Hyper-IgE Syndrome in an Infant

Giberson, Mariah
Finlayson, Laura

¹Dalhousie Medicine New Brunswick, Saint John, NB, Canada
²Dalhousie Division of Clinical Dermatology and Cutaneous Science, Halifax, NS, Canada

Corresponding Author:

Mariah Giberson, Dalhousie Medicine New Brunswick, 100 Tucker Park Road, PO Box 5050, Saint John, NB E2L 4L5, Canada.

Journal of Cutaneous Medicine and Surgery 20(4):p 340-342, July-August 2016. | DOI: 10.1177/1203475416629593

Abstract

Background:

Hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder that affects multiple systems. One of the early findings is a papulopustular rash, which has a wide differential diagnosis.

Method:

The authors report the case of a male newborn diagnosed with HIES. He presented with papulopustular dermatitis on the scalp. The authors also present a review of current theory on the pathophysiology, clinical presentation, and management of HIES.

Conclusion:

Although HIES is a multisystem disorder, many of the manifestations of HIES may present after the neonatal period. The cutaneous manifestations of HIES are usually present shortly after birth, and the presentation of pustules in a newborn may be one of the reasons a dermatologist would be asked to assess a patient in the neonatal period.