IDF US Information: Hyper IgE Syndrome
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چکیده
Hyper-IgE syndrome (HIES) is a complex primary immunodeficiency disorder characterized by a spectrum of abnormalities related to the immune system, bones, connective tissue and teeth. The cause of HIES is not known. The disease is also known as Job Syndrome because skin boils, a hallmark of the syndrome, are reminiscent of the biblical character Job, who was smitten by Satan “with sore boils from the sole of his foot unto his crown.” HIES was initially defined as a triad of clinical problems involving skin boils, severe episodes of pneumonia and very high serum IgE levels.
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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...
متن کاملPsoriasis 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...
متن کاملTh17 Lymphocytes Percentage in Peripheral Blood of Iranian Patients with Autosomal Recessive Hyper IgE Syndrome
Background and Aims: Patients with Hyper-IgE syndrome suffer from fungal and bacterial infections, especially Candida albicans and Staphylococcus aureus. Due to the important role of T helper17 (Th17) lymphocytes in defense against fungal infections, the percentage of Th17 lymphocytes was studied in the patients with autosomal recessive hyper-IgE syndrome (AR-HIES). Materials and Methods: In...
متن کاملThe hyperimmunoglobulin E syndrome - clinical manifestation diversity in primary immune deficiency
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 ...
متن کاملRecurrent Skin and Lung Infections in Autosomal Dominant Hyper IgE Syndrome with Transactivation Domain STAT3 Mutation
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...
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