BEKHCHETA DISEASE (N of Behcet, the Turkish dermatologist, 1889 — 1948) — a chronic general disease with a characteristic triad: aphthous canker of mucous membranes of an oral cavity, eyes and generative organs.
B. is for the first time described by Bekhchet in 1937. it is observed everywhere, but meets on the Middle East more often. As a rule, middle-aged persons are ill. Men are ill severe forms generally.
Etiology it is not known, it is supposed virus. Bekhchet described virus-like inclusions in cells of ulcer exudate. The numerous messages on cases of diseases in several generations of a family allowing to assume participation of genetic factors in B.'s development are saved up.
Histologically nonspecific, preferential kruglokletochny, perivascular infiltrates, vein thromboses are characteristic.
Pathogeny. In B.'s development. the great value is attached to autoimmune reactions since the circulating antibodies in a mucous membrane of an oral cavity are found in patients.
A clinical picture
For B. the recurrent current with periodic remissions of various duration is characteristic. The disease begins sharply, with a characteristic triad. However in some cases the triad can temporarily be incomplete: only the mucous membrane of a mouth and generative organs or eyes and an oral cavity is surprised. On mucous membranes sharply disease superficial aphthous process or deep ulcers is observed. Afta begin with bubbles or pustules. They can be single or multiple, to dia, to 1,5 cm, rounded shape, with a pale yellow bottom, soft bright red edges; the discharge of these sores has yellowish color. Ulcers begin with submucosal hillocks with the subsequent ulceration. Healing aft and ulcers happens scarring. The most frequent localization of ulcer process — a lip, gums, cheeks, language, a scrotum, only in hard cases is surprised a throat, a gullet, a penis, a vagina, a neck of uterus and a crotch. At the same time on skin quite often there are rashes like a knotty or mnogoformny erythema, papulo-pustular folliculites (aknepodobny), sometimes hyponychial abscesses, etc.
Eye symptoms are diverse — conjunctivitis, a keratitis, irites, uveites; hypopyon iritis, an atrophy of an optic nerve can lead to easing of visual acuity up to a blindness; there can be secondary glaucoma.
Except characteristic damages of mucous membranes, in hard cases angiites, arthralgias and arthritises, preferential knee and ankle joints, and also superficial and deep thrombophlebitises, inflammations of veins of a retina, top and bottom venas cava, an aortic aneurysm develop. Passing perikardita, orkhita and epididymites are described, various nonspecific skin rashes, a canker went. - kish. path, hepatolienal syndrome. Heavy and predictively an adverse symptom of a disease are defeats of c. N of page (meningoentsefalita, psychoses, etc.).
Shifts of laboratory indicators at B. are not specific and reflect only existence of inflammatory reaction (a leukocytosis, acceleration of ROE, hyper alfa2-and gamma globulinemiya).
Diagnosis it is put in the presence of characteristic aphthous cankers of mucous membranes and eye symptoms.
Differential diagnosis carry out with a disease of Reuters (see. Reuters disease ), a mnogoformny exudative erythema (see E ritema exudative mnogoformny ), with Stephens's syndrome — Johnson (see. Erythema ), with pemphigus (see).
Forecast. At damage of eyes the blindness can develop. At the long course of a disease there comes the invalidism of patients. The forecast at defeat of c is especially adverse. N of page
Treatment complex. Corticosteroids inside in a dose of 40 — 45 mg a day (in terms of Prednisolonum) in combination with vitamins of group B, drugs of calcium, Ascorutinum and antibiotics. Outwardly — rinsings with disinfecting solutions (boric to - you, Rivanolum, a camomile, potassium permanganate), corticosteroid ointments. There were messages on beneficial effect on disease of leukeranum on 10 — 15 mg a day in combination with corticosteroids inside and locally. In recent years for B.'s treatment. with success apply fibrinolitik (see. Fibrinolitic means ).
Bibliography: Galperin E. A. To clinic of a disease of Bekhchet, Klin, medical, t. 43, No. 6, page 146, 1965, bibliogr.; At r in and the p E. And. and d river. About Bekhchet's syndrome, in book: Aktualn, vopr. dermas, and veins., under the editorship of V. Ya. Arutyunov, page 329, M., 1962, bibliogr.; These T on G. F. K to a question of a syndrome Bekhcheta, Oftalm. zhurn., No. 3, page 223, 1972: In e h ς e t H. t)ber rezidi-vierende, aphthose, durcli ein Virus ve-rursachte Geschwiire am Mund, am Auge und an den Genitalien, Derm. Wschr., bd 105, S. 1152, 1937; G aro it M. et S o 1 n i with a J. Les manifestations articu-laircs du syndrome de Behget, in book: L’actualite rhumatol. <pr£sent5e au praticien 1967, pubi, par J. Amouroux e. a., p. 20, P., 1968, bibliogr.; Savin L. H. Behcet’s syndrome and its differential diagnosis, Trans, ophthal. Soc. U. Κ., v. 83, p. 17, 1964, bibliogr.
V. A. Nasonova.