This is an X-linked immunodeficiency disorder which presents with eczema, thrombocytopenia (bleeding), and recurrent otitis media. There is an increased risk of lymphoproliferative disease.

Patients have increased levels of IgE and IgA, and decreased levels of IgM. They get recurrent infections from S. pneumonia, S. aureus, H. influenza Type B, CMV, PCP, and HSV.

Treatment is supportive (IVIG and antibiotics), and patients frequently die of hemorrhage from the thrombocytopenia (rarely survive into adulthood).