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Immunol Allergy Clin North Am. 2008 May;28(2):277-91, viii. doi: 10.1016/j.iac.2008.01.005.

The hyper-IgE syndromes.

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1
Laboratory of Clinical Infectious Disease, National Institute of Allergy and Infectious Disease, Building 10, CRC B3-4141, MSC 1684, 9000 Rockville Pike, Bethesda, MD 20892-1684, USA.

Abstract

The hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, rash, and recurrent bacterial infections of the skin and lung. Autosomal dominant HIES, the most common disease in this group, results from STAT3 mutations and has a variety of connective tissue and skeletal abnormalities. The genetic etiologies of the more rare autosomal recessive forms still need delineation. Treatment of these syndromes has relied on prophylactic and therapeutic antimicrobial agents and aggressive skin care. The new and evolving genetic and immunologic understandings of this previously elusive set of diseases should lead to more effective disease-specific therapies.

PMID:
18424333
PMCID:
PMC2683262
DOI:
10.1016/j.iac.2008.01.005
[Indexed for MEDLINE]
Free PMC Article
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