Katzen D R, Leiferman K M, Weller P F, Leung D Y
Am J Dis Child. 1986 Jan;140(1):62-4. doi: 10.1001/archpedi.1986.02140150064038.
A 2 1/2-year-old girl presented with monthly episodes of angioneurotic edema, eruption of pruritic papules, and fever. During acute episodes, white blood cell counts rose as high as 52,100/cu mm with 62% eosinophils, and body weights increased up to 20% of remission weight. Short courses of prednisone acetate caused rapid defervescence, resolution of angioneurotic edema, and lowering of eosinophil counts. In a one-year follow-up no evidence was found for cardiac or other visceral organ involvement. Findings of extensive diagnostic evaluations revealed no evidence for atopy, neoplasm, collagen-vascular disease, or parasitic infestation. Results of immunologic studies were essentially normal with the exception that this patient had a high level of circulating activated helper T cells. Biopsy specimens of the skin lesions revealed dermal infiltration of lymphocytes and eosinophils with deposition of eosinophil major basic protein in the extracellular matrix. Awareness of this clinical entity and its distinction from the hypereosinophilic syndrome is important because of its favorable prognosis and rapid response to corticosteroid therapy.
一名2岁半女童，每月发作血管性水肿、瘙痒性丘疹及发热。急性发作期，白细胞计数高达52,100/立方毫米，嗜酸性粒细胞占62%，体重增加至缓解期体重的20%。短期服用醋酸泼尼松可使体温迅速下降、血管性水肿消退及嗜酸性粒细胞计数降低。随访一年，未发现心脏或其他内脏器官受累迹象。广泛的诊断评估结果未发现特应性、肿瘤、胶原血管病或寄生虫感染证据。免疫学研究结果基本正常，只是该患者循环活化辅助性T细胞水平较高。皮肤病变活检标本显示真皮层淋巴细胞和嗜酸性粒细胞浸润，细胞外基质中有嗜酸性粒细胞主要碱性蛋白沉积。认识到这一临床实体及其与高嗜酸性粒细胞综合征的区别很重要，因为其预后良好且对皮质类固醇治疗反应迅速。
