Hyperimmunoglobulin E syndrome
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Notes
Hyperimmunoglobulin E syndrome
Immune system
Hypersensitivity reactions
Anaphylaxis
Asthma
Food allergy
Type I hypersensitivity
Autoimmune hemolytic anemia
Goodpasture syndrome
Graves disease
Hemolytic disease of the newborn
Myasthenia gravis
Pemphigus vulgaris
Rheumatic heart disease
Type II hypersensitivity
Poststreptococcal glomerulonephritis
Serum sickness
Systemic lupus erythematosus
Type III hypersensitivity
Contact dermatitis
Graft-versus-host disease
Type IV hypersensitivity
Immunodeficiences
Asplenia
Common variable immunodeficiency
Hyperimmunoglobulin E syndrome
IgG subclass deficiency
Isolated primary immunoglobulin M deficiency
Selective immunoglobulin A deficiency
X-linked agammaglobulinemia
Adenosine deaminase deficiency
Ataxia-telangiectasia
Hyper IgM syndrome
Severe combined immunodeficiency
Wiskott-Aldrich syndrome
Complement deficiency
Hereditary angioedema
Cytomegalovirus infection after transplant (NORD)
Post-transplant lymphoproliferative disorders (NORD)
Chediak-Higashi syndrome
Chronic granulomatous disease
Leukocyte adhesion deficiency
Immune system pathology review
Blood transfusion reactions and transplant rejection: Pathology review
Immunodeficiencies: Combined T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Phagocyte and complement dysfunction: Pathology review
Immunodeficiencies: T-cell and B-cell disorders: Pathology review
Assessments
Hyperimmunoglobulin E syndrome
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USMLE® Step 1 questions
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High Yield Notes
10 pages
Flashcards
Hyperimmunoglobulin E syndrome
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Questions
USMLE® Step 1 style questions USMLE
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External References
First Aid
2022
2021
2020
2019
2018
2017
2016
Autosomal dominant disorders
hyper-IgE syndrome p. 114
Autosomal dominant hyper-IgE syndrome
Eczema
hyper-IgE syndrome p. 114, 716
Hyper-IgE syndrome
presentation p. 716
Summary
Hyperimmunoglobulin E syndrome (HIES) is a genetic disorder caused by STAT3 gene mutation. It is characterized by Th17 cell deficiency, impaired neutrophil recruitment to sites of infection, and increased serum IgE concentrations. People with HIES physically present with coarse facial features, recurrent staphylococcal abscesses, retained primary teeth, and eczema.
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