D82.4

HCC Category Mapping

What This Code Means

A rare immune disorder characterized by abnormally high levels of IgE antibodies, leading to recurrent skin and lung infections.

Coding Tips

• •Confirm elevated IgE levels are documented in laboratory results
• •Document associated infections (skin, respiratory) separately if they are the focus of treatment

Clinical Significance

Hyperimmunoglobulin E syndrome, also known as Job syndrome in its autosomal dominant form, is a rare primary immunodeficiency characterized by markedly elevated serum Immunoglobulin E levels, recurrent staphylococcal skin abscesses (classically described as 'cold' abscesses lacking typical inflammatory signs), eczema, and recurrent pneumonias with pneumatocele formation. Skeletal and dental abnormalities are also common in the autosomal dominant form.

Documentation Requirements

• ✓Documentation must include serum Immunoglobulin E levels (typically exceeding 2,000 IU/mL), specific infection history with organisms identified, skin examination findings including abscess characteristics, pulmonary imaging showing pneumatoceles if present, skeletal features (scoliosis, pathologic fractures), dental abnormalities (retained primary teeth), and genetic testing if performed (STAT3 or DOCK8 mutations).

Commonly Confused Codes