[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.
Krüger
K
K
Klinikum Benjamin Franklin, Freie Universität, Berlin.
Fritz
K
K
Daniel
V
V
Zouboulis
C C
CC
D000911:Antibodies, Monoclonal / Q000276:immunology*; D001249:Asthma / Q000235:genetics / Q000276:immunology / Q000473:pathology; D001528:Behcet Syndrome / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D017252:CD3 Complex / Q000276:immunology*; D002648:Child; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D017099:IgG Deficiency / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D007074:Immunoglobulin G / Q000097:blood*; D008213:Lymphocyte Activation / Q000235:genetics / Q000276:immunology; D008297:Male; D009504:Neutrophils / Q000276:immunology / Q000473:pathology; D010641:Phenotype; D012867:Skin / Q000276:immunology / Q000473:pathology
D000911:Antibodies, Monoclonal; D017252:CD3 Complex; D006680:HLA Antigens; D007074:Immunoglobulin G