CTICasesJob's Syndrome (Hyper-IgE Syndrome) with Chronic Staphylococcal Infections
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Imaging findings
A 22-year-old male with Job's syndrome presented with a dramatic radiograph showing large cystic lesions or pneumatoceles with air-fluid levels and chronic scarring in the right lower lobe. CT revealed extensive fluid-filled lesions within a background of severe bronchiectasis.
Key takeaways
This patient with Job's syndrome (hyper-IgE syndrome) had recurrent staphylococcal infections of the skin and lungs. The syndrome, named after the biblical figure Job, is due to a genetic mutation in the JAK-STAT pathway, with hyper-IgE being a symptom rather than the cause. Patients often have other abnormalities, including persistent primary teeth and characteristic facial features. The predilection for skin and lung infections is related to IL-17 regulation in neutrophils and lymphocytes in these specific tissues. The patient had a left lower lobectomy at age 1 for what was likely misdiagnosed ABPA, and non-compliance with prophylactic antibiotics contributed to severe recurrent infections. This case represents a very extreme manifestation of Job's syndrome.
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