[Non-infectious oral aphthous disorders. 2. Behçet's syndrome (malignant aphthosis)]

Behçet's (Behçet-Touraine's resp.) disease is a very serious systemic vasculitis exhibiting the clinical features of simultaneously or successively recurrent oral aphthosis, genital lesions and ophthalmitis (mainly of uveitis type). During relapsing acute phases of generalisation multifocal visceral, arthritic and/or neurological manifestations with life-threatening complications may develop. The disease which in some geographic areas (Japan, Near East) is observed with rather high prevalence rates, is also assumed to occur in Central Europe more frequently as being diagnosed so far. As to early recognition, the recurrent oral aphthosis spread in the entire oro-pharynx ("malignant aphthosis") is of importance. The present view of aetiopathogenesis deals with an abnormal increase of circulating immune complexes (mainly IgG complexes) against mucosal and/or microbial antigens, which accumulate in both small and larger blood vessels elicidating a severe immunocomplex vasculitis with necrotising tissue damage. For therapeutic management systemic high dose treatment with corticosteroids including immunosuppressive drugs is mandatory.