Hyperimmunoglobulin-E syndrome

Two Saudi girls aged 8 years and 5 years were seen over a period of 6 years and 5 years. Their clinical presentations consisted of recurrent bilateral otitis media, repeated episodes of tonsillitis and chest infection. Cultures from the ears grew on numerous occasions Staphylococcus aureus, Streptococcus pneumoniae, Pseudomonas aeroginosa, Proteus species and Providencia species. The 8-year-old had a serum IgE level of 1431 iu/L, with normal levels of other immunoglobulin classes. The 5-year-old had an immunoglobulin E value of 1119 iu/L with normal values of other immunoglobulin classes. Both were human immuno-deficiency virus negative and no other causes for elevated immunoglobulin E were found. The mothers of both cases had elevated immunoglobulin E levels of 1216 iu/L and 1992 iu/L. Both fathers had normal IgE levels. A 13-year-old sibling of case one had a grossly elevated immunoglobulin E level of 2259 iu/L. She had diffuse lamellar icthyosis and recurrent episodes of chest infection and conjunctivitis. There was a good clinical response of patient one to monthly intravenous human immunoglobulin.