Bigdelu Leila, Maadarani Ossama, Azari Ali, Heidari-Bakavoli Ali, Oskoueyan Zahra, Bitar Zouhair
Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Critical Care Unit, Internal Medical Department, Ahmadi Hospital-Kuwait Oil Company, Al Ahmadi, Kuwait.
JACC Case Rep. 2025 Sep 17;30(28):104911. doi: 10.1016/j.jaccas.2025.104911.
Hyperimmunoglobulin E syndromes (HIES) are a heterogeneous group of primary immunodeficiencies sharing manifestations including recurrent lung infection, staphylococcal skin abscesses, eczema, and significantly raised serum levels of immunoglobulin E. Arterial aneurysms are among the rare vascular manifestations of HIES that may lead to devastating outcomes. We present a case of a 17-year-old girl who had a known history of HIES since childhood. She manifested a recurrent chest infection and skin rash, and it was discovered that she had a large thoracic descending aortic aneurysm, which required surgical repair to prevent devastating complications.
高免疫球蛋白E综合征（HIES）是一组异质性原发性免疫缺陷病，具有反复肺部感染、葡萄球菌皮肤脓肿、湿疹以及血清免疫球蛋白E水平显著升高等共同表现。动脉瘤是HIES罕见的血管表现之一，可能导致严重后果。我们报告一例17岁女孩，她自幼就有HIES病史。她表现为反复的胸部感染和皮疹，经检查发现她患有一个巨大的胸降主动脉瘤，需要进行手术修复以预防严重并发症。
