Understanding and Managing Hyper IgE Syndromes.

Abstract

Hyper-IgE syndromes represent an increasingly recognized and heterogeneous group of disorders characterized phenotypically by eczema, recurrent infections, and markedly elevated serum IgE levels. The identification of novel molecular defects in recent years has complicated definitive diagnosis, underscoring the genetic and clinical diversity of this group. In addition to immunological abnormalities, non-immunological manifestations-particularly those affecting connective tissue-contribute to significant comorbidities. The primary objectives of management are to control infections, prevent long-term complications, and improve quality of life. In this review, we summarize the clinical and laboratory features of disorders currently classified under hyper-IgE syndromes according to the most recent International Union of Immunological Societies framework and provide perspectives on their management.