Merten D F, Buckley R H, Pratt P C, Effmann E L, Grossman H
Radiology. 1979 Jul;132(1):71-8. doi: 10.1148/132.1.71.
Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2--8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.
婴儿期起病的复发性葡萄球菌皮肤和呼吸道感染易感性与极高的免疫球蛋白E血症相关，这是一种最近才被描述的原发性免疫缺陷综合征。其他临床特征包括细胞免疫低下和抗体形成不足。11例患者的影像学病程以复发性肺炎和囊肿形成为特征，囊肿持续存在并不断扩大。5例囊肿经持续抗葡萄球菌治疗后消退；2例经手术切除未复发；4例在手术和/或使用抗生素后囊肿仍持续存在（2 - 8年）。囊肿表面致密、坏死，有纤维壁，有嗜酸性粒细胞和其他炎症细胞浸润，且常有持久的支气管连接。影像学检查还发现鼻窦炎（9/9）和乳突炎（3/4）。
