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Behçets syndrome is a multisystem disorder presenting with recurrent oral and/or genital ulcerations, chronic relapsing uveitis that may cause blindness and neurologic impairments. Although there is no invariable feature of Behçets disease, certain features occur often enough to constitute a definable syndrome and serve as the basis for diagnostic criteria.
The diagnostic criteria established by the International Study Group for Behçets disease requires the presence of recurrent oral ulceration, in the absence of other clinical explanations, and two of the following: recurrent genital ulceration, eye lesions, skin lesions and/or a pathergy test (table I) . The pathergy test is the pustular reaction of the skin in response to intradermal needle prick. This nonspecific skin inflammatory reactivity denotes increased neutrophil chemotaxis and was once thought to be pathognomonic of Behçets disease. However, this reaction occurs in no more than 70% of the patients, usually in those with more extensive disease.
The recurrent oral ulcers are a sine qua non for the diagnosis. The ulcers are usually painful, are round or oval, are 2 to 10 mm in diameter, are shallow or deep with a central yellowish necrotic base, are found anywhere in the oral cavity and may appear singly or in crops, which is more common. These ulcers persist for 1 to 2 weeks and subside without leaving scars. The genital ulcers resemble the oral ones, however, isolated genital ulcers are seldom indicative of Behçets disease. While vaginal ulcers are usually painless, those on the external genitalia may be painful.
Ulcers occur elsewhere, as in the gut and on the skin. In addition, an assortment of nonulcerative skin lesions may be present such as folliculitis, erythema nodosum, photosensitivity, an acne-like exanthem and infrequently vasculitis.
Eye involvement is the most dreaded complication, since it may result in blindness. Decreased visual acuity results from inflammation, secondary glaucoma, cataracts, or vitreous hemorrhage. In addition, iritis, retinal vessel occlusions and optic neuritis can be found. Hypopyon uveitis (pus in the anterior chamber of the eye), which is considered the hallmark of Behçets disease, is in fact a rare manifestation.
The arthritis of Behçets disease is usually intermittent, self-limited, not deforming and localized to the knees and ankles. Phlebitis or arteritis occurs in as many as one-fourth of the patients and predisposes to thrombosis or aneurysms. The superior vena cava is obstructed occasionally; pulmonary embolus is a rare complication. Pulmonary vasculitis has been reported in approximately 5% of patients and produces dyspnea, cough, hemoptysis, chest pain and infiltrates on chest roentgenograms. It is a significant cause of death.
The nervous system involvement may be acute or gradual in onset, and they may resolve completely or cause death. Aseptic meningitis occurs in almost all cases of neurologic Behçets disease. Other common lesions are benign intracranial hypertension, a multiple sclerosis-like picture, psychiatric disturbances and pyramidal involvement.
Although it has a worldwide distribution, the Behçets disease is rare in the Americas and Europe and is more prevalent in Turkey and the Middle and Far East. It affects mainly young adults, with men having more severe disease than women.
Abnormalities of the immune system are inconstant, providing no clues to the cause and pathogenesis of Behçets disease, which remain unknown. Since vasculitis is the main pathologic lesion and circulating autoantibodies to human oral mucous membrane are found in approximately 50% of the cases, it is considered an autoimmune disease.
The severity of the syndrome abates with time. The life expectancy seems to be normal, except in the cases with neurologic complications. The only serious complication is blindness. Young male patients are at increased risk for ocular complications and require aggressive medical management.
Treatment of Behçets disease is symptomatic and empirical. Arthritis responds to analgesics and rest. Mucous membrane involvement may respond to topical corticosteroids in the form of paste or mouthwash.
Immunosuppressants are considered to be the main line of treatment for eye involvement. Azathioprine has shown to maintain visual acuity and to prevent the emergence of new eye disease. Cyclosporin A is an effective alternative to azathioprine. However, nephrotoxicity, the rather regular relapses with cessation of therapy and the high cost of the drug often limit its use.
The therapy of CNS lesions usually entails high dose glucocorticosteroids and immunosuppressants. Gastrointestinal involvement may be managed with sulfasalazine.
Criteria For The Diagnosis of Behçet's Syndrome |
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| In the absence of other clinical explanations, patients must have: 1. Recurrent Oral Ulcerations (aphthous or herpetiforme) recurring at least three times in one 12-month period; and two of the following: 2. Recurrent Genital Ulceration. 3. Eye Lesions: anterior or posterior uveitis, cells in the vitreous by slit lamp examination or retinal vasculitis observed by an ophthalmologist. 4. Skin Lesions: erythema nodosum, pseudofolliculitis, papulopustular lesions or acneiform nodules in postadolescent patients not on corticosteroids. 5. Pathergy, read at 24-48 hours. |
1. Yazici H. Behçets Syndrome. In:Klipple JH, Dieppe PA, eds. Pratical Rheumatology. London: Mosby; 1995:393-394
2. Moutsopoulos HM. Behçets Syndrome. In: Isselbacher K J, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL, eds. Harrisons Principles of Internal Medicine, 13E. United States of America, McGraw-Hill Inc; 1994:1669-1670.
3. Ball EV. Behçets Syndrome. In: Bennett JC, Plum F, eds. Cecil Textbook of Medicine, 20E. Philadelphia, WB Saunders; 1996:1506-7.
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