Histopathologic Findings of Pneumatocele in a Patient with Hyper-IgE Syndrome, Compatible with Cystic Adenomatoid Malformation

Hyper-immunoglobulin e syndrome is a rare primary immunodeficiency diseasecharacterized by recurrent abscess formation, respiratory tract infections and very high titersof serum ige associated with peculiar face and skeletal features.we report a seven-year old girl presenting with persistent productive cough and historyof chronic eczematoid facial lesions since infancy and two episodes of hospitalizations dueto pneumonia and perianal abscess. additionally, in physical examination finger tip clubbing,laxity of joints and crackles in both lungs were detected. immunologic work up revealedmarkedly raised ige level and eosinophilia. the patient was diagnosed as hyper igesyndrome based on his clinical and laboratory findings. chest x-ray revealed multiple largecystic lesions in left lung which were confirmed by spiral c'i-scan. pneumonectomyspecimen examination showed cystic adenomatoid malformation, characterized by thepresence of various cysts lined by epithelium in different sizes.there are few reports of cystic adenomatoid malformation in children. to our bestknown, this is the first report of cystic adenomatoid malformation in a child with hyper igesyndrome. early diagnosis and 'surgical therapy are helpful in prevention of repeatedinfections in these patients