[Hyper-IgE syndrome]
[Article in
German]
- PMID: 3614229
Item in Clipboard
[Hyper-IgE syndrome]
[Article in
German]
Monatsschr Kinderheilkd.
1987 Jun.
Abstract
The Hyper-IgE-syndrome (Job-, Buckley-) is characterized by recurrent staphylococcal infections of the skin, the ears and the lungs, by an eczematoid dermatitis from early infancy on, and by extreme elevation of serum IgE. The inconstantly found decreased chemotaxis of the polymorphonuclear leukocytes seems to be a secondary sign of a so far unknown deficiency possibly of the T-cell mediated immunity. Thus, therapy is restricted to antibiotic and surgical treatment. Many patients have a typical coarse face and some involvement of the bones (osteoporosis, craniosynostosis).
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