Pulmonary Manifestations of Hyper IgE Syndrome: Case Series and Literature Review


1 Department of Pediatrics,

2 Chronic Respiratory Disease Research Center, NRITLD, Shahid Beheshti University , MC, TEHRAN-IRAN.

3 Department of Pediatrics


This study aimed to present four children suffering from recurrent pulmonary infections in the context of hyper IgE syndrome. All patients had recurrent pulmonary infections including pneumonia, bronchiectasis, pulmonary abscess and hydropneumothorax. Serum IgE level was greater than 2000 IU/ml in all cases. Microbial cultures showed Staphylococcus aureus and Pseudomonas aeruginosa in two cases. All responded well to the wide spectrum intravenous antibiotics. Extra-pulmonary manifestations included purulent lymphadenitis as well as skin and brain abscesses. Hyper IgE syndrome causes recurrent pulmonary and extra-pulmonary infections which respond fairly well to wide spectrum antibiotic therapy. (Tanaffos 2010; 9(1): 54-58)