[Juvenile Adamantiades-Behçet disease in decreased stimulation with anti-CD3 monoclonal antibody].

A 12 year old boy developed the complete symptom complex of Adamantiades-Behçet's disease over a two-year period. He presented with the mucocutaneous variant with recurrent to persistent oral ulcers which extended into the pharynx, recurrent genital ulcers, perianal lesions, and a positive pathergy test. Ocular involvement and other symptoms associated with the disease were absent. However, the early onset of the disease and the male gender indicated a bad prognosis. The patients father deficiency of suffered from bronchial asthma with recurrent respiratory infections. An IgG-3 subclass was detected. Lymphocyte transformation tests showed markedly diminished response to stimulation with anti-CD3 monoclonal antibody in both patients, while response to PWM, CoA and PHA was normal. In addition, the concentration of serum soluble interleukin 6 receptor was reduced in both patients.