Gleich G J, Schroeter A L, Marcoux J P, Sachs M I, O'Connell E J, Kohler P F
N Engl J Med. 1984 Jun 21;310(25):1621-6. doi: 10.1056/NEJM198406213102501.
We studied four patients with recurrent attacks of angioedema, urticaria, and fever. During attacks, body weights increased up to 18 per cent, and leukocyte counts reached 108,000 per microliter (88 per cent eosinophils). The disease did not appear to threaten the function of vital organs. The two children received prednisone intermittently; the adults did not require treatment or were given alternate-day prednisone. Glucocorticoid therapy caused defervescence and diuresis and decreased total leukocyte and eosinophil counts. No patient had evidence of cardiac involvement (follow-up, 2 to 17 years). One patient remained in spontaneous remission for 20 years before symptoms recurred. Histologic studies showed that eosinophils localized and degranulated in the dermis, and they appeared to induce edema. Although this syndrome might be classified as a variant of the hypereosinophilic syndrome, we believe it is a separate entity because of its distinctive characteristics and its benign course.
我们研究了4例反复出现血管性水肿、荨麻疹和发热症状的患者。发作期间，体重增加高达18%，白细胞计数达到每微升108,000（嗜酸性粒细胞占88%）。该疾病似乎并未威胁重要器官的功能。两名儿童间歇性接受泼尼松治疗；成人无需治疗或隔日给予泼尼松。糖皮质激素治疗导致退热、利尿，并使白细胞总数和嗜酸性粒细胞计数下降。所有患者均无心脏受累的证据（随访2至17年）。1例患者自发缓解20年后症状复发。组织学研究显示，嗜酸性粒细胞在真皮中定位并脱颗粒，且似乎会引发水肿。尽管该综合征可能被归类为高嗜酸性粒细胞综合征的一种变体，但因其独特的特征和良性病程，我们认为它是一个独立的病种。
