Abstract
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 childhood. Early diagnosis and treatment prevent progressive pulmonary sequellae and increase survival. About 200 cases of HIES has been reported worldwide. The authors report a new case of HIES with one of the worst pulmonary sequellae found in the literature on this subject and review this infrequent topic.
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