Behcet’s disease is a chronic, relapsing inflammatory disorder marked by eye inflammation, oral and genital ulcers, and certain other skin lesions, as well as varying, multisystem involvement including the joints, blood vessels, central nervous system, and gastrointestinal tract.
In 1937, Hulusi Behcet, a Turkish dermatologist, described a disease associating uveitis (inflammation of the uvea, the middle coat of the eye comprising the choroid, ciliary body, and the iris) with genital and oral ulcers. Fifty years later, Behcet’s disease has expanded and is now recognized as a chronic, multisystem disorder with vasculitis (inflammation of blood vessels) as its underlying pathological process.
The cause of Behcet’s disease is unknown. Genetic predisposition, autoimmune mechanisms, and viral infection are under consideration.
Seen most frequently in the Middle East and Asia, the disease occurs twice as often in men between 20 and 30 years old than in women, and is a leading cause of blindness. However, in the U.S. and Australia it is more prevalent in women than in men and is less severe. In the U.S. about 15,000 persons may be affected.
The most frequent sign is aphthous stomatitis, (inflammation of the mucosa of the mouth) with the lesions healing in a few days to a month, but recurring. Similar genital lesions recur less frequently.
Ocular symptoms include posterior uveitis, iridocyclitis, a transient hypopyon (pus in the anterior chamber of the eye), iritis, and chorioretinitis (inflammation of the choroid and retina). Although the ocular lesions may resolve, chronic recurrence of the inflammation may result in partial loss of vision or complete blindness after 4 to 8 years.
Skin hypersensitivity is seen in most Behcet patients with pus and redness of the skin developing within 24 hours at the site of a pinprick into sterile skin. The skin lesions typically resemble erythema nodosum (redness of the skin), most commonly over the legs. Vascular involvement includes thrombophlebitis (venous inflammation) of the large veins and arterial closing and aneurysm (dilation of an artery). Rarely, pulmonary embolism occurs.
The lesions of aphthous stomatitis may be found elsewhere in the gastrointestinal tract. Symptoms vary from mild gastrointestinal discomfort to ulcerative colitis or regional enteritis and malabsorption problems. Arthritis occurs in about two-thirds of patients, most commonly affecting the knees and ankles.
The medical history and physical exam findings are important. Blood tests may be performed. Ophthalmological evaluation may be necessary.
For most patients, Behcet’s disease is not life threatening, and the outlook is dominated by disability due to blindness or neurological lesions, the identification of which is greatly facilitated by magnetic resonance imaging (MRI).
Topical corticosteroids may relieve the pain of oral lesions and lidocaine mouthwash also will alleviate pain. Systemic corticosteroids continue to be useful therapy for most manifestations of Behcet’s syndrome. Corticosteroid therapy often leads to rapid control of skin, mucosal, and joint inflammation.
The management of severe Behcet’s syndrome remains more problematic. Supplemental therapy with other immunomodulatory agents is often necessary to control serious manifestations such as uveitis and meningoencephalitis and to reduce the incidence of long-term steroid toxicity. Drugs that are used include azathioprine, chlorambucil, pentoxifylline, and cyclosporine. One report suggests that plasma exchange may be beneficial in maintaining remission.
The clinical course with any treatment may be chronic but is often characterized by remissions and exacerbations.
What tests need to be done to diagnose the condition?
Is this a primary disorder or secondary to another disorder?
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