of eyes at Bekhchet's disease consists in risk of development of secondary glaucoma, an atrophy of an optic nerve, partial or full loss of sight. Sharp decrease or a total blindness develop within 2-5 years from a debut of a disease of Bekhchet. The encephalomeningitis can lead to paralyzes, hearing loss, a blindness, intellectual backwardness. Thrombosis of peripheral arteries at Bekhchet's disease quite often comes to an end with development of gangrene of extremities.
At the isolated form of the disease of Bekhchet affecting only damage of external mucous membranes, the forecast satisfactory. In cases of involvement of eyes, TsNS, vessels, internals, the course of disease predictively is adverse. Prevention of a disease Bekhcheta is not developed in view of an ambiguity of the reasons and mechanisms of its development.