CTICasesHyper-IgE Syndrome (Job's Syndrome) with Bronchiectasis
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Imaging findings
High-resolution chest CT demonstrates a few sparse, localized foci of bronchiectasis and minimal air trapping. The chest wall also displays a prominent funnel-shaped chest deformity (pectus excavatum).
Key takeaways
Hyper-IgE syndrome, or Job's syndrome, is a rare primary immunodeficiency characterized by recurrent staphylococcal skin abscesses, cyst-forming pneumonias, and high serum IgE levels. The bronchiectasis seen on imaging represents the permanent sequela of recurrent childhood pulmonary infections. Patients also characteristically exhibit skeletal anomalies, including joint hyperextensibility and chest wall deformities.
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