Behc¸et’s syndrome
A chronic, relapsing multisystem vasculitis characterised by oral ulceration.
Rare.
Commonest in 20–40 years.
M > F
Much more common in Turkey, Iran, China, Korea and Japan.
It is thought to be the result of an environmentally triggered autoimmune reaction in a genetically susceptible individual.
Patients have recurrent oral aphthous or herpetiform ulcers. Other manifestations include genital ulceration, ocular disease (uveitis), skin lesions (erythema nodosum), arthritis, gastrointestinal upset, renal, lung and neuro-logical involvement. Patients demonstrate pathergy (a papule or pustule forms at sites of skin puncture) – this is a highly specific finding.
Corticosteroids and immunosuppressive agents are used for severe disease. Colchicine may be of benefit for erythema nodosum and arthralgia.
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