Behcet's (beh-CHETS) disease, also called Behcet's syndrome, is a rare disorder that causes chronic inflammation in blood vessels throughout your body.
The exact cause of Behcet's is unknown, but it may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells.
Both genetic and environmental factors may be responsible for Behcet's disease.
The inflammation of Behcet's disease leads to numerous symptoms that may initially seem unrelated. The signs and symptoms of Behcet's disease — which may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores — vary from person to person and may come and go on their own.
The diagnosis of Behcet’s disease depends on the recognition of the clinical manifestations of the disease previously described. These may not all appear at the same time.
There are no diagnostic laboratory tests. Complete blood counts and blood chemical analysis are within normal limits.
The plasma globulins may be slightly elevated, and the erythrocyte sedimentation rate also is generally elevated. The common histopathologic lesion of all of the clinical manifestations is a vasculitis characterized by perivascular infiltrates with mononuclear cells, resembling the lesion seen in a delayed hypersensitivity reaction to an intradermal antigen.
Therapy of this disease is unsatisfactory and difficult to evaluate, in part because of the unpredictability of the natural course of Behcet’s disease.
Some investigators begin therapy with oral prednisone 50 mg daily. This drug is then tapered over several days to a maintenance dose between 5 and 20 mg. Other investigators add cytotoxic agents such as azathioprine to the prednisone.
O’Duffy et al. recently reported favorable responses in patients with uveitis and meningoencephalitis whose Behcet’s disease was treated with 0.1 mg/kg daily doses of chlorambucil for periods of 3 months to 4 years. However, no controlled studies are available that identify ideal medical therapy.
The exact cause of Behcet's is unknown, but it may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells.
Both genetic and environmental factors may be responsible for Behcet's disease.
The inflammation of Behcet's disease leads to numerous symptoms that may initially seem unrelated. The signs and symptoms of Behcet's disease — which may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores — vary from person to person and may come and go on their own.
Diagnosis :
The diagnosis of Behcet’s disease depends on the recognition of the clinical manifestations of the disease previously described. These may not all appear at the same time.
There are no diagnostic laboratory tests. Complete blood counts and blood chemical analysis are within normal limits.
The plasma globulins may be slightly elevated, and the erythrocyte sedimentation rate also is generally elevated. The common histopathologic lesion of all of the clinical manifestations is a vasculitis characterized by perivascular infiltrates with mononuclear cells, resembling the lesion seen in a delayed hypersensitivity reaction to an intradermal antigen.
Treatment :
Therapy of this disease is unsatisfactory and difficult to evaluate, in part because of the unpredictability of the natural course of Behcet’s disease.
Some investigators begin therapy with oral prednisone 50 mg daily. This drug is then tapered over several days to a maintenance dose between 5 and 20 mg. Other investigators add cytotoxic agents such as azathioprine to the prednisone.
O’Duffy et al. recently reported favorable responses in patients with uveitis and meningoencephalitis whose Behcet’s disease was treated with 0.1 mg/kg daily doses of chlorambucil for periods of 3 months to 4 years. However, no controlled studies are available that identify ideal medical therapy.
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