CASE REPORTS
JOURNAL ARTICLE
Add like
Add dislike
Add to saved papers

Association between nasal polyposis, Dubowitz syndrome and hyper-IgE syndrome.

Dubowitz syndrome is a rare, autosomal recessive disorder characterized by intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, and psychomotoric retardation. The hyper-immunoglobulin E (hyper-IgE or Job syndrome) is a primary immunodeficiency characterized by recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia, and an elevated serum IgE level of >2000 IU/ml. We present the first case to our knowledge of an association between Dubowitz syndrome, hyper-IgE syndrome, and nasal polyposis (due to allergic fungal sinusitis) in a 14-year-old girl. Eosinophilic inflammatory reaction is the feature present in all three conditions. Unlike most cases of allergic fungal sinusitis, this case was not treated with an initial booster of oral steroids due to the risk of disseminated invasive fungal infection, reported in other cases of hyper-IgE syndrome. The case and its management is presented and discussed.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app