hyper ige syndrome (hies) is a rare primary immunodeficiency disease. most of hies cases are sporadic. hies type ad is caused by mutation in signal transducer and activator of transcription-3 (stat-3). a number of mosaicism hies has been reported that is associated with intermediate phenotype. autosomal recessive hies (ar-hies) is due to mutation in dock-8 or cytokine sis 8 and tyk2 or tyrosine...

the hyperimmunoglobulin e  syndrome is a rare complex primary immunodeficiency characterized by high serum ige levels, eczema, and recurrent infections. we present a case of 2-years-old girl with eczema and repeated bacterial skin and lung infections since the period of infancy. the patient also had eosinophilia, high serum levels of ige, and cows-milk hypersensitivity. we describe the case, in...

Background: Hyper IgE syndrome (HIES) is a rare primary immune deficiency, described as Job`s syndrome characterized by increased serum levels of IgE, eczema, recurrent cutaneous and pulmonary infections. In this paper, we presented a case of Hyper IgE syndrome.Case Presentation: A 16-year-old Iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of pso...

background: hyper ige syndrome (hies) is a rare primary immune deficiency, described as job`s syndrome characterized by increased serum levels of ige, eczema, recurrent cutaneous and pulmonary infections. in this paper, we presented a case of hyper ige syndrome.case presentation: a 16-year-old iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of pso...

The hyper-IgE syndromes are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity, by particular susceptibility to staphylococcal and mycotic infections as well as by a heterogeneous genetic origin. Two distinct entities--the classical hyper-IgE syndrome which is inherited in an autosomal dominant pattern and the autosomal recessive hyper-IgE syndrome--have ...

BACKGROUND Hyper IgE syndrome (HIES) is a rare primary immune deficiency, described as Job(`)s syndrome characterized by increased serum levels of IgE, eczema, recurrent cutaneous and pulmonary infections. In this paper, we presented a case of Hyper IgE syndrome. CASE PRESENTATION A 16-year-old Iranian boy presented with a one year history of skin lesions in knees and elbows was diagnosed of ...

hyper - immunoglobulin e syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum ige associated with peculiar face and skeletal features. we report a seven-year old  girl  presenting with persistent productive cough and  history of chronic eczematoid facial lesions since infancy  and  two episod...