Background Hyperimmunoglobulin E syndrome (HIES) is a rare desease caused by a primary complex immunodeficiency, with IgE serum levels over 2,000U/mL. The syndrome is characterized by the following triad: 1) recurrent staphylococcal abscesses, 2) recurrent airway infections; 3) elevated serum IgE concentration. Our pacient presented signs that are compatible with HIES in distinct moments of his...

the hyperimmunoglobulin e syndromes (hiess) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. hies are characterized by the classic triad of high serum levels of immunoglobulin e (ige), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. most cases of hies are s...

We report the case of a 34 year old woman with acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC) due to methicillin-resistant Staphylococcus aureus (MRSA) sepsis with hyperimmunoglobulin E syndrome (HIES). Although chemotactic activity of neutrophils was impaired in this patient, neutrophils accumulated in the lungs as assessed by bronchoalveolar lavage...

Job's syndrome or Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent soft tissue infections, coarse face, skeletal and vascular abnormalities, and markedly high levels of Immunoglobulin E. Eczema that resembles atopic dermatitis but is refractory to traditional treatment and severe and recurrent bacterial pneumonias often recognized during childh...