Destructive staphylococcal pleuropneumonia in a two-year-old boy with hyperimmunoglobulin-E syndrome.

Hyperimmunoglobulin-E syndrome (HIES) is a rare immunodeficiency disorder that is characterized by elevated serum concentration of IgE, eosinophilia and severe, recurrent bacterial and fungal infections. Poor regulation of immune system is evident, with decreased production of cytokines, especially interferon. Production of specific antibodies to capsular polysaccharide antigens is decreased Skeletal malformations have been reported in these patients. They can be caused by excessive production of interleukin-4, which may lead to pathologic bony tissue resorption. Due to immune system deficiency and malformations of skeletal and connective tissue, HIES is a multisystem disorder. We present a patient with recurrent bacterial infections since the early age. At the age of two years he presented with severe destructive staphylococcal pneumonia with pleural effusion, pneumatocela formation and pneumothorax. The patient also had a dysmorphic face and skeletal malformations that were most evident at the head. The diagnosis of HIES was made on the basis of elevated serum concentrations of IgE, hypereosinophily, and decreased leukocyte function in vivo and in vitro. Family history of our patient showed an autosomal-dominant inheritance pattern of HIES.