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  • Title: Role of omalizumab in a patient with hyper-IgE syndrome and review dermatologic manifestations.
    Author: Chularojanamontri L, Wimoolchart S, Tuchinda P, Kulthanan K, Kiewjoy N.
    Journal: Asian Pac J Allergy Immunol; 2009 Dec; 27(4):233-6. PubMed ID: 20232578.
    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 pneumatocele 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 abscesses 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 monoclonal antibody against IgE, subcutaneous omalizumab, was successfully used in this patient.
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