Background: Hyperimmunoglobulinemia E (Hyper-IgE) syndrome is a rare disease that characterized with
recurrent staphylococcal cutaneous and lung infections with abscess pneumatocele formation,
which begins in infancy and is associated with extremely elevated levels of IgE in serum.
Case Report: Here, a patient with Hyper-IgE syndrome who admitted with staphylococcus aurous pneumonia and pneumatoceles was presented. History revealed that he had multiple episodes of pneumonia
and pustular skin lesions localized on the face, neck and the legs from childhood. Chest x-ray and
computerized tomography of the chest illustrated multiple thin wall cysts with characteristic of
pneumatocele in both side of the lung. The results of the laboratory analysis revealed extremely
high serum IgE level.
Conclusions: This article describes a case of Hyper-IgE syndrome as a cause of recurrent lung infections with multiple abscesses and pneumatoceles formation.

Keywords: Hyper IgE, pneumatocele, recurrent pneumonia