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Autosomal dominant hyper IgE syndrome


Other Names for this Disease

  • AD hyperimmunoglobulin E syndrome
  • AD-HIES
  • HIES autosomal dominant
  • Hyper Ig E syndrome, autosomal dominant
  • Hyperimmunoglobulin E recurrent infection syndrome, autosomal dominant
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Autosomal dominant hyper IgE syndrome (AD-HIES), formerly known as Job syndrome, affects several body systems including the immune system. AD-HIES is characterized by abnormally high levels of an immune system protein called immunoglobulin E (IgE) in the blood.  Signs and symptoms may include recurrent infections (e.g., pneumonia, skin infections), eczema, and occasionally bone and tooth abnormalities. The eczema and skin infections may cause rashes, blisters, collections of pus (abscesses), open sores, and scaling of the skin. Some cases of AD-HIES are caused by mutations in the STAT3 gene. In other cases, the cause is unknown.[1]
Last updated: 8/25/2010

References

  1. Job syndrome. Genetics Home Reference (GHR). February 2008; http://ghr.nlm.nih.gov/condition=jobsyndrome. Accessed 11/13/2008.
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Please contact us with your questions about Autosomal dominant hyper IgE syndrome. We will answer your question and update these pages with new resources and information.

Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Autosomal dominant hyper IgE syndrome. This website is maintained by the National Library of Medicine.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Allergy and Infectious Diseases (NIAID) supports scientists developing better ways to diagnose, treat, and prevent the many infectious, immunologic, and allergic diseases that afflict people worldwide. Click on the link to view information on this topic. 
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Autosomal dominant hyper IgE syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • AD hyperimmunoglobulin E syndrome
  • AD-HIES
  • HIES autosomal dominant
  • Hyper Ig E syndrome, autosomal dominant
  • Hyperimmunoglobulin E recurrent infection syndrome, autosomal dominant
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.