Hyperimmunoglobulin E [IgE] syndrome
ICD-10 D82.4 is a billable code used to indicate a diagnosis of hyperimmunoglobulin e [ige] syndrome.
Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disorder characterized by elevated levels of immunoglobulin E (IgE) in the blood, recurrent infections, and various allergic manifestations. Patients with HIES often experience recurrent skin infections, pneumonia, and other respiratory infections due to their impaired immune response. The condition can be associated with eczema, food allergies, and elevated eosinophil counts. HIES can be caused by genetic mutations affecting the immune system, particularly in the STAT3 gene, which plays a crucial role in immune regulation. Diagnosis is typically made through clinical evaluation, family history, and laboratory tests showing elevated IgE levels. Management focuses on treating infections, managing allergic symptoms, and monitoring for complications such as lung disease or autoimmune disorders. Patients may require immunoglobulin replacement therapy and antibiotics for recurrent infections. The prognosis varies, with some individuals leading relatively normal lives with appropriate management, while others may experience significant morbidity.
Detailed immunological assessments, including IgE levels and infection history.
Patients presenting with recurrent infections, eczema, and elevated IgE levels.
Ensure comprehensive documentation of all allergic manifestations and infections.
Growth and development assessments, family history of allergies or immunodeficiencies.
Children with recurrent respiratory infections and skin conditions.
Monitor for developmental delays or complications related to infections.
Used for obtaining blood samples to measure IgE levels.
Document the reason for blood draw and any relevant clinical history.
Ensure proper labeling and handling of samples for accurate testing.
Common symptoms include recurrent skin infections, pneumonia, eczema, and elevated IgE levels. Patients may also experience food allergies and other allergic conditions.