Hyperimmunoglobulinaemia E syndrome: pulmonary imaging features

Abstract

Hyperimmunoglobulinaemia E (hyper-IgE) syndrome (Job's syndrome) is a rare disease that presents with recurrent cutaneous and sinopulmonary infections, and which begins in infancy and is associated with extreme hyper-IgE. The pulmonary imaging features typically consist of recurrent alveolar lung infections, pneumatocoeles and, occasionally, pneumothorax. Various other causes of pneumatocoeles in children can be excluded on the basis of clinical history and other data. Computed tomography may add valuable information for the management of these patients.