J. Deen1, L. Buchanan2, A. Godbolt2
Behcet Disease (BD) is a progressive diffuse inflammatory disorder characterised by recurrent orogenital ulceration, panuveitis and cutaneous inflammation. The underlying pathologic lesion is a small-vessel vasculitis predominately involving venules, however it can astonishingly affect a wide variety of blood vessels of all types and sizes, including both veins and arteries. It is therefore a multisystem polysymptomatic disease with the potential to involve all organs of the body concurrently or sequentially. BD, unlike most forms of vasculitis, can have a genetic predisposition, with the presence of HLA B51 associated with the disease. Approximately only 5% of cases are familial which lead to the possibility that other causative factors play a role in disease development, like infections and environmental exposures.
We report the case of a 58 year-old male with distant Chinese heritage, who presented with recurrent painful oral aphthae, genital aphthae, a papulopustular facial eruption and a panniculitis- like nodular eruption to the trunk. This was associated with migratory monoarthritis of small and large joints. There was no history of pathergy or other systemic involvement. A diagnosis of BD was made based on the Revised International Criteria for Behcet Disease. The patient clinically improved on methotrexate and oral prednisone after failed therapy with azathioprine and mycophenolate mofetil. BD is infrequently seen in adults over 50 years and is rarely seen in non-endemic areas. We report an unusual but interesting presentation of this condition, which responded to systemic treatment.