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ESSENTIALS OF DIAGNOSIS
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Most commonly occurs among persons of Asian, Turkish, or Middle Eastern background, but may affect persons of any demographic profile
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Recurrent, painful aphthous ulcers of the mouth and genitals Additional cutaneous findings include erythema nodosum like lesions, a follicular rash, and the pathergy phenomenon (formation of a sterile pustule at the site of a needle stick) Either anterior or posterior uveitis Posterior uveitis may be asymptomatic until significant damage to the retina has occurred Variety of neurologic lesions that can mimic multiple sclerosis, particularly through involvement of the white matter of the brainstem
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Central nervous system involvement is another cause of major potential morbidity in Beh et s disease The central nervous system lesions that may mimic multiple sclerosis radiologically often result in serious disability or death Findings include sterile meningitis (recurrent meningeal headaches associated with a lymphocytic pleocytosis), cranial nerve palsies, seizures, encephalitis, mental disturbances, and spinal cord lesions Finally, patients with Beh et s disease have a hypercoagulable tendency that may lead to complicated venous thrombotic events, particularly multiple deep venous thrombosis, pulmonary emboli, cerebral sinus thrombosis, and other problems associated with clotting The clinical course may be chronic but is often characterized by remissions and exacerbations
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Named after the Turkish dermatologist who first described it, this disease is of unknown cause Essentially all of its protean manifestations, however, are believed to result from vasculitis that may involve all types of blood vessels: small, medium, and large, on both the arterial and venous side of the circulation
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B Laboratory Findings
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There are no pathognomonic laboratory features of Beh et s disease Although patients with Beh et s disease often have elevated acute phase reactants, there is no autoantibody or other assay that is distinctive No markers of hypercoagulability specific to Beh et s have been identified Beh et s disease is known to have a genetic risk factor (HLA B51), but this gene is neither necessary nor sufficient to cause the disease and is of little help in making the diagnosis or determining prognosis
Clinical Findings
A Symptoms and Signs
The hallmark of Beh et s disease is painful aphthous ulceration in the mouth These lesions, which usually occur multiply, may be found on the tongue, gums, and inner surfaces of the oral cavity Genital lesions, similar in appearance, are also common but do not occur in all patients Other cutaneous lesions of Beh et s disease include tender, erythematous, papular lesions that resemble erythema nodosum (On biopsy, however, many of these lesions are shown to be secondary to vasculitis rather than septal panniculitis) These erythema nodosum like lesions have a tendency to ulcerate, a major difference between the lesions of Beh et s disease and the erythema nodosum seen in cases of sarcoidosis and inflammatory bowel disease An erythematous follicular rash that occurs frequently on the upper extremities may be a subtle feature of the disease The pathergy phenomenon is frequently underappreciated (unless the patient is asked); in this phenomenon, sterile pustules develop at sites where needles have been inserted into the skin (eg, for phlebotomy) in some patients A nonerosive arthritis occurs in about two-thirds of patients, most commonly affecting the knees and ankles Eye involvement may be one of the most devastating complications of Beh et s disease Posterior uveitis, in essence a retinal venulitis, may lead to the insidious destruction of large areas of the retina before the patient becomes aware of visual problems Anterior uveitis, associated with photophobia and a red eye, is intensely symptomatic This complication may lead to a hypopyon, the accumulation of pus in the anterior chamber If not treated properly with mydriatic agents to dilate the pupil and corticosteroid eyedrops to diminish inflammation, the anterior uveitis of Beh et s may lead to synechial formation between the iris and lens, resulting in permanent pupillary distortion
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