高 IgE 综合征一例报告并文献复习

摘要

Objective To investigate the pathogenesis, keypoints of diagnosis and treatment and to reduce misdiagno-sis and mistreatment of Hyper IgE Syndrome. Methods The clinical data of a case of Hyper IgE Syndrome was analyzed and related literature was reviewed. Results The patient was admitted for generalized recurrent abscess for ten years. He had been diagnosed with Furuncle and treated with anti-infection medication therapy and incision and drainage many times in other hospitals. According to the positive culture for staphylococcus aureus after abscess puncture and significantly increased level of serum IgE detected in our hospital, the patient was confirmed with HIES. After systemic treatment such as anti-infection and adjusting immune system, the patient recovered and was discharged. Conclusion Early clinical manifestations of HIES is un-specific and easy to be misdiagnosed and mistreated. Clinicians should pay attention to the possibility of HIES in the infant pa-tients with refractory furunculosis and atopic dermatitis. Relevent tests can help to avoid misdiagnosis and mistreatment.%目的：探讨高 IgE 综合征(hyper-IgE syndrome, HIES)的发病机制及诊治要点,减少误诊误治。方法对我院收治的高 IgE 综合征1例的临床资料进行回顾性分析,并复习相关文献。结果本例因全身反复脓肿10余年入院,外院按疖肿多次予抗感染及切开引流治疗,后在我院经颈部脓肿穿刺脓液细菌培养示有金黄色葡萄球菌生长、查血清 IgE 显著升高确诊为 HIES。予抗感染、调节免疫等治疗后病情好转出院。结论 HIES 早期临床表现无特异性,易误诊误治。对婴儿时期出现难治性疖肿、特应性皮炎者,应考虑到 HIES 的可能,及时行相关检查,避免误诊误治。