Hyperimmunoglobulin E [IgE] syndrome

This code represents a rare primary immunodeficiency characterized by recurrent infections, particularly staphylococcal skin abscesses, pneumonias, and mucocutaneous candidiasis. Patients typically exhibit extremely high serum IgE levels, often accompanied by eczema, facial dysmorphism, and skeletal abnormalities.

Use this code for patients diagnosed with Hyperimmunoglobulin E syndrome, also known as Job's syndrome. Documentation should clearly indicate the diagnosis, often supported by elevated IgE levels, characteristic clinical features, and sometimes genetic testing results.

• Distinguish from other primary immunodeficiencies; this code is specific to the IgE-mediated syndrome.
• Code any associated infections or manifestations separately as secondary diagnoses.
• Ensure the medical record explicitly states "Hyperimmunoglobulin E syndrome" or "Job's syndrome" for accurate coding.