ABSTRACT
Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum IgE associated with peculiar face and skeletal features. We report a seven-year old girl presenting with persistent productive cough and history of chronic eczematoid facial lesions since infancy and two episodes of hospitalizations due to pneumonia and perianal abscess. Additionally, in physical examination finger tip clubbing, laxity of joints and crackles in both lungs were detected. Immunologic work up revealed markedly raised IgE level and eosinophilia. The patient was diagnosed as hyper IgE syndrome based on his clinical and laboratory findings. Chest X-ray revealed multiple large cystic lesions in left lung which were confirmed by spiral CT-scan. Pneumonectomy specimen examination showed cystic adenomatoid malformation, characterized by the presence of various cysts lined by epithelium in different sizes. There are few reports of cystic adenomatoid malformation in children. To our best known, this is the first report of cystic adenomatoid malformation in a child with hyper IgE syndrome. Early diagnosis and surgical therapy are helpful in prevention of repeated infections in these patients