Genetic linkage of hyper-IgE syndrome to chromosome 4

Hyper-IgE syndrome (HIES), or Job syndrome, a rare multisystem disorder, a primary immunodeficiency involving pneumonia, recurrent skin abscesses, and very elevated serum IgE levels, is discussed with the information that it is linked to chromosome 4. Nonimmunologic abnormalities of bones, dentition and connective tissue are part of the disorder and it can be transmitted as an autosomal dominant trait with variable espressivity. Nineteen kindreds with multiple cases were scored for laboratory/clinical findings and genotyped with polymorphic markers in a candidate region.