Clinical Research
Clinical Analysis of hyper-immunoglobulin E syndrome
Li Wang, Bangqin Wang, Ling Lan, Yuting Zhang, Bo Yang, Lijuan Gou, Wenjie Zheng, Fengchun Zhang
Published 2015-09-15
Cite as Chin J Rheumatol, 2015, 19(9): 598-600. DOI: 10.3760/cma.j.issn.1007-7480.2015.09.005
Abstract
ObjectiveTo summarize the clinical characteristics of patients with hyper-immunoglobulin E syndrome (HIES).
MethodsThe clinical data of HIES patients, who were hospitalized in Peking Union Medical College Hospital from January 2000 to December 2013, were retrospectively analyzed.
ResultsTwo were female and 5 were male, with the mean age of (10±4) year. Eczema (7/7) was the most common symptom followed by atopic dermatitis (6/7), pustular and folliculitis (6/7), pneumonia (6/7), pathologic fracture (3/7), articular (2/7) and muscular (2/7) infections. Increased serum immunoglobulin (Ig) E (406->5 000 U/ml) was found in all cases. Eosinophilic granulocytes elevated in 4 cases. Haemophilus influenza and staphylococcus epidermidis were the common pathogens in these patients. Treated by antimicrobial (7/7) and immunoen-hancers such as intravenous immunoglobulin (IVIG, 2/7), 6 patients clinically improved, 1 patient left the hospited due to non-medical reasons.
ConclusionHIES is a rare primary immune deficiency syndrome characterized by elevated serum IgE level, dermatitis, recurrent skin and lung infections, and musculoskeletal abnormalities. It needs to be distinguished with rheumatic diseases and can be ameliorated by antimicrobial and symptomatic treatment.
Key words:
Job's syndrome; Immunoglobulin E; Disease atributtes
Contributor Information
Li Wang
Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100730, China
Bangqin Wang
Department of Immunology and Rheumatology, the People's Hospital of Guangxi Zhuang Autonomous Region, Nanning 530021, China
Ling Lan
Yuting Zhang
Bo Yang
Lijuan Gou
Wenjie Zheng
Fengchun Zhang