Cruz-Portelles A, Estopiñan-Zuñiga D
Critical Care Medicine Department, V. I. Lenin University General Hospital, Holguín, Cuba.
Internal Medicine Department, V. I. Lenin University General Hospital, Holguín, Cuba.
Rev Port Pneumol. 2014 Mar-Apr;20(2):107-10. doi: 10.1016/j.rppneu.2013.07.007. Epub 2014 Feb 18.
Job's syndrome or Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent soft tissue infections, coarse face, skeletal and vascular abnormalities, and markedly high levels of Immunoglobulin E. Eczema that resembles atopic dermatitis but is refractory to traditional treatment and severe and recurrent bacterial pneumonias often recognized during childhood. Early diagnosis and treatment prevent progressive pulmonary sequellae and increase survival. About 200 cases of HIES has been reported worldwide. The authors report a new case of HIES with one of the worst pulmonary sequellae found in the literature on this subject and review this infrequent topic.
乔布综合征或高免疫球蛋白E综合征（HIES）是一种罕见的原发性免疫缺陷病，其特征为反复出现软组织感染、面容粗糙、骨骼和血管异常以及免疫球蛋白E水平显著升高。湿疹类似特应性皮炎，但对传统治疗无效，严重且反复的细菌性肺炎常在儿童期出现。早期诊断和治疗可预防进行性肺部后遗症并提高生存率。全球已报道约200例HIES病例。作者报告了一例新的HIES病例，其肺部后遗症是该主题文献中发现的最严重的病例之一，并对这一罕见主题进行了综述。
