Hyper-IgE recurrent infection syndrome

Patients present with atypical eczema and recurrent bacterial (staphylococcal) infections, particularly of the skin. The staphylococcal infection may involve the skin, lungs, joints and other sites. Pneumatocoeles due to staphylococcal infection are a diagnostic feature. Osteopenia due to decreased bone density is another feature and may lead to recurrent fractures. Some patient have 'leonine' facies, they are fair and redheaded. Some present with neutrophils that doesn't function normally. Signs of allergy, e. g. eczema, asthma and/or runny noses, are sometimes present.

Therapeutic options

  • Intravenous immunoglobulins should be used for the antibody deficiency. Antibiotic therapy (10-14 days) together with physiotherapy and postural drainage in case of lung damage. Ciprofloxacin is a valuable antibiotic but is not licensed for small children. Oral poliovaccine should not be given because there is risk of paralytic disease.
  • Hyperimmunoglobulinemia E (Job) Syndrome, eMedicine
  • Job Syndrome, eMedicine

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