Hyperimmunoglobulinemia E syndrome: pulmonary imaging considerations

The Hyper IgE syndrome is a rare disease consisting of recurrent sinusitis and pneumonia, pneumatoceles, chronic dermatitis, and elevated serum levels of IgE. The primary radiographic abnormalities are recurrent alveolar lung disease and pneumatoceles. Pneumothorax may occasionally occur as in one of our cases. Other causes of pneumatoceles are usually easily excluded by the history and other clinical data. Pulmonary scintigraphy and computed tomography may add information valuable to the management of these patients.