Autosomal recessive hyperimmunoglobulin E syndrome (Q94304): Difference between revisions

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Latest 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
default for all languages	ID_1909398175
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.