Indian Journal of Dermatology
CASE REPORT
Year
: 2018  |  Volume : 63  |  Issue : 1  |  Page : 79--81

Autosomal-Recessive Hyper-IgE syndrome


Mohapatra Liza, Dash Gaurav, Mohanty Prasenjeet, Jena Swapna, Behera Binodini 
 Department of Skin and VD, SCB Medical College, Cuttack, Odisha, India

Correspondence Address:
Dr. Mohapatra Liza
Department of Skin and VD, SCB Medical College, Cuttack - 753 007, Odisha
India

The hyper-IgE syndrome (HIES) is a rare group of primary immunodeficiency characterised by recurrent infections, eczema, and elevated serum levels of IgE. Autosomal dominant HIES is caused by mutations in transcription factor – signal transducer and activator of transcription-3. Autosomal-recessive (AR) HIES was described in 2004 due to mutation of tyrosine kinase 2 gene, and subsequently, another mutation in dedicator of cytokinesis 8 gene was discovered in 2009. Although both the forms have many common clinical features, few characteristic findings help in differentiating them. AR-HIES is characterized by recurrent bacterial and viral infections, atopic eczema, and raised serum IgE levels. We report a case of a 4-year-old girl presenting with the features of AR-HIES to highlight the presentation of this rare disease.


How to cite this article:
Liza M, Gaurav D, Prasenjeet M, Swapna J, Binodini B. Autosomal-Recessive Hyper-IgE syndrome.Indian J Dermatol 2018;63:79-81


How to cite this URL:
Liza M, Gaurav D, Prasenjeet M, Swapna J, Binodini B. Autosomal-Recessive Hyper-IgE syndrome. Indian J Dermatol [serial online] 2018 [cited 2019 Oct 21 ];63:79-81
Available from: http://www.e-ijd.org/article.asp?issn=0019-5154;year=2018;volume=63;issue=1;spage=79;epage=81;aulast=Liza;type=0