Syndrome of Elevated Immunoglobulin E Eczema and Recurrent Infection Job Syndrome

Clinical Manifestations

• Chronic eczema, delay in shedding primary teeth, hyperextensible joints, scolio-sis, osteopenia, and tendency to fractures, growth retardation, and coarse facies

• Recurrent severe staphylococcal abscesses

• Recurrent cutaneous, pulmonary, and joint abscesses

• Chronic candidiasis of mucosa and nails.

Laboratory Findings

• Very high serum IgE level (greater than 2500 IU/mL)

• Defect in T lymphocytes that results in reduced production of IFN-y and tumor necrosis factor

• Molecular basis of hyper IgE syndrome unknown

• Striking defect in neutrophil granulocyte chemotactic responsiveness; neutrophil migration, phagocytosis, and bactericidal activity normal.

Treatment

• Prophylactic antibiotics: trimethoprim/sulfamethoxazole or dicloxacillin

• rhIFN-y: 50 |g/m2 subcutaneously three times per week; however, evidence-based data for its efficacy are not available.

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