Stover Daniel G, Freeman Alexandra F, Wright Patty W, Hummell Donna S, Ness Reid M
Department of Medicine and Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.
South Med J. 2010 Dec;103(12):1261-3. doi: 10.1097/SMJ.0b013e3181fa5f0e.
Autosomal dominant hyperimmunoglobulin E syndrome (HIES, or Job syndrome) is a rare primary immunodeficiency characterized by elevated immunoglobulin E (IgE), eosinophilia, recurrent skin and pulmonary infections, dermatitis, and connective tissue and skeletal abnormalities. A 26-year-old male with known HIES presented with abdominal pain and diarrhea. Imaging showed sigmoid diverticulitis without abscess or perforation. Conservative management with antibiotics failed, and he developed a peridiverticular abscess, which was percutaneously drained with plans for elective resection. He returned four days later with progression of his diverticulitis, requiring partial colectomy with primary anastomosis. To our knowledge, this is the first case of diverticulitis in HIES. Diverticulitis is rare in younger individuals, raising the possibility that the connective tissue abnormalities of HIES patients may predispose them to colonic diverticula. Although the majority of complications are sinopulmonary and skin infections, diverticulitis should be considered in the differential of intra-abdominal processes in HIES.
常染色体显性高免疫球蛋白E综合征（HIES，即乔布综合征）是一种罕见的原发性免疫缺陷病，其特征为免疫球蛋白E（IgE）升高、嗜酸性粒细胞增多、反复发生皮肤和肺部感染、皮炎以及结缔组织和骨骼异常。一名已知患有HIES的26岁男性出现腹痛和腹泻。影像学检查显示乙状结肠憩室炎，无脓肿或穿孔。使用抗生素的保守治疗失败，他发展为憩室周围脓肿，经皮引流，并计划择期切除。四天后他因憩室炎进展再次就诊，需要行部分结肠切除术并一期吻合。据我们所知，这是HIES患者发生憩室炎的首例病例。憩室炎在年轻人中很少见，这增加了HIES患者的结缔组织异常可能使他们易患结肠憩室的可能性。尽管大多数并发症是鼻窦肺部和皮肤感染，但在HIES患者腹部疾病的鉴别诊断中应考虑憩室炎。
