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A multisystem, inflammatory, relapsing, and chronic disorder that may include mucocutaneous, ocular, genital, articular, vascular, CNS, and GI involvement. Etiology is unknown; however, histopathologic vasculitic changes are common to all involved organs. Immunologic (including autoimmune) and viral causes, and an HLA-related immunogenetic predisposition, have been suggested. The syndrome generally begins in the 3rd decade and occurs twice as often in men as in women. Some cases have been reported in children. The syndrome is more common in Mediterranean and certain Asian countries, especially Japan. Although uncommon in the USA, Beh?et's syndrome must be considered frequently in differential diagnosis.
Symptoms and Signs
Almost all patients have recurrent painful oral ulcers resembling those of aphthous stomatitis and, in most patients, these ulcers are the first manifestations of the disease. Similar ulcers occur on the penis and scrotum, where they are painful, or on the vulva and vagina, where they may be asymptomatic. Other symptoms follow in days to years. Ocular disease occurs in most cases: The most common is a relapsing iridocyclitis, sometimes with hypopyon and often initially presenting as pain, photophobia, and hazy vision. The posterior segment also may be involved, with choroiditis, retinal vasculitis, and papillitis. Untreated posterior uveitis may cause blindness. Various skin lesions occur in 80% of cases: papules, pustules, vesicles, and folliculitis. Particularly suggestive are erythema-nodosum-like lesions and (in about 40% of patients) inflammatory reactions to minor trauma, eg, needle punctures. A relatively mild, self-limiting, and nondestructive arthritis involving the knees and other large joints occurs in 50% of patients. Recurrent superficial or deep migratory thrombophlebitis develops in 25% and may lead to vena caval obstruction. CNS involvement (18%) may present as chronic meningoencephalitis, benign intracerebral hypertension, or life-threatening brainstem and spinal cord lesions. GI manifestations vary from nonspecific abdominal discomfort to a syndrome resembling regional enteritis (Crohn's disease). The generalized vasculitis may cause aneurysms or thrombosis and also may involve the kidneys in the form of a usually asymptomatic focal glomerulonephritis. The lungs rarely are involved, with vasculitis and aneurysms of the pulmonary arteries.
Diagnosis
Reiter's syndrome, Stevens-Johnson syndrome, SLE, regional enteritis (Crohn's disease), ulcerative colitis, ankylosing spondylitis, and herpes simplex infection, especially with recurrent aseptic meningitis. Beh?et's syndrome has no specific findings that exclude all alternative possibilities, but often is distinguished by the relapsing course and multiple organ involvement.
Laboratory abnormalities are nonspecific but characteristic of inflammatory disease (elevated ESR and alpha2-and gamma-globulins, and mild leukocytosis). Numerous immunologic abnormalities may be detected, including the presence of autoantibodies to affected tissues and of circulating immune complexes.
Prognosis and Treatment
The syndrome generally is benign but with periods of remission and relapse that may last from weeks to years and even extend over several decades. Blindness, vena caval obstruction, and paralysis may complicate the course; the occasional fatalities usually are associated with neurologic, vascular, and GI involvement. Symptomatic therapy of the various manifestations is reasonably successful. Needle punctures should be avoided when possible, since they provoke inflammatory skin lesions. Topical corticosteroids may provide temporary symptomatic relief for ocular and oral disease. However, topical or systemic corticosteroids do not alter the frequency of relapses. Occasional patients with severe uveitis or CNS involvement will respond to high doses of systemic corticosteroids (prednisone 60 to 80 mg/day). Patients with posterior uveitis that does not respond to prednisone should be treated with cyclosporine, initially 5 mg/kg/day increasing incrementally up to 10 mg/kg/day until a therapeutic effect is observed; trough levels of cyclosporine should be maintained between 50 and 200 ng/mL.
RELAPSING POLYCHONDRITIS
Fluid & Electrolyte
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