Behçet's disease - a kind of clinical form of systemic vasculitis characterized by recurrent erosive and ulcerative lesions of the mucous membranes of the mouth and \ or the genitalia, in combination with two or more syndromes - cutaneous, articular, involving the eye, gastrointestinal tract and nervous system. This is the only systemic vasculitis, which may develop secondary amyloidosis. The disease is caused by immuno-genetic: no significant association with BB antigens HLA-B5, B12, B51.
To a large clinical signs of Behcet's disease include four types of changes:
oral - in the form of a deep and very painful canker sores, gingivitis, glossitis, pharyngitis;
eye - hypopyon, horeoretinit, iridocyclitis, and often panuveit with progressive decline of vision;
Necrotic genital ulceration and subsequent scarring rude;
articular syndrome in the form of asymmetric monooligoartrita middle joints without the development of destructive changes;
erosive and ulcerative lesions of the digestive tract throughout;
thrombophlebitis of large veins - the upper and lower hollow vein, severe CNS involvement - meningoencephalitis, polyneuropathy, dementia.
The beginning may be acute or gradual. It is essential that as a child of the first symptoms of the disease ("unmotivated" fever, headaches, recurrent stomatitis) to the expanded picture of the disease Behcet's may take several years (from 1 to 10 years).
Behcet's disease during highly variable. Typically the undulating course with more frequent relapses in the first years of the disease and rare exacerbation of disease after 5-7 years. In children, secondary renal amyloidosis and bowel is rare. Risk factors for onset of amyloidosis believe in the juvenile age, male gender, the full form of the disease, the severity and duration of illness. There are cases of the rapid development of amyloidosis in 1.5-2 years. In addition to amyloidosis Behcet's disease may be complicated by pulmonary artery aneurysm, intracranial arterial aneurysms, thrombosis of the lower and upper hollow vein. Spend hormone therapy, cytotoxic drugs administered, symptomatic therapy.