JUVENILE ADAMANTIADES-BEHCETS-DISEASE WIT H DIMINISHED RESPONSE TO STIMULATION WITH ANTI-CD3 MONOCLONAL-ANTIBODY

A 12 year old boy developed the complete symptom complex of Adamantiad es-Behcet's disease over a two-year period. He presented with the muco cutaneous variant with recurrent to persistent oral ulcers which exten ded into the pharynx, recurrent genital ulcers, perianal lesions, and a positive pathergy test. Ocular involvement and other symptoms associ ated with the disease were absent. However, the early onset of the dis ease and the male gender indicated a bad prognosis. The patients fathe r deficiency of suffered from bronchial asthma with recur rent respira tory infections. An IgG-3 subclass was detected. Lymphocyte transforma tion tests showed markedly diminished response to stimulation with ant i-CD3 monoclonal antibody in both patients, while response to PWM, CoA and PHA was normal. In addition, the concentration of serum soluble i nterleukin 6 receptor was reduced in both patients.