Hyperimmunoglobulin E syndrome in adult patients: a case report and literature review

VernacularTitle:成人高IgE综合征五例并文献复习
Author: Na XU ¹ ; Hongwei FAN ; Jing ZHAO ; Peng WANG ; Hongmei SONG ; Xuejun ZENG
Author Information

1. 100730,中国医学科学院北京协和医学院北京协和医院普通内科
Keywords: Hyperimmunoglobulin E; Infection
From: Chinese Journal of General Practitioners 2017;16(12):955-960
CountryChina
Language:Chinese
Abstract: The clinical manifestations,laboratory test results,treatments and prognoses of 5 adult patients with hyperimmunoglobulin E syndrome (HIES) were retrospectively analysed.All 5 patients experienced disease onset within 1 year of birth and had recurrent multiple infections,all had recurrent rash and 4 had skeletal abnormalities.Recurrent infections lasted for a mean of 19.6 years and predominantly affected the skin and respiratory tract.Other manifestations included chronic otitis media,renal abscess and osteomyelitis.Two cases had skin abscess (methicillin-susceptible Staphylococcus aureus infection),which was alleviated after antibiotic treatment and abscess drainage.Two cases had respiratory tract infection and one case had a foot skin ulcer,and all symptoms improved after antibiotic treatment.HIES should be suspected when an adult patient experiences repeated infection since birth,particularly when skeletal abnormalities and a history of neonatal rash is also present.Early diagnosis facilitates targeted anti-infection therapy,leading to earlier remission and an improved prognosis.