Behcet's disease in adults

Behcet's disease (synonyms: Turoth's large aphthus, Behcet's syndrome, triple syndrome) is a multi-organ, inflammatory disease of unknown etiology, whose clinical picture consists of aphthous stomatitis and lesions of genital organs, eyes and skin.

The disease was first described by the Turkish dermatologist Behcet in 1937. The most common is in Japan (1: 10,000), in the countries of Southeast Asia and the Middle East. Behcet's disease is more common among men and most often affects people between the ages of 20 and 40.

[1], [2], [3], [4], [5], [6], [7]

The causes and pathogenesis of Behcet's disease

The causes and pathogenesis of Behcet's disease are not fully understood. Assume the viral nature of the disease. The detection of antibodies in blood serum, the oral mucosa indicates the involvement of immune (autoimmune) mechanisms in the pathogenesis of dermatosis. Family cases of the disease, the presence of Behcet's disease in several generations in one family, suggest the involvement of genetic factors in the development of the disease. It assumes viral nature, the role of infectious-allergic, immune (autoimmune) disorders is shown. The importance of genetic factors is not ruled out, as can descriptions of family cases of the disease, including twins. There is an association with the antigens of the blood group HLA-B5, HLA-B12, HLA-B27, HLA-BW51, HLA-DR-2, HLA-DR-7, HIA-Cw2. Some believe that the disease is based on vasculitis.

Pathomorphology of Behcet's disease

At the heart of lesions is allergic vasculitis involving capillaries, venules and arterioles, the walls of which are infiltrated by lymphocytes and plasma cells. This is accompanied by the proliferation of the entolleocytes, the deposition of fibrin in the walls of the vessels and in the surrounding tissue. In the active phase of the process, the number of tissue basophils in the upper parts of the dermis and in the podepithelial layer of the mucous membranes that release histamine increases, which increases tissue permeability. In the skin with lesions of the type of necrotic pseudofolliculitis, superficial necrosis areas covered by the crust, enlarged capillaries with a swollen endothelium and subepidermal hemorrhages are found. Around the epithelial hair follicles develops an infiltrate of lymphocytes, plasmocytes and tissue basophils. In lesions of the type of erythema nodosum, deep infiltrates and small hemorrhages, located in the dermis and in the subcutaneous tissue, are found. In places of infiltration, homogenization and fragmentation of collagen fibers are detected. In aphthous ulcers, lymphocytes, mononuclear cells and neutrophytic granulocytes are found that contact epithelial cells at the edges of ulcers. Epithelioid-cell histiocytic infiltrates in the form of tuberculoid structures are sometimes found around necrosis areas.

The histogenesis of Behcet's disease has not been studied. The main role in the pathogenesis of the disease belongs to antibody-dependent reactions to the epithelium of the oral mucosa. T. Capeko et al. (1985) were found in lesion lesions, both aphthous and erythema nodosum, IgM deposits, as well as fluorescence with serum against streptococcal antigen in the walls of the vessels and in the cells of the inflammatory infiltrate. Part of the research indicates a viral etiology of the disease, in particular association with the virus of idleness of gerpeca. By the method of direct immunofluorescence, deposits of the IgM and C3 complement components are found, located diffusely among the infiltrate cells and in the walls of the vessels of deep lesions. Deposits of IgG and IgA are found only on the surface of aphthous ulcers. In the blood serum of patients there are immune complexes. Scientists have found deposition in the vascular walls of IgM, IgG and IgA, associated with C3 and C9 components of complement, indicating an immunocomplex mechanism of the development of this disease. Reaction with the use of monoclonal antibodies revealed a defect of T-lymphocytes. In the early stage of the disease there was a decrease in the number of OKT4 + - and an increase in OCT + cells in the peripheral blood. At the same time, part of the patients noted the activation of the B-cell link of immunity: there are data on the detection in the serum of patients with anti-cardiolipin antibodies.

