Skip navigation
MedlinePlus Trusted Health Information for You U.S. National Library of MedicineNational Institutes of Health
Contact Us FAQs Site Map About MedlinePlus
español Home Health Topics Drug Information Medical Encyclopedia Dictionary News Directories Other Resources

 

Medical Encyclopedia

Other encyclopedia topics:  A-Ag  Ah-Ap  Aq-Az  B-Bk  Bl-Bz  C-Cg  Ch-Co  Cp-Cz  D-Di  Dj-Dz  E-Ep  Eq-Ez  F  G  H-Hf  Hg-Hz  I-In  Io-Iz  J  K  L-Ln  Lo-Lz  M-Mf  Mg-Mz  N  O  P-Pl  Pm-Pz  Q  R  S-Sh  Si-Sp  Sq-Sz  T-Tn  To-Tz  U  V  W  X  Y  Z  0-9 

Hyperimmunoglobulin E syndrome

Printer-friendly versionEmail this page to a friend
Contents of this page:

Alternative names   

Job syndrome; Hyper IgE syndrome

Definition    Return to top

Hyperimmunoglobulin E syndrome is a suspected genetic defect that results in the production of high levels of the antibody immunoglobulin (IgE), causing serious skin and lung infections as well as eczema.

Causes, incidence, and risk factors    Return to top

Hyperimmunoglobulin E syndrome is also know as Job syndrome, after the biblical character Job whose faithfulness was tested by a lifelong affliction of draining skin sores and pustules. People afflicted have chronic severe skin infections and abscess formation. The sinuses and airways (including lungs) are also frequently infected. Other sites of infection include bone and visceral abscesses. The most commonly involved bacteria is Staphylococcus aureus. Other common organisms are Streptococcus pneumoniae (bacterium), Hemophilus influenzae (bacterium), Candida albicans (yeast), and the herpes virus (virus).

Hyper IgE syndrome is in most cases inherited in a dominant fashion, meaning that it can run in families with high frequency.

Symptoms    Return to top

Signs and tests    Return to top

Tests to confirm a diagnosis: Other tests may be done in response to infections:

Treatment    Return to top

There is no known cure for the condition. The goal of treatment is control of the recurrent infections. Medications include antibiotic treatment of infections with whatever antibiotic is specific for the organism causing the infection. Antifungal agents are prescribed when appropriate. Antiviral agents are given when appropriate.

Sometimes surgical drainage of abscesses is needed.

IV gamma globulin may help to build up the immune system temporarily when there are severe infections.

Expectations (prognosis)    Return to top

Job syndrome is a lifelong chronic condition. Each new infection requires treatment.

Complications    Return to top

Calling your health care provider    Return to top

Call your health care provider if you are experiencing or observing a collection of the above signs and symptoms.

Prevention    Return to top

There is no proven infection. Good general hygiene is helpful. Some doctors may recommend prophylactic antibiotics in individuals with many infections, particularly with Staphylococcus aureus. This does not change the underlying disorder but rather its consequences.

Update Date: 10/30/2003

Updated by: Donald Accetta, MD, MPH. President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network.

adam.com logo

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.