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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hyper IgE syndrome


Other Names for this Disease
  • HIES
  • Hyper-IgE recurrent infection syndrome
  • Hyperimmunoglobulin E syndrome
  • Job syndrome
  • Job's syndrome
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Your Question

Does the eczema associated with Job syndrome respond to dietary restriction?  In particular, is the eczema due to food allergies or will these kids have eczema unrelated to food?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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.[1] Unlike eczema however, people with Job syndrome usually lack other common symptoms, such as wheezing, allergic features, or a family history of eczema.[2] 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, cellulitisabscesses, and lymphadenitis.[1][2] 

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.[3][2] Steroid creams may be used to treat inflamed, but uninfected skin. Antihistamines may be recommended to reduce itchiness.[2] Regular evaluation for signs of skin infection is also recommended.[2] Prophylactic treatment with antibiotics, such as trimethoprim-sulfamethoxazole, may be advised to prevent skin infections.[2]

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: 3/20/2013

References