Job syndrome (hyper-IgE syndrome) - DermNet
Job syndrome, also called hyper- IgE syndrome, is the name given to a rare inherited immune deficiency characterised by severe infections of the skin and other organs. What are the clinical features of Job syndrome? Clinical features of Job syndrome include: High levels of eosinophils in the blood. What is the cause of Job syndrome?
Hyper IgE syndrome | Immune Deficiency Foundation - Primary …
Hyper IgE Syndromes (HIES) are rare forms of primary immunodeficiencies (PI) characterized by recurrent eczema, skin abscesses, lung infections, eosinophilia (high numbers of eosinophils in the blood), and high serum levels of immunoglobulin E (IgE).
Hyperimmunoglobulin E syndrome - Wikipedia
Autosomal dominant Hyper-IgE Syndrome caused by STAT3 defects, called Job Syndrome, have characteristic facial, dental, and skeletal abnormalities. Patients with STAT3 HIES may have either delay of or failure in shedding of primary teeth. …
The Hyper IgE Syndromes - PMC - PubMed Central (PMC)
The hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, rash and recurrent bacterial infections of the skin and lung. Autosomal dominant HIES, the most common disease in this group, results from STAT3 ...
The Many Faces of the Hyper-IgE Syndrome - ScienceDirect
2014年1月1日 · Typical findings include characteristic asymmetric facies, scoliosis and hyperextensible joints; recurrent fractures upon minimal trauma, especially in the long bones; and delayed shedding of three or more primary teeth, which is probably caused by the lack of resorption of primary tooth roots 7., 8., 9..
Hyper-IgE syndrome: a case report - PMC - PubMed Central (PMC)
Characteristic facial features that typically appear in early adolescence or late childhood include asymmetry, a prominent forehead, deep-set eyes, a broad nasal bridge, a wide fleshy nasal tip, rough facial skin, an increased inter-alar distance, prognathism, and a high-arched palate.
Hyper IgE syndrome - PMC - National Center for Biotechnology …
Hyper IgE syndrome (HIES) is a rare disorder characterized by eczema, recurrent infections of the skin and lungs, typically with Staphylococcus aureus, Candida albicans and certain viruses, and elevated levels of serum IgE. Other clinical manifestations include characteristic facies (prominent forehead, broad nasal bridge and facial asymmetry ...
Hyper-IgE syndrome, 2021 update - ScienceDirect
2021年10月1日 · Clinically and pathologically, the patients with hyper-IgE syndrome present similar skin manifestations to common atopic dermatitis. The original hyper-IgE syndrome is characterized by diminished inflammatory response, in combination with Staphylococcus aureus skin abscess and pneumonia followed by pneumatocele formation.
Entry - #147060 - HYPER-IgE SYNDROME 1, AUTOSOMAL …
Hyper-IgE syndrome-1 with recurrent infections (HIES1) is an autosomal dominant immunologic disorder characterized by chronic eczema (atopy), recurrent Staphylococcal infections, increased serum IgE, and eosinophilia.
The Hyper-IgE Syndromes - Immunology and Allergy Clinics
The hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, rash, and recurrent bacterial infections of the skin and lung. Autosomal dominant HIES, the most common disease in this group, results from STAT3 mutations and has a variety of connective tissue and skeletal abnormalities.
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