Snapshot A 3-year-old boy with a history of multiple infections presents with an intensely itchy rash. His skin is covered with an erythematous, crusty, scaly rash. Excoriations are seen where he scratched his skin. His face has thickened skin (coarse facies) and a wide-set nose. He has several abscesses without any pus formation over his arms. Labs come back with IgE levels of 3000 IU/mL. Introduction Primary immunodeficiency syndrome from defect in neutrophil chemotaxis Also known as Job syndrome Also known as hyperimmunoglobulinemia E syndrome (HIES) Genetics login to view 2 more bullets Pathogenesis login to view 4 more bullets characterized by FATED login to view 5 more bullets Presentation Symptoms login to view 1 more bullet Physical exam login to view 10 more bullets Evaluation ↑ IgE ↑ eosinophils ↓ IFN-γ Differential Atopic dermatitis Wiskott-Aldrich syndrome SCID Treatment Skin care for eczematoid dermatitis Prophylactic trimethoprim-sulfamethoxazole to prevent infections Antibiotics to treat infections Prognosis, Prevention, and Complications Prognosis login to view 2 more bullets Complications login to view 1 more bullet