Hyperimmunoglobulinemia E Syndrome, Pediatric

Rachel G. Robison, MD Reviewed 10/2018

BASICS

DESCRIPTION

Hyperimmunoglobulinemia E syndrome (HIES) is a primary immunodeficiency with markedly elevated serum IgE associated with recurrent skin abscesses, pulmonary infections, and eczematoi...

DIAGNOSIS

HISTORY

Recurrent infections
- Skin
  - Abscesses
  - Furuncles
  - Cellulitis
  - Lymphadenitis
- Sinopulmonary infections
  - Pneumonia with aberrant healing, forming pneumatoceles and bronchiectasis
- Fungal infections: mo...

TREATMENT

GENERAL MEASURES

Implement good management of dermatitis with regular hydration and emollient use.
Control pruritus with antihistamines.
Dilute bleach baths to decrease staphylococcal skin colo...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Blood pressure monitoring in patients, especially those with known vascular anomalies, as hypertension is common

PROGNOSIS

Pulmonary complication...

ADDITIONAL READING

Buckley RH. The hyper-IgE syndrome. Clin Rev Allergy Immunol. 2001;20(1):139–154. [View Abstract on OvidInsights]
Davis SD, Schaller J, Wedgwood RJ. Job’s syndrome. Recurrent, ...

CODES

ICD9

288.1 Functional disorders of polymorphonuclear neutrophils

ICD10

D82.4 Hyperimmunoglobulin E [IgE] syndrome

SNOMED

50926003 Job’s syndrome (disorder)

FAQ

Q: How do AD-HIES and AR-HIES differ?
A: Aside from arising from different mutations, AD-HIES has more skeletal and facial anomalies, whereas AR-HIES is more often associated with viral skin infect...

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