immunodeficiency disease Autosomal recessive hyper-IgE syndrome UMLS:C1968689 ICD10:D82.4 Autosomal recessive HIES orphanet Hyperimmunoglobulin E syndrome type 2 AR-HIES OMIM:618523 Non-skeletal hyper-IgE syndrome OMIM:243700 OMIM:611521 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.