Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterized 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; see this term), but other features such as persistent cutaneous viral infections are unique to AR-HIES. The disease is Orphanet_169446 (Autosomal recessive hyper-IgE syndrome). Also known as: AR-HIES, Autosomal recessive HIES, Hyperimmunoglobulin E syndrome type 2, Non-skeletal hyper-IgE syndrome.