Primary presentation |
Sepsis and bacteremia (gram-negative bacteria, encapsulated pathogens) |
Diarrhea |
Dermatitis |
Frequent upper respiratory infections |
Meningitis |
Other reported presentations |
Elevated IgE |
Allergic disease |
Chronic idiopathic/immune thrombocytopenic purpura |
Large warts (epidermodysplasia verruciformis) |
Splenomegaly |
Growth retardation in children |
Possible presentations |
Autoimmunity (increased response to self-antigens?) |
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