4A01.34/D82.4 Hyperimmunoglobulin E syndromes



Hyperimmunoglobulin E (HIE) syndromes are a group of rare, inherited immunodeficiency disorders characterized by high levels of immunoglobulin E (IgE) antibodies. These conditions are caused by mutations in genes that are responsible for regulating the production of IgE.


Diagnosis of HIE syndromes is based on the presence of high levels of IgE antibodies in the blood, as well as the presence of other signs and symptoms associated with the condition. Additional testing, such as genetic testing, may be used to confirm the diagnosis.

Differential diagnosis

Differential diagnosis of HIE syndromes includes other immunodeficiency disorders, such as common variable immunodeficiency (CVID), Wiskott-Aldrich syndrome, and ataxia-telangiectasia.


Treatment of HIE syndromes typically involves the use of immunosuppressive medications, such as corticosteroids and cyclosporine A, to reduce the production of IgE antibodies. In some cases, bone marrow transplantation may be used to treat the condition.


The prognosis of HIE syndromes is generally good, with most patients responding to treatment. However, long-term complications, such as recurrent infections and organ damage, may occur in some patients.

How medically accurate was this information?

Click on a star to rate it

Average rating 0 / 5. Vote count: 0

No votes so far! Be the first to rate this post.

DISCLAIMER: Please note that all explAInations are generated by AI and are not fact checked by a medical professional. ICD ExplAIned do not assume liability for any injuries or harm based on the use of this medical information.