Episodic angioedema associated with eosinophilia

Four patients with recurrent attacks of angioedema, urticaria, and fever were seen. During attacks, body weights increased up to 18% and leukocyte counts reached 108,000/microliters (88% eosinophils). Glucocorticoid therapy caused defervescence, diuresis, and decreased total leukocyte and eosinophil counts. The two children received prednisone intermittently; the adults did not require treatment or their conditions were controlled by alternate-day prednisone administration. No patient had evidence of cardiac involvement (follow-up, 2-17 years). The disease does not threaten the function of vital organs. One patient remained in spontaneous remission for 20 years before symptoms recurred. Although it might be classified as a variant of the hypereosinophilic syndrome, we believe that this syndrome is a separate entity because of its distinctive characteristics and its benign course.