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Behcet's disease in children
Last reviewed: 17.10.2021
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Behcet's disease - systemic vasculitis of small vessels with necrosis of the vascular wall and perivascular lymphomonocyte infiltration; is characterized by a triad in the form of recurrent aortic stomatitis; genital ulceration and uveitis. It occurs at any age, but in children Behcet's disease is a rare pathology. The genetic predisposition, communication with HLA-B5, B-51h, DRW52 was revealed.
[Causes of Behcet's disease in children
The cause of Behçet's disease is unknown. The virus, bacterial factors, and environmental factors provoke the disease.
Pathogenesis. At the heart of the disease is vasculitis of small vessels. Circulating immune complexes, increased local immune globulins, C 3 and C 9 complement components and neutrophil chemotaxis are detected. Vascular wall necrosis and perivascular lymphomonocyte infiltrates are noted.
Recently, in the development of thrombotic changes, along with cytokines of endothelial cells, the participation of the vasoconstrictive peptide - endothelin - is being discussed.
Symptoms of Behcet's disease in children
An acute or gradual onset. Unmotivated fever, recurrent stomatitis, eye damage in the form of bilateral uveitis. In children, changes in the skin are noted in the form of erythema nodosum, ulceration followed by deep scarring. There may be articular syndrome in the form of monoarthritis or oligoarthritis of middle joints by the type of synovitis. In generalized vascular lesions, a severe course with a lesion of the digestive tract (erosive esophagitis, ulcerative colitis reminiscent of Crohn's disease), CNS damage (mental disorders, meningoencephalitis, children rarely have genital ulcers.
Diagnosis of Behcet's disease in children
In the acute phase - an increase in ESR, leukocytosis, mild anemia, hypergammaglobulinemia, hyperimmunoglobulinemia. "Incomplete" forms of Behçet's disease are described. In such cases, the following diagnostic criteria are used:
- Recurrent aphthous stomatitis.
- Synovitis.
- Cutaneous vasculitis.
- Uveitis.
- Genital ulceration.
- Meningoencephalitis.
In combination with the mandatory symptom (recurrent ulcerative stomatitis), two or three signs should be present (in case of incomplete form).
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Treatment of Behcet's disease in children
Glucocorticosteroids are prescribed 1 mg / kg / day, cyclophosphamide, levamisole in a dose of 50-150 mg / day 1-2 days a week. Colchicine 1 -1.5 mg / day with the transition to maintenance therapy at a dose of 0.5-0.25 mg / day. All drugs in maintenance doses are prescribed from several months to several years. In severe cases, acyclovir intravenously at a dose of 5 mg / kg per injection, administered every 8 hours for 5 days. Plasmapheresis. Prednisolone 1 mg / kg / day.
The prognosis is doubtful for severe forms of the disease and with the involvement of the kidneys (it is rare).