Hyperimmunoglobulin E

From Anvita Health Wiki

Contents 1 Epidemiology 2 Pathology 3 Genetics 4 Clinical-manifestations 5 Laboratory 6 Management 7 More General Terms 8 Additional Terms 9 References

Epidemiology

• rare

Pathology

• associated with primary immunodeficiency
• phagocyte dysfunction

• reduced chemotaxis (some patients)

• reduced suppressor T-cell activity

• skewed T helper 1 ( Th1) cell/ Th2 cell ratio

• involvement of chemokines

Genetics

• most cases are sporadic
• autosomal dominant & autosomal recessive forms
• associated with defects in STAT3

Clinical-manifestations

• chronic eczematoid or pruritic dermatitis
• recurrent staphylococcal abscesses
• sporadic & autosomal dominant

• generally part of a multisystem disorder including

• abnormalities of soft tissue, skeleton, & dentition

• recurrent pneumonias with Staphylococcus aureus

• bronchopleural fistulas
• cyst formation
• bronchiectasis
• pneumatoceles

• scoliosis

• autosomal recessive

• severe molluscum contagiosum & other viral infections
• may develop severe neurological complications
• lack skeletal or dental involvement
• do not develop lung cysts

• other variants

• restrictive lung disease
• sinusitis
• mucocutaneous candidiasis
• atopy
• coarse facies

Laboratory

• high serum levels of IgE & IgD

• serum IgE > 2000 IU/mL
• high serum anti S. aureus IgE

• normal serum levels of IgA, IgG & IgM

• low or absent serum & salivary anti S. aureus IgA

• sputum

• gram stain & culture

• Staphylococcus aureus
• Streptococcus pneumoniae
• gram negative bacilli

• fungal stains & culture

• Candida albicans
• Aspergillus species

• complete blood count

• mild eosinophilia

Management

• prevention & management of infections

• sustained systemic antibiotics & antifungals

• topical therapy for eczema
• drainage of abscesses
• anti- staphylococcal antibiotic prophylaxis may be useful
• interferons, immunoglobulin supplementation, & low-dose cyclosporine A of suggested benefit in selected patients, but generally not indicated

More General Terms

• lung disease
• immunologic disease
• skin disease (dermatologic disorder, dermatopathy, dermatosis)
• phagocytic disorder
• syndrome

Additional Terms

• IgE in serum
• immunoglobulin-E (IgE)

References

1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 747
2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 356
3. Grimbacher B, Holland SM, Puck JM. Hyper-IgE syndromes. Immunol Rev. 2005 Feb;203:244-50. Review. PMID: [1]

hyperimmunoglobulin E (Job's syndrome)