Disease Information for Job syndrome (Hyper IgE syndrome)

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Clinical Manifestations
Signs & Symptoms
Carbuncle/recurrent/present/sign
Cold abscess/skin
Furuncles present
Painful subcutaneous nodules
Reddish nodule/skin
Tender nodules
Hyperextensible digits
Hyperextensible joints Laxity Instability
Chronically ill patient/signs
Clinical Presentation & Variations
Presentation/Recurrent Infections
Presentation/Lymphopenia Candidal diarrhea
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Recurrent illness pattern
Demographics & Risk Factors
Established Disease Population
Patient/Red hair
Population Group
Child
Infant
Population/Pediatrics population
Family History
Family history/Immune defects
Sex & Age Groups
Population/Boy patient
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Female
Population/Girl patient
Population/Infant
Population/Infant female
Population/Infant male
Population/Male
Laboratory Tests
Microbiology & Serology Findings
Microlab/Sputum/Eosinophiles on smear
Abnormal Lab Findings (Non Measured)
Chronic lymphopenia
Abnormal Lab Findings - Decreased
Lymphocytes (Lab)
Abnormal Lab Findings - Increased
Anti-staphylococcal IGE antibodies (Lab)
Eosinophiles (LAB)
IGE/Immunoglobulin E (Lab)
Lymphocytes (Lab)
Diagnostic Test Results
Other Tests & Procedures
Skin test anergy
Associated Diseases & Rule outs
Rule Outs
Acne Fulminans
Eczema/Dermatitis, atopic
Associated Disease & Complications
Anergic status
Bronchitis, acute
Candida albicans infection
Carbuncle
Cellular immunity defect/deficiency
Cutaneous abscess/subcutaneous
Eczema/Dermatitis, atopic
Eosinophilia Hypereosinophilia
Furunculosis
Immunodeficiency
Infections
Job syndrome
Lymphocytopenia
Lymphocytosis
Metastatic staphylococcus abscess syndrome
Muscle abscess
Otitis externa/acute
Pneumonia, staphylococcus
Sinusitis
Staphylococcus aureus infection
Staphylococcus sepsis
Multiple Abscesses
Disease Mechanism & Classification
Class
CLASS/Humoral Immune System Disorder (ex)
CLASS/Pediatric disorders (ex)
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Spleen/thymus/RES/immune system (category)
Pathophysiology
Pathophysiology/Abscess tendency
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Necrosis and abscess formation
Pathophysiology/Chemotaxis failure/phagocytes/effect
Pathophysiology/Decreased T Cells
Pathophysiology/Defective cellular immunity
Pathophysiology/Immune physiology/defective
Pathophysiology/Immune/cellular system disorder (ex)
Pathophysiology/Impaired neutrophile chemotaxis/dermis
Pathophysiology/Poor anamnestic response/vaccines
Process
PROCESS/Allergy/collagen/autoimmune (category)
PROCESS/Congenital/developmental (category)
PROCESS/Eponymic (category)
PROCESS/Hereditary/Genetic predisposition (ex)
PROCESS/Hereditofamilial (category)
PROCESS/Immunodeficiency disorder/Primary
Synonyms
Synonym
HIE, Hyper IgE, Synonym/Atopic dermatitis/Neutrophile chemotaxis def, Synonym/Buckley's syndrome, Synonym/Hyper IGE/Recurrent infection syndrome, Synonym/Hyper-IGE syndrome, Synonym/Hyperimmunoglobulin E syndrome, Synonym/Job-Buckley syndrome, Synonym/Neutrophile chemotaxis failure/atopic derm.
Treatment
Drug Therapy - Indication
RX/Antibiotic
RX/Anti-staph/synthetic penicillin
RX/Prophylactic measures/medication
Surgical Procedures or Treatments
SX/Surgery Incision/Drainage
Definition

Job Syndrome; HIE Syndrome; Hyper-IgE Syndrome; Hyperimmunoglobulin E Syndrome; Hyperimmunoglobulin E-Recurrent Infection Syndrome; Hyperimmunoglobulinemia E-Staphylococcal; Job-Buckley Syndrome; Job Syndrome is an immunodeficiency syndrome characterized by recurrent bacterial (staphylococcal) infections, particularly of the skin, and markedly elevated IgE [Immunoglobulin E] levels; some autosomal dominant inheritance; Staphylococcal furuncles may involve the skin, lungs, joints and other sites; Some patients have coarse features; some are fair and redheaded; Decreased bone density and frequent fractures are common; Some dysfunctional neutrophils; Signs of allergy such as eczema, asthma and/or runny noses often; Treatment consists of intermittent or continuous antibiotics; Sexes are about equal frequency--------(NORD 2005)------------------------

Features include: coarse facies; lung infections;

skin infections; cold abscesses, which may affect bone and skin with staph as well as candida--------[gpnotebook website 2005]----------------

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External Links Related to Job syndrome (Hyper IgE syndrome)
Google
Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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