Syndrome d'hyperimmunoglobulinémie E avec infections récurrentes (syndrome de Job)

Abstract

Hyperimmunoglobulinemia E syndrome with recurrent infections (Job's syndrome). Introduction. — To provide definition and nosology of hyperimmunoglobulinemia E previously termed Job's syndrome or Buckleys' syndrome in the literature. Generalization of IgE dosages makes it more commonly diagnosed, though sometimes diagnosis may not be accurate. Current knowledge and key points. — The clinical picture is dominated by recurring cutaneous and visceral bacterial infections, particularly infections due to staphylococci, and severe generalized eczema, which may be associated with osteoporosis. Biochemistry includes a high level of total and specific IgE and immunological abnormalities dominated by frequent absence of immune antibodies to staphylococci and disorders of neutrophil chemotaxis. Current pathogenic studies show dysregulation of TH1 and TH2 lymphocytes in favor of TH2 activation with depressed TH1 activation, leading to an imbalance in cytokine synthesis. Future prospects and projects. — New therapeutical possibilities with alpha and gamma interferon.