Separate
Separate authors paid attention to presumably not casual combinations of syndrome with and with an accompanying infection (in particular, hyponychial), that can indirectly specify in immune disturbances inherent to disease. Laboratory indicators. In an active phase of illness usually note ESR augmentation, unsharp a leukocytosis, a moderate anaemia, rising of level of S-jet fiber, a fibrinogen and a2-globulins. The polyclonal hypergammaglobulinemia and rising of level of separate classes of immunoglobulins is often found out. At exacerbations of syndrome note increase of level of immune complexes, 9th component of a complement and activity of neutrophils, formation clumps of thrombocytes round neutrophils, occurrence of cryoglobulins and antibodies to cells of mucosas, fibrinolysis depression. The Russian Federation, nuclear antibodies and LE-cells are not defined. The diagnosis. For the diagnosis of syndrome it is considered sufficient a combination of an ulcerative (aphthous) stomatitis, a canker of genitals and an inflammation of tissues of an eye (first of all a uveitis). Very much great value such signs, as an arthritis have also, a meningocephalitis and the dermal changes surveyed above. J. O’Duffy (1981) suggests to consider the diagnosis authentic in the presence of three of the following clinical signs: 1) a relapsing aphthous stomatitis; 2) aphthous ulcers of genitals; 3) a uveitis; 4) a synovitis; 5) a dermal vasculitis; 6) a meningocephalitis. Presence of an aphthous stomatitis is an obligatory symptom. If aphthous ulcers of an oral cavity or genitals are combined only with one of the listed clinical signs with some basis the “incomplete” form of syndrome can be diagnosed. It is important to mean, that all surveyed symptoms can develop . It is necessary to remember about such actually specific (though also absolutely unessential) a symptom, as occurrence of dermal pustules after any injections. Doubtless value have and the certain national features noted above. In the former USSR disease meets usually among aboriginals of caucasus and Transcaucasia. In single instances there is a question on differentiation of syndromes and to which such general lines as a lesion of a skin and mucosas are inherent. An acute asymmetrical polyarthritis, an inflammation of tissues of an eye, sometimes also a neurologic pathology. However at syndrome the stomatitis happens very seldom, ulcers of genitals and a mouth mucosa are usually painless, the eye pathology is characterised, as a rule, a conjunctivitis (instead of a uveitis), the lesion of nervous system is faster an exception and does not differ special gravity. Besides, the thrombophlebitis and a nodulose erythema are absolutely unusual for syndrome . At the same time the obvious clinical picture of a spondylitis in ready smaller degree is inherent in syndrome . Separate patients with an ulcerative colitis can have the clinical picture practically indistinguishable from syndrome with symptoms of a colitis (relapsing aphthous ulcers of a mucosa of a mouth, an arthritis, a nodulose erythema, an iritis). However at an ulcerative colitis ulcers of genitals and a back uveitis do not develop, and oral cavity aphthas usually do not happen serious. Besides, at a colonoscopy it is possible to establish, that at patients with syndrome and presence of ulcers in a colon these ulcers (unlike ulcers at actually ulcerative colitis) are surrounded outwardly normal, instead of the inflamed mucosa. In differential diagnostics it is necessary to mean also Stevens-Johnson’s syndrome which develops more often at men till 20 years, and it is especially frequent at children’s age. Disease begins, as a rule, acutely, with a heat and fast subsequent development of a serious stomacace. Ulcerative changes can arise also on a nose mucosa, a conjunctiva, genitals and (that is important for the diagnosis) round a fundament. In some days the picture an exudative erythema is brightly shown, on a skin there is a violent or vesicular eruption. From system implications the pneumonitis is possible. In some cases disease develops after a drug intake and can be surveyed as allergic reaction to them.
Skin changes
Skin changes are inherent approximately 70 % of patients with syndrome and are surveyed as diverse implications of a dermal vasculitis. Erythematic papules, pustules, blisters and elements of type of a nodulose erythema concern them. The last can differ nothing from a “usual” nodulose erythema, but in some cases show some features. So, knots sometimes settle down clumps, are localised on arms, and at individual patients even . At separate patients elements of a necrosis and the pyeses of a skin reaching appreciable diffusions - a so-called gangrenous pyoderma are expressed. For syndrome though and meeting at all at all patients, formation of a small pustule or a nodule (sometimes with a pink crown) in a day after any injections in a place of introduction of a needle is practically pathognomonic. This feature can be used and in the diagnostic purposes - after introduction of an isotonic solution of sodium of Sodium chloridum or even a nyxis the sterile pustule arises a dry needle sometimes. Lesion of vessels. Besides surveyed above implications of a dermal vasculitis, the thrombophlebitis of superficial or deep veins quite often develops. Is more often it is localised standing and sometimes it appears the reason of an embolism of pulmonary vessels. Clottages of the top or bottom vena cava are very seldom observed. Sometimes the phlebitis develops immediately after a venipuncture. Involving of arteries in pathological process is observed seldom and the aneurysm is shown by clottages and formation. These changes found out in an aorta, ulnar, femoral and popliteal arteries, eyeground vessels. It is described also a pulmonary artery. The thrombosing of arteries of feet can lead to a gangrene of feet. Neurologic pathology. Involving in process of nervous system meets quite often - at serious forms of illness almost at 1/4 patients. The basic neurologic pathology at syndrome - a meningocephalitis arising usually not at early stages of illness and shown by a fever, a sharp headache, a stiff neck. The spinal liquid is thus sterile and is characterised by a lymphocytosis and the normal maintenance of gamma-globulins. The meningocephalitis which can proceed with exacerbations and remissions, does the forecast of disease by much more serious. There are other variants of a lesion of nervous system - and spinal disorders, disturbances conductions, reversible rising of intracranial pressure a papilledema, a transient pathology of cranial nerves less often. Peripheric are observed seldom. At separate patients hemipareses, tetraplegias, “good-quality” rising of intracranial pressure are described. Psychoses rather seldom become perceptible. Digestive tube lesion. With syndrome diarrhoeia, a nausea, proctitis symptoms, perianal ulcers are inherent to a number of patients. Sometimes intestine lesions are indistinguishable from an ulcerative colitis and illness the Crone. Some authors underline pathogenetic affinity of these diseases and specify, that at primary illness the Crone there can be all signs of syndrome . At separate patients with a combination of signs of syndrome and illnesses the Crone the resection of the amazed department of a colon led also to appreciable reduction of symptoms of syndrome . In individual patients found out duodenum ulcers. Gastroenteric implications are especially frequent at Japanese (50 % of all patients). Other implications of illness. Among more rare symptoms of syndrome it is necessary to specify in involving possibility in process of lungs with their diffusive infiltration, a pneumorrhagia and a pleuritis (it is almost exclusive at the Turkish population). Heart lesions (are known also especially at serious generalised forms of illness): a myocarditis, a pericarditis, an endocarditis with formation of moderately expressed valval defects. At a small part of patients epididymites and an amenorrhea. Reports on a pancreatitis concern curiosities, and not serious nephritis. There are individual descriptions of the hypercoagulative syndrome accompanied by the lowered fibrinolysis. Infrequent complication of syndrome - a system secondary amyloidosis, especially among inhabitants of the Near East.