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Behcet's disease in children
Last reviewed: 05.07.2025
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Behcet's disease is a systemic vasculitis of small vessels with vascular wall necrosis and perivascular lymphomonocytic infiltration; it is characterized by a triad of recurrent aortic stomatitis; genital ulcers and uveitis. It occurs at any age, but in children Behcet's disease is a rare pathology. A genetic predisposition has been identified, as well as a connection with HLA-B5, B-51h, DRW52.
[ Causes of Behcet's disease in children
The cause of Behcet's disease is unknown. The disease is provoked by viral, bacterial and environmental factors.
Pathogenesis. The disease is based on small vessel vasculitis. Circulating immune complexes, increased local immune globulins, complement components C3 and C9, and neutrophil chemotaxis are detected . Necrosis of the vascular wall and perivascular lymphomonocytic infiltrates are noted.
Recently, in the development of thrombotic changes, along with endothelial cell cytokines, the participation of the vasoconstrictor peptide, endothelin, has been discussed.
Symptoms of Behcet's disease in children
Acute or gradual onset. Unmotivated fever, recurrent stomatitis, eye damage in the form of bilateral uveitis. In children, skin changes are observed in the form of erythema nodosum, ulceration followed by deep scarring. There may be a joint syndrome in the form of monoarthritis or oligoarthritis of the middle joints by the type of synovitis. With generalized vascular damage, a severe course is observed with damage to the digestive tract (erosive esophygitis, ulcerative colitis resembling Crohn's disease), damage to the central nervous system (mental disorders, meningoencephalitis. Genital ulcers are rare in children.
Diagnosis of Behcet's disease in children
In the acute phase - increased ESR, leukocytosis, moderate anemia, hypergammaglobulinemia, hyperimmunoglobulinemia. "Incomplete" forms of Behcet's disease have been described. In such cases, the following diagnostic criteria are used:
- Recurrent aphthous stomatitis.
- Synovitis.
- Cutaneous vasculitis.
- Uveitis.
- Genital ulcers.
- Meningoencephalitis.
In combination with the obligatory symptom (recurrent ulcerative stomatitis), 2 or 3 more symptoms (in the incomplete form) must be present.
What tests are needed?
Who to contact?
Treatment of Behcet's disease in children
Glucocorticosteroids are prescribed at 1 mg/kg/day, cyclophosphamide, levamisole at a dose of 50-150 mg/day 1-2 days a week. Colchicine 1-1.5 mg/day with a 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 administration, administered every 8 hours for 5 days. Plasmapheresis. Prednisolone 1 mg/kg/day.
The prognosis is questionable in severe forms of the disease and when the kidneys are involved (rare).
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