Hyper Immunoglobulin E (IgE); an immune deficiency (Job Syndrome)

Abstract

Hyper-IgE Syndrome (HIES), also known as Job Syndrome; is a rare genetic disorder that affects the immune system and can lead to various health complications. The text mentions cornerstone of HIES therapy is proper skin care, infection prevention, and aggressive infection treatment. Treatments for HIES can include antibiotics that target S. aureus, typically used to reduce the incidence of pneumonia and the risk of lung damage. Skincare routines can involve bleach baths or chlorhexidine washes, oral anti-staphylococcal prophylactic antibiotics, and other treatments to avoid abscesses. Antifungal prophylaxis may be beneficial for HIES patients who have chronic Candida infections. Defects in antibody synthesis can vary, making it difficult to provide general recommendations for immune globulin replacement in HIES.