ABSTRACT
Hyper-IgE syndrome (HIES) is a rare idiopathic primary immunodeficiency. It is characterized by a triad of findings, including high levels of serum IgE, recurrent skin abscesses and pneumonia and leads to pneuma-tocele formation. The diagnosis of HIES is complicated by a diversity of clinical and immunological spectrums and a heterogeneous set of genetic defects. The National Institute of Health (NIH) developed a scoring system for HIES in which a score greater than 14 indicates a probable diagnosis. Our patient presented with recurrent multiple ab-scesses on her scalp, recalcitrant eczema, candida onychomycosis, alopecia universalis, and highly elevated levels of serum IgE. Using the NIH scoring system, a 30 total-point score in this patient indicated the likelihood of carrying the HIES genotype. To our knowledge, there are no specific treatments of HIES. The humanized recombinant mo-noclonal antibody against IgE, subcutaneous omalizumab, was successfully used in this patient.