Clinical manifestation of hyper-immunoglobulin E syndrome (HIES) and the differential diagnosis of HIES and atopic dermatitis
Abstract：Hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by immune and connective tissue abnormalities. With the primary manifestations of eczematous dermatitis, elevated serum levels of IgE and eosinophilia, HIES and atopic dermatitis(AD) which were commonly seen in children, have many similarities of skin symptoms. HIES patients also suffer from problems more than those of the immune system such as significant recurrent bacteria or virus infections, skeletal dysplasia, and the malignancies are also likely to be found in some of the patients. Due to the numerous resemblances on clinical manifestations and lab experimental results between HIES and AD, and the fact that HIES is an uncommon disease with low morbidity, as well as clinicians lack sufifcient understanding on this disease, clinicians are likely to omit or mistaken it for AD in clinical practice. This article aims to help the clinicians deepen the understanding of the disease, through the comprehensive comparison and analysis of the characteristics of the manifestations of HIES and its similarities and differences with the AD, so as to minimize the risks of omission or misdiagnose.
Keywords：Hyper-immunoglobulin E syndromeDermatitisatopicDifferential diagnosis
Publication Date：2016-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 384-386,390 )