Background: Hyperimmunoglobulinemia E syndrome (Hyper-IgE syndrome) is an idiopathic primary immunodeficiency disorder, 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. Other findings that are associated with this disorder are: coarse facies, eosinophilia, and skeletal abnormalities. According to variability of findings in this disorder that easily may be mistaken with other rare immunodeficiency syndromes, in this article a case of Hyper-IgE syndrome that was referred to Kashan Shahid Beheshti Hospital in 2002 is presented.

Case report: A 30 years old man was admitted with history of fever, dyspnea and purulent cough. He had multiple episodes of pneumonia and pustular skin lesions since childhood. In physical examination multiple skin scars on the face, neck and the legs were observed. Chest radiography revealed multiple thin-walled pulmonary cysts suggestive of pneumatoceles. Computed tomography of the chest showed bilaterally pneumatocele with abscess formation. Considering systemic and recurrent infections, evaluation of immunodeficiency state in-patient was performed and according to extremely elevated levels of IgE in serum and eosinophilia, the diagnosis of Hyper-IgE syndrome was confirmed. Treatment with intravenous antistaphilococcal antibiotics was done and after treatment of acute stage, the patient underwent longterm oral prophylaxis with co-trimoxasole and cloxacillin. In follow- up he was doing well.

Conclusion: It is necessary to consider the diagnosis of Hyper-IgE syndrome according to clinical findings and recurrent skin infections from infancy and pneumatoceles associated with extremely elevated levels of IgE in serum. It is recommended that after treatment of acute stage, prophylaxis with oral antistaphylococcal antibiotics is continued.