Catalog

Autosomal dominant hyperimmunoglobulin E syndrome

No MMS code

Link to WHO Definition

Autosomal dominant hyperimmunoglobulin E syndrome – http://id.who.int/icd/entity/1561846514

Autosomal dominant hyper-IgE syndrome (AD-HIES) is a very rare primary immunodeficiency disorder characterised by the clinical triad of high serum IgE (>2000 IU/ml), recurring staphylococcal skin abscesses, and recurrent pneumonia with formation of pneumatoceles.