Life-threatening idiopathic anaphylaxis associated with hyperimmunoglobulinemia E

After five years of mild, intermittent urticaria, a man had life-threatening idiopathic anaphylactic reactions associated with transient myocardial ischemia, small bowel obstruction, and orthostatic hypotension. There was no evidence of causes for the anaphylactic reactions over a two and a half year period. The total serum IgE level was increased substantially, with sharp elevations occurring during three episodes of anaphylactic reactions. The elevation in total IgE levels, which was not associated with peripheral blood eosinophilia, is the first recognized serologic abnormality in this syndrome.