[Behçet's disease]

Behçet's disease is a systemic vascularitis appearing primarily by an oral and/or genital aphtose and an uveitis, the unit constituting the tripod of call of the diagnosis having led to its initial description. But its clinical demonstrations are protean with other quasi-specific cutaneous signs and a whole panel of organic attacks, the complicated syndromic forms being the entero-, the angio- and the neuro-Behçet. Its diagnosis is sometimes difficult in the absence of clinical or biological specific signs, in particular when the tripod diagnosis is incomplete. The prognosis of this disease remains heavy, in particular on the ophthalmologic functional level, because of the absence of specific curative treatment, its etiology remaining ignored to date. However, ongoing therapeutic progress are observed for severe and refractory forms to conventional immunosuppressive drugs with the advent of anti-TNFalpha drugs of which the precise place in the therapeutic arsenal, still badly codified, and the long-term effectiveness remain to be specified.