Symptoms and Signs
Oral ulceration tends to be the earliest manifestation of Behcet's Disease and often appears before any other signs or symptoms. The ulcers are painful, shallow or deep, round or oval, and may have a white or yellow base with a red halo. Their size varies from 1-20 mm. The ulcers generally affect the buccal mucosa, tongue, lips, gingiva, tonsils, uvula, palate or the pharynx. Multiple lesions may be present during flares, but some patients may experience a single ulcer, while others may have multiple ulcers. The lesions heal within 10-20 days without scarring.
Genital ulcers occur slightly less often than oral ulcers, developing in approximately 75% of patients. The lesions tend to be larger and deeper and often heal with scarring. In males, the ulcers generally appear on the scrotum and less frequently on the shaft or on the glans (head) of the penis. Genital lesions in men may be associated with epididymitis. In females, the lesions may affect the vulva, the vagina, and the cervix.
Cutaneous manifestations of Behcet's Disease generally occur in 60-90% of patients. Erythema nodosum, an inflammation occurring in the fatty layer of the subcutaneous tissue, causes red tender nodules to form under the skin. These nodules frequently ulcerate, unlike the erythema nodosum lesions which occur with other diseases. Erythema nodosum occurs more commonly in women. Other cutaneous manifestations include acneiform nodules, pseudofolliculitis and papulopustular lesions. In males, the lesions commonly develop over the neck and face.
Ocular inflammation is one of the hallmark manifestations of Behcet's Disease. Behcet's Disease is one of a few autoimmune diseases capable of causing both anterior and posterior uveitis. Anterior uveitis (inflammation of the uveal tract in the front portion of the eye) can present with pain, blurred vision, light sensitivity, and excessive production of tears. Many cases occur in healthy individuals. Anterior uveitis is the most common form of uveitis predominantly occurring in the young and middle aged population. Anterior uveitis may be intense and present with hypopion (a grossly visible layer of pus in the anterior chamber of the eye).
Posterior uveitis (inflammation of the uveal tract of the back of the eye) is less common and may involve the retina and the choroid. It can present with blurred vision, floaters, eye pain, redness and sensitivity to light. In addition to Behcet's Disease, there are many infectious and non-infectious causes which may result in posterior uveitis. Posterior uveitis can only be detected during a complete eye exam and must be treated in order to avoid loss of vision and other serious complications. Early diagnosis and treatment of recurrent or persistent ocular inflammation is essential, as eye inflammation, if left untreated, is one of the leading causes of disability in Behcet's patients.
Peripheral arthritis or spondylitis develops in approximately 50% of patients with Behcet's Disease. The peripheral arthritis may be monoarticular (affecting only one joint) or polyarticular (affecting multiple joints). Arthritis causes pain, swelling and stiffness of the joints in the knees, ankles, wrists and elbows. The spondylitis usually presents as sacroilitis ( with low back or buttock pain). Arthralgias from Behcet's Disease last days to weeks and do not cause permanent damage. The peripheral arthritis is usually not deforming.
Gastrointestinal involvement develops in approximately 25% of Behcet's patients. The signs tend to appear years after the onset of oral ulcers, but gastrointestinal involvement may occur at any time. GI involvement may present with ulcers of the ileum and cecum, but any portion of the gastrointestinal tract, from mouth to anus, may be involved. The most frequent manifestations are anorexia (lack of appetite), pain, vomiting, diarrhea, and rectal bleeding. Esophageal ulcerations appear to be more common in American patients. The predilection for Behcet's Disease to involve veins may result in Budd-Chiari syndrome in some patients. Careful evaluation is essential to rule out other diseases which affect the gastrointestinal tract..
Lung involvement is less common, but may occur in Behcet's Disease. Lung involvement may present with cough, dyspnea (shortness of breath), and rarely, pulmonary artery aneurysms. Aneurysms (outpouching of blood vessel walls) of the lungs can at times rupture, and produce lung hemorrhage.
Central Nervous System
Central nervous system involvement may be more common in American and European Behcet's patients, affecting as many as 30%. The disease tends to involve the white matter of the brain and the brain stem. The neurologic features are variable and include headache, confusion, strokes, personality changes and memory loss. Central nervous system symptoms in Behcet's Disease may be due to aseptic meningitis or lesions resulting in focal brain dysfunction.
Large vessel vasculitis may produce bruits in the chest or abdomen. Clinically important cardiac disease develops infrequently. Renal disease rarely occurs. MAGIC Syndrome (mouth and genital ulcers with inflamed cartilage) is diagnosed in patients with features of both Behcet's Disease and relapsing polychondritis.
Credits: NINDS, NIH, ABDA. Images courtesy of Johns Hopkins University, Division of Rheumatology. Copyrights reserved.