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Hyperimmunoglobulinemia E syndrome in Infant/Neonate
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Hyperimmunoglobulinemia E syndrome in Infant/Neonate

Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Hyperimmunoglobulinemia E syndrome (also known as hyper-IgE syndrome and Job syndrome) is a multisystem immunodeficiency disorder characterized by recurrent skin and pulmonary infections / abscesses (primarily bacterial) and variably high levels of immunoglobulin E (IgE). The most frequent responsible pathogen is Staphylococcus aureus, followed by Haemophilus influenzae, Streptococcus pyogenes, Escherichia coli, Pseudomonas, and Candida albicans. It is inherited in an autosomal dominant fashion. Associated features may include coarse facies, delay of shedding of primary teeth, recurrent fractures, and scoliosis. Neutrophils have impaired chemotaxis and inflammation is minimal, producing "cold" abscesses.

There is an autosomal recessive form that manifests without connective tissue and skeletal involvement.

Codes

ICD10CM:
D82.4 – Hyperimmunoglobulin E [IgE] syndrome

SNOMEDCT:
50926003 – Hyperimmunoglobulin E syndrome

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Last Updated:04/11/2022
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Hyperimmunoglobulinemia E syndrome in Infant/Neonate
Hyperimmunoglobulinemia E syndrome : Axilla, Crust, Diaper area, Face, IgE elevated, Scalp
Clinical image of Hyperimmunoglobulinemia E syndrome
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