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 (IgE) is a disorder of immune system characterized by assemblage symptoms including recurrent skin and pulmonary infections, skeletal anomalies elevated serum IgE (>2000 IU/ml). More recently, apart from the typical features, neurological manifestations have also been described central nervous facial nerve palsy demyelinating disorders. Here we describe three patients ...

Background: Job’s syndrome or hyper-immunoglobulin E (IgE) (HIES) is an extremely rare primary immunodeficiency disease with approximate annual incidence of less than 1/1000000. It characterized by recurrent cold staphylococcal infections, unusual eczematous dermatitis, severe lung and extensively high concentrations the serum antibody IgE. Case Presentation: A typical case a family history Kaw...

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 ...

Dubowitz syndrome is a rare, autosomal recessive disorder characterized by intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, and psychomotoric retardation. The hyper-immunoglobulin E (hyper-IgE or Job syndrome) is a primary immunodeficiency characterized by recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia, and an elevated serum IgE le...

Background. Hyper IgE is a rare systemic disease characterized by the clinical triad of high serum levels of IgE (>2000 IU/mL), eczema, and recurrent staphylococcal skin and lung infections. The presentation of hyper IgE syndrome is highly variable, which makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiency disorders. Case Report. A 23-year-old Hispani...

Patients with autosomal-dominant (AD) hyper immunoglobulin E (IgE) syndrome (HIES) or Job syndrome develop frequent dermatologic and pulmonary infections. As patients have an extreme elevation of IgE levels, this database analysis study sought to study the association between AD HIES, Job syndrome, and anaphylaxis. HIES is a heterogeneous group of immune disorders characterized by extremely ele...

objectives: the idiopathic minimal change nephritic syndrome (nephrosis) is responsible in 80% of nephritic syndromes in children. it is a clinical entity characterized by inter and outer renal parameters. main factor in glomerolar damages is proteinuria and the role of ige is possible. aim: in this research, serum ige concentrations were measured in children with nephrosis in three stages: rel...

Food protein-induced enterocolitis syndrome (FPIES) is a potentially severe non-IgE-mediated food allergy usually caused by cow's milk or soy, and more rarely by solid foods such as rice, oats, barley, chicken, turkey, egg white, green peas and peanuts. In children with FPIES, the presence of specific IgE antibodies to the causative food, either at presentation or during follow-up, defines an "...