Symptoms of Behcet's Disease

The most common symptom is a triad of symptoms: aphthous stomatitis, an erosion-ulcerous lesion of the anogenital area, changes in the eyes. Skin changes of a polymorphous nature: erythema nodosum type, pyogenes, nodular elements, acneiform, hemorrhagic rashes developing against a background of fever and general malaise. Less common thrombophlebitis, meningoencephalitis, damage to the cardiovascular system and other visceral organs. The disease develops sharply, proceeds paroxysmally, with the passage of time the frequency of exacerbations decreases. The prognosis depends on the degree of involvement of the internal organs.

The clinical manifestation of Behcet's disease includes orogenital ulcers, skin lesions, anterior or posterior uveitis, arthralgia, neurologic lesions and vascular thrombosis. The main symptoms are divided according to their frequency in the following way: aphthous stomatitis (in 90-100% of patients), ulcers on the genitals (in 80-90% of patients), eye symptoms (in 60-185% of patients). Further frequency is followed by thrombophlebitis, arthritis, rashes on the skin, damage to the nervous system, etc. Lesions of the oral mucosa are characterized by the formation of aphthous yeast-like erosions and ulcers having different outlines. They appear on the tongue, soft and hard palate, palatine arches, tonsils, cheeks, gums and lips and are accompanied by severe pain. Usually, rashes start with a limited painful compaction of the mucosa, on which a superficial, fibrous coating is formed first, and then a crater-like ulcer with a slight hyperemia around. The ulcer can grow in size up to 2-3 cm in diameter. Sometimes the disease begins as a surface aphtha, but after 5-10 days at the base of such an aphthae appears infiltrate, and the aphtha turns into a deep ulcer. After healing, soft, superficial, smooth scars remain. At the same time, there may be 3-5 lesions. Such aphthous foci occur on the mucous membrane of the nose, larynx, esophagus, gastrointestinal tract. Lesions on the genitals consist of small blisters, surface erosions and ulcers. On the genitals of men, ulcers are located on the scrotum, near the root of the penis, the inner surface of the thighs. The outlines of ulcers are wrong, the bottom is uneven, covered with a serous-purulent coating, sharply pronounced soreness. Women on large and small labia in large quantities find painful palpation of an ulcer the size of a pea 10 five-kopeck coins.

Eye damage begins with periorbital pain and photophobia. The main sign is the damage to the vessels of the retina, which ends in blindness. In the beginning of the disease, there are phenomena of conjunctivitis, and later - hypopion. In addition, there is iridocyclitis. In the absence of treatment, atrophy of the optic nerve, glaucoma or cataract, which lead to blindness, is noted. With Behcet's disease, both eyes are affected. On the skin of the trunk and extremities appear rashes in the form of nodular and multiforme exudative erythema, nodules, pustules and hemorrhagic elements, etc. In 15-25% of patients with Behcet's disease, surface recurrent thrombophlebitis occurs and in 46% - cardiovascular complications. In this regard, the opinion is expressed that Behcet's disease is a systemic disease based on vasculitis.

Usually, the disease is accompanied by a severe general condition of the patient, fever, severe headache, general weakness, joint damage, meningoencephalitis, paralysis of the cranial nerves, etc. Periodically, there is an improvement and even a spontaneous remission, which is replaced by a relapse.

Differential diagnosis

Behcet's disease should be distinguished from aphthous stomatitis, acute Chapin-Lipshutz ulcer, aphthous-ulcerative pharyngitis, oculo-genito-urethral syndrome of Reuter, bullous form of multiforme exudative erythema.

[8], [9], [10], [11], [12], [13]

Treatment of Behcet's Disease

Depending on the severity of the disease, corticosteroids, cytostatics, antibiotics alone or in combination are recommended. The pronounced effect is noted from the administration of colchicine (0.5 mg twice daily for 4-6 weeks), cyclophosphamide, dapsone. External apply disinfectant solutions, corticosteroid ointments.