• 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.
• 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.
• 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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