Behcet's Syndrome: Clinical, Immunological and Therapeutic Evaluation of 17 Patients†††

Summary: Seventeen patients with Behcet's syndrome were evaluated clinically, immunologically and therapeutically. The variable and extensive clinical manifestations included scantyfungiform papillae in six patients, bullous skin eruption in two, non-specific skin sensitivity in six, bladder ulceration in two, and psychoneurosis. Immunological abnormalities included raised α2 globulin in 14 patients, hypogammaglobulinaemia in one, cryoproteins in three, foetal oral mucosa antibodies in three and pemphigus antibody in two. Intradermal injection of autologous plasma produced an Arthus reaction in eight out of 11 patients tested. It is suggested that some plasma component may be responsible for vasculitis in these patients. Treatment was attempted with various combinations of corticosteroid, cyclophosphamide, phenformin and ethyloestrenol. Seven patients had a satisfactory response in that all manifestations of their syndrome were well controlled.