Number VII Behçet's disease (Adamantiades syndrome).

Behçet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations may also be seen. BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101). Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated. An inflammatory disorder, BS is now considered as a systemic vasculitis, characterised by a very wide spectrum of clinical features and by unpredictable exacerbations and remissions.