Short answer · Medically reviewed summary · Last updated: 2026-04-07
The ICD-10 code for Hyper IgE Syndrome, specifically the autosomal dominant form known as Job syndrome, is D81.82, while the ICD-9 code historically associated with this condition is 279.06. As a clinician who has walked alongside many patients navigating the complexities of Hyper IgE Syndrome, I understand that these numerical codes are more than just administrative identifiers; they are often the keys to unlocking access to specialized care, insurance coverage, and the diagnostic clarity you deserve. Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency characterized by the triad of elevated serum IgE levels, recurrent staphylococcal skin abscesses, and recurrent pneumonia. Clinical Classification and Coding While D81.82 is the specific code for the STAT3-deficient form of Hyper IgE Syndrome, medical coding can sometimes be fluid depending on the specific manifestations present in a patient’s chart.
ICD10 code of Hyper IgE Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Hyper IgE Syndrome, with classification details for clinicians, coders and patients.
The ICD-10 code for Hyper IgE Syndrome, specifically the autosomal dominant form known as Job syndrome, is D81.82, while the ICD-9 code historically associated with this condition is 279.06.
As a clinician who has walked alongside many patients navigating the complexities of Hyper IgE Syndrome, I understand that these numerical codes are more than just administrative identifiers; they are often the keys to unlocking access to specialized care, insurance coverage, and the diagnostic clarity you deserve. Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency characterized by the triad of elevated serum IgE levels, recurrent staphylococcal skin abscesses, and recurrent pneumonia.
Clinical Classification and Coding
While D81.82 is the specific code for the STAT3-deficient form of Hyper IgE Syndrome, medical coding can sometimes be fluid depending on the specific manifestations present in a patient’s chart. Because this is a multisystem disorder, some clinicians may also utilize codes reflecting the specific skeletal, dental, or pulmonary complications that define the clinical presentation of Hyper IgE Syndrome. It is essential to ensure that your medical records reflect the genetic or clinical diagnosis accurately to facilitate coordinated care between immunologists, dermatologists, and pulmonologists.
Navigating the Rare Disease Journey
Receiving a formal diagnosis of Hyper IgE Syndrome can be an emotionally overwhelming experience. Please know that you are not alone in this; our community at DiseaseMaps.org is a testament to the resilience of those living with this condition. Understanding your diagnosis codes is a practical step toward advocacy, but it is equally important to prioritize your mental well-being as you manage the chronic nature of this syndrome. Whether you are dealing with skin manifestations or the stress of frequent medical monitoring, connecting with others who share your experience can provide a unique sense of validation and support that clinical data alone cannot offer.
Disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Hyper IgE Syndrome
- Orphanet: Autosomal dominant hyper-IgE syndrome
- OMIM (Online Mendelian Inheritance in Man): Hyper-IgE recurrent infection syndrome (HIES)
