Hyper-IgE and recurrent infection syndrome (Job's syndrome): a rare disease with common problems

معرفی یک مورد سندرم ازدیاد Hyper-IgE Syndrome) IgE)

Hyper IgE syndrome (Job’s syndrome) is a primary immunodeficiency disease with recurrent infections especially staphylococcal, coarse face, skeletal abnormality and significant increase in serum IgE level (IgE >2000IU/ml). We present a 16 years old boy admitted with chronic cough, dyspnea, eczema and pneumatocele. He had a history of chronic dermal infection since 1 month after birth. The dia...

Psoriasis in hyper IgE syndrome – a case report

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...

Hyper-IgE syndrome

Autosomal dominant hyper-IgE syndrome

Th e hyper-IgE recurrent infection syndromes (HIES) comprise a group of primary immunodefi ciency dis orders that exhibit markedly elevated IgE levels, recur rent staphylococcal skin abscesses, eczema and pulmonary infections. Both autosomal dominant and autosomal recessive forms of the disorder have been described. Most autosomal dominant HIES (AD-HIES) have been found to be due to mutations i...

Pediatric anaphylaxis and hyper IgE syndrome

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...