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Hyperimmunoglobulinemia E syndrome - Skin
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Hyperimmunoglobulinemia E syndrome - Skin

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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Nancy Esterly MD
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Synopsis

Hyper-IgE syndrome (Job's syndrome) is a multisystem immunodeficiency disorder characterized by recurrent skin and pulmonary infections/abscesses (primarily bacterial) and variably high levels of 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.

For information on the autosomal dominant variety, see OMIM.

For information on the autosomal recessive variety, see OMIM.

Codes

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

SNOMEDCT:
50926003 – Hyperimmunoglobulin E syndrome

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Last Updated: 07/19/2017
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Hyperimmunoglobulinemia E syndrome - Skin
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Hyperimmunoglobulinemia E syndrome : Axilla, Crust, Diaper area, Face, IgE elevated, Scalp, Pustules
Clinical image of Hyperimmunoglobulinemia E syndrome
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