Clinical Analysis of hyper-immunoglobulin E syndrome

Objective To summarize the clinical characteristics of patients with hyper-immunoglobulin E syndrome (HIES). Methods The clinical data of HIES patients, who were hospitalized in Peking Union Medical College Hospital from January 2000 to December 2013, were retrospectively analyzed. Results Two 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. Conclusion HIES 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