Clinical Syndrome of Chemotaxis Defect, Infections, and Hyperimmunoglobulinemia E

Abstract

A 17-year-old girl had a clinical syndrome that consisted of neutrophil chemotaxis defect, recurrent bacterial infections, and hyperimmunoglobulinemia E. Neutrophil chemotaxis was quantitated by a radioassay technique that used 51Cr-labeled neutrophils and a double micropore filter chemotaxis chamber. A table of the features of other cases of this syndrome is presented. Since ten of the 13 patients who are described had a chronic eczematoid dermatitis, we suggest that this may be a fourth feature of this syndrome. Red hair and fair skin were not consistent findings in these patients. In the evaluation of patients with chronic, recurrent bacterial infections of the skin of undetermined origin, we recommend determination of serum IgE levels. If IgE is found to be elevated, one should consider the presence of this syndrome, and neutrophil chemotaxis should then be examined.