EPISODIC ANGIOEDEMA ASSOCIATED WITH EOSINOPHILIA
EPISODIC ANGIOEDEMA ASSOCIATED WITH EOSINOPHILIA
复制标题
DOI：
10.1056/nejm198406213102501
复制
发表时间：
1984-01-01
影响因子：
158.5
通讯作者：
KOHLER, PF
中科院分区：
文献类型：
--
作者：
GLEICH, GJ;SCHROETER, AL;KOHLER, PF
研究对象为4例反复发作的血管性水肿、荨麻疹和发热患者。在攻击期间，体重增加了18%，白细胞计数达到10.8万个/微升(88%的嗜酸性粒细胞)。这种疾病似乎没有威胁到重要器官的功能。两名儿童间歇性地接受泼尼松治疗；成年人不需要治疗或隔日服用强的松。糖皮质激素治疗导致退热和利尿，并降低总白细胞和嗜酸性粒细胞计数。没有患者有心脏受累的证据(随访2-17年)。1例患者在症状复发前保持了20年的自然缓解状态。组织学研究表明，嗜酸性粒细胞定位于真皮中并脱颗粒，它们似乎能引起水肿。虽然这种综合征可能被归类为高嗜酸性粒细胞综合征的变体，但由于其独特的特征和良性的病程，它被认为是一个独立的实体。
Four patients with recurrent attacks of angioedema, urticaria, and fever were studied. During attacks, body weights increased up to 18%, and leukocyte counts reached 108,000/ul (88% eosinophils). The disease did not appear to threaten the function of vital organs. The 2 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 2-17 yr). One patient remained in spontaneous remission for 20 yr 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, it is believed to be a separate entity because of its distinctive characteristics and its benign course.