Autosomal recessive hyperimmunoglobulin E syndrome (Q94304): Difference between revisions
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Revision as of 09:26, 16 August 2026
Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterised by the clinical triad of highly elevated serum IgE levels, recurring staphylococcal skin abscesses, and recurrent pneumonia. The clinical triad is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; ), but other features such as persistent cutaneous viral infections are unique to AR-HIES.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1909398175 |
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| English | Autosomal recessive hyperimmunoglobulin E syndrome |
Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterised by the clinical triad of highly elevated serum IgE levels, recurring staphylococcal skin abscesses, and recurrent pneumonia. The clinical triad is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; ), but other features such as persistent cutaneous viral infections are unique to AR-HIES. |
