Preferred Label : Autosomal dominant hyperimmunoglobulin E syndrome;
ICD-11 definition : Autosomal dominant hyper-IgE syndrome (AD-HIES) is a very rare primary immunodeficiency
disorder characterized by the clinical triad of high serum IgE ( 2000 IU/ml), recurring
staphylococcal skin abscesses, and recurrent pneumonia with formation of pneumatoceles.;
ICD-11 synonym : Autosomal dominant hyper-IgE syndrome; Hyperimmunoglobulin E syndrome type 1; AD-HIES - [Autosomal dominant hyperimmunoglobulin E] syndrome; Hyperimmunoglobulin E-recurrent infection syndrome; AD-HIES syndrome;
Origin ID : 1561846514;
Automatic exact mappings (from CISMeF team)
Autosomal dominant hyper-IgE syndrome (AD-HIES) is a very rare primary immunodeficiency
disorder characterized by the clinical triad of high serum IgE ( 2000 IU/ml), recurring
staphylococcal skin abscesses, and recurrent pneumonia with formation of pneumatoceles.
