Moderate-dose intravenous immunoglobulin treatment of Job's syndrome. Case report

Minerva Med. 2000 May-Jun;91(5-6):113-6.

Abstract

Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic "cold" abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after IVIG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Adult
  • Chemotaxis, Leukocyte
  • Female
  • Fluorometry
  • Humans
  • Immunoglobulin E / blood
  • Immunoglobulins, Intravenous / administration & dosage*
  • Job Syndrome / complications
  • Job Syndrome / immunology
  • Job Syndrome / therapy*
  • Neutrophils
  • Pneumonia, Staphylococcal / diagnosis
  • Pneumonia, Staphylococcal / etiology
  • Pneumonia, Staphylococcal / therapy
  • Radiography, Thoracic

Substances

  • Immunoglobulins, Intravenous
  • Immunoglobulin E