Hyper IgE syndrome

What is hyper IgE syndrome?

Hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, skin inflammation (dermatitis) and recurrent skin and lung infections. There are two forms of HIES, which have the above characteristics in common but otherwise have distinct presentations, courses and outcomes: autosomal dominant HIES (AD-HIES) and autosomal recessive HIES (AR-HIES). Click on the embedded links to learn more about autosomal dominant HIES (or Job syndrome) and autosomal recessive HIES.

Last updated on 05-01-20

Is diet modifications helpful in treating eczema in children with Job syndrome?

A skin rash that is very similar in appearance to eczema is characteristic for Job syndrome. Unlike eczema however, people with Job syndrome usually lack other common symptoms, such as wheezing, allergic features, or a family history of eczema. The rash in Job syndrome occurs as a result of immune system abnormalities. These abnormalities also make people with Job syndrome prone to Staphylococcal and Candida skin infections. These skin infections can cause crusting dermatitis, folliculitis, cellulitis, abscesses, and lymphadenitis.

In general, approach to treatment of skin rashes and infections in Job syndrome differs from that of eczema. Treatment involves frequent soaks in a bath followed by the use of moisturizing cream. Steroid creams may be used to treat inflamed, but uninfected skin. Antihistamines may be recommended to reduce itchiness. Regular evaluation for signs of skin infection is also recommended. Prophylactic treatment with antibiotics, such as trimethoprim-sulfamethoxazole, may be advised to prevent skin infections.

Dietary restriction for treatment of skin rashes due to Job syndrome is not routine. It is however possible for a child with Job syndrome to also have food or other allergies. We strongly recommend concerns regarding food or other allergies be discussed with a healthcare provider. Likewise, treatment needs to be tailored to each patient. We strongly recommend that you work with a healthcare provider when making treatment decisions.

Last updated on 05-01-20

What is the long-term outlook for people with hyper IgE syndrome (HIES)?

The long-term outlook for individuals with HIES depends on the type of the condition present (the autosomal dominant or autosomal recessive form) and how severely affected the individual is.

Most individuals with the autosomal dominant form (AD- HIES) survive into mid-adulthood, but a shortened life span is common. The oldest reported affected individual was approximately 60 years of age. Deaths in the second and third decades of life due to severe pulmonary disease and infection of pneumatoceles (thin-walled, air-filled cysts) have been reported. Other reported complications have included myocardial infarction (heart attack) related to coronary artery aneurysm and subarachnoid hemorrhage related to intracranial (brain) aneurysm. Lymphomas occur more commonly in affected individuals. Other malignancies (cancers) have also been reported.

Prognosis for individuals with the autosomal recessive form (AR- HIES) is poor with most affected individuals not reaching adulthood if untreated. AR-HIES has high mortality due to sepsis, central nervous system (CNS) infections, and early onset of malignancies. Individuals with mutations in the DOCK8 gene are known to have frequent complications with cutaneous viral infections caused by varicella-zoster, herpes simplex viruses, HPV, and molluscum contagiosum virus at a younger age. Individuals with AR-HIES are also known to develop severe chronic refractory molluscum contagiosum infections (resistant to treatment).

Last updated on 05-01-20

Name: Immune Deficiency Foundation 110 West Road, Suite 300
Towson, MD, 21204, United States
Toll Free: 1-800-296-4433 Fax : +1-410-321-9165 Email: https://www.primaryimmune.org/services/ask-idf/ Url: https://www.primaryimmune.org/
Name: United States Immunodeficiency Network (USIDNET) 110 West Road, Suite 300
Towson, MD, 21204-4803, United States
Phone: 443-632-2558 Toll Free: 800-296-4433 (se habla español) Email: contact@usidnet.org Url: https://www.usidnet.org
Name: Canadian Immunodeficiencies Patient Organization (CIPO) 25 La Grave St Winnepeg, MB
R3V 1J1, Canada
Phone: 877-262-2476 (toll-free) Fax : 866-942-7651 (toll-free) Email: http://www.cipo.ca/#contact Url: http://cipo.ca
Name: Immune Deficiencies Foundation Australia PO Box 969 Penrith NSW 2751
Phone: 800-100-198 Email: info@idfa.org.au Url: http://www.idfa.org.au/
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: Primary Immune Deficiency UK PID UK PO Box 6970
Basingstoke, RG24 4XL, United Kingdom
Toll Free: 0800 987 8986 Email: hello@piduk.org Url: http://www.piduk.org/

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