RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164074245 T1 Job Syndrome T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164074245 RD 2024/10/03 AB A genetic immunologic disorder characterized by recurrent skin (eczema-like) and severe lung infections, mainly caused by Staphylococcus aureus, multiple fractures, eczematous dermatitis, coarse facies, and elevated immunoglobulin(Ig)E. The presence of pneumatoceles in association with the skin lesions is a characteristic of this medical condition. In the recent years, other features have been described with this condition and include vascular abnormalities, including coronary aneurysm without atherosclerosis, focal cerebral hyperintensities, and Chiari Type I malformations. Mortality is usually associated to recurrent lung infections in adulthood period.