Hyperimmunoglobulinemia E (Job) Syndrome Questions & Answers

Updated: Jul 10, 2019
  • Author: Harumi Jyonouchi, MD; Chief Editor: Russell W Steele, MD  more...
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Questions & Answers

Overview

What is hyperimmunoglobulin E syndrome (HIES)?

What is the pathophysiology of hyperimmunoglobulin E syndrome (HIES)?

What is the prevalence of hyperimmunoglobulin E syndrome (HIES)?

What is the mortality and morbidity associated with hyperimmunoglobulin E syndrome (HIES)?

What are the racial predilections of hyperimmunoglobulin E syndrome (HIES)?

What are the sexual predilections of hyperimmunoglobulin E syndrome (HIES)?

At what age is hyperimmunoglobulin E syndrome (HIES) typically diagnosed?

Presentation

Which clinical history findings are characteristic of hyperimmunoglobulin E syndrome (HIES)?

Which physical findings are characteristic of hyperimmunoglobulin E syndrome (HIES)?

What causes hyperimmunoglobulin E syndrome (HIES)?

DDX

How is atopic dermatitis differentiated from hyperimmunoglobulin E syndrome (HIES)?

Which primary immunodeficiencies with eczematous dermatitis are included in the differential diagnoses of hyperimmunoglobulin E syndrome (HIES)?

How is Omenn syndrome (OS) differentiated from hyperimmunoglobulin E syndrome (HIES)?

How is common variable immunodeficiency disease (CVID) differentiated from hyperimmunoglobulin E syndrome (HIES)?

How is immune dysregulation, polyendocrinopathy, enteropathy, X-linked inheritance (IPEX) syndrome differentiated from hyperimmunoglobulin E syndrome (HIES)?

How is HIV infection differentiated from hyperimmunoglobulin E syndrome (HIES)?

What are the differential diagnoses for Hyperimmunoglobulinemia E (Job) Syndrome?

Workup

What is the role of lab testing in the workup of hyperimmunoglobulin E syndrome (HIES)?

What is the role of imaging studies in the workup of hyperimmunoglobulin E syndrome (HIES)?

What is the role of cultures in the workup of hyperimmunoglobulin E syndrome (HIES)?

What is the role of bronchoscopy in the workup of hyperimmunoglobulin E syndrome (HIES)?

Which histologic findings are characteristics of hyperimmunoglobulin E syndrome (HIES)?

Treatment

How is hyperimmunoglobulin E syndrome (HIES) treated?

What is the role of surgery in the treatment of hyperimmunoglobulin E syndrome (HIES)?

Which specialist consultations are beneficial to patients with hyperimmunoglobulin E syndrome (HIES)?

Which dietary modifications are used in the treatment of hyperimmunoglobulin E syndrome (HIES)?

Which activity modifications are used in the treatment of hyperimmunoglobulin E syndrome (HIES)?

Medications

What is the role of medications in the treatment of hyperimmunoglobulin E syndrome (HIES)?

Which medications in the drug class Antifungal agents are used in the treatment of Hyperimmunoglobulinemia E (Job) Syndrome?

Which medications in the drug class Second-line antistaphylococcal antibiotics are used in the treatment of Hyperimmunoglobulinemia E (Job) Syndrome?

Which medications in the drug class Topical calcineurin inhibitors are used in the treatment of Hyperimmunoglobulinemia E (Job) Syndrome?

Which medications in the drug class Topical corticosteroids are used in the treatment of Hyperimmunoglobulinemia E (Job) Syndrome?

Which medications in the drug class Antibiotics are used in the treatment of Hyperimmunoglobulinemia E (Job) Syndrome?

Follow-up

What is included in the long-term monitoring of hyperimmunoglobulin E syndrome (HIES)?

When is inpatient care indicated for the treatment of hyperimmunoglobulin E syndrome (HIES)?

When is patient transfer needed for the treatment of hyperimmunoglobulin E syndrome (HIES)?

When is prenatal diagnosis of hyperimmunoglobulin E syndrome (HIES) possible?

How are infections prevented in patients with hyperimmunoglobulin E syndrome (HIES)?

What are the possible complications of hyperimmunoglobulin E syndrome (HIES)?

What is the prognosis of hyperimmunoglobulin E syndrome (HIES)?

What is included in patient education about hyperimmunoglobulin E syndrome (HIES)?