Abstract
Hyper-IgE syndrome (HIES) is a primary immunodeficiency disorder characterized by eczema, cold abscesses, pneumonia, eosinophilia, and a very high serum IgE concentration. An association with celiac disease is rare. Immunodeficiency and autoimmunity are two manifestations of immune system dysfunction that can be associated with common pathophysiological links. We present the case of a 3-year-old child with psychomotor retardation and a history of recurrent infections who had generalized eczema, failure to thrive, and abdominal distension with hepatosplenomegaly. The patient improved after receiving a monthly intravenous immunoglobulin infusion and a gluten-free diet.
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