[Behçet's syndrome. Possible differential diagnosis in multi-system symptoms]

Behçet's syndrome was described as a triad with recurrent ulcers of the mouth, aphthae of the genitals as well as changes of the eyes. A fourth later added main criterion comprises multifarious dermatological symptoms. Facultative signs of a disease, which, however, occasionally may stand also in the centre of the clinical picture are vascular, gastrointestinal and central-nervous participations, arthritic pains and swellings as well as an epididymitis. On account of the infrequency of the disease outside the endemic regions and the development of the individual manifestations over larger periods it is often recognized very late. Out of the six casuistics described three of them fulfilled the criteria of a complete Behçet's syndrome with evidence of all four main criteria, in the remaining casuistics the incomplete clinical picture was present with three main and one to four side criteria. In all cases the course was relatively benign. Recently, streptococcal antigens were considered to be etiological factors; correlations with the HLA-system are ascertained for patients from endemic regions. In phases of clinical activity the combination of immunosuppressive agents with cortisonoids is the therapy of choice.