Hadian Fatemeh, Jaker Sams, Helm Emma
Final Pr. ID: Poster #: CR-029
Hyperimmunoglobulin E syndrome, or Job syndrome, is a rare primary immunodeficiency disorder characterised by a clinical triad of eczema, recurrent skin and lung infections, and high serum IgE that often presents in childhood. Hyper IgE syndrome is most commonly caused by a STAT3 genetic mutation, which impairs the appropriate inflammatory response leading to recurrent infections. Respiratory infections are usually staphylococcal and less commonly due to H. Influenzae and S. pneumoniae. Complications include lung abscess formation, bronchiectasis, pneumatocoeles and secondary opportunistic infections including aspergillosis.
We present radiographic and CT imaging findings of a series of three pediatric patients who presented to our hospital with varying degrees of severity and complications.
Case 1 is a 3 year old child who initially presented via dermatology with a postauricular abscess and recurrent scalp infections. They went on to develop a large multiloculated pneumatocoele further complicated by a large hydropneumothorax.
Case 2 is a 4 year old child who presented with eczema since infancy; further history revealed multiple staphylococcal abscesses including a liver abscess at 6 months of age requiring ITU admission. Imaging revealed cystic bronchiectasis and multiple pneumatocoeles. They went on to be investigated for fungal lung infection.
Case 3 is an 11 year old child who presented with respiratory tract symptoms and pleuritic chest pain. Imaging revealed large lung abscesses, which resolved over serial imaging. They went on to develop varicose bronchiectasis and allergic bronchopulmonary aspergillosis.
Early diagnosis is crucial in children to preserve respiratory function and improve longterm survival. The radiologist should be aware of the radiological manifestations and complications of Hyper IgE syndrome, particularly in the respiratory tract, as complications of respiratory infection are among the most common causes of mortality. Read More
Authors: Hadian Fatemeh , Jaker Sams , Helm Emma
Keywords: Job, hyperimmunoglobulin