An Unusual Association of Hyper-IgE Syndrome with Celiac Disease: A Case Report
Ghita Hachim, Jihane El Mahi, Abdelilah Radi, Abdelhakim Ourrai, Rachid Abilkassem, Amal Hassani, Aomar Agadr
Journal of Advances in Medical and Pharmaceutical Sciences · pp. 8–13 · Published 25 Oct 2022
10.9734/jamps/2022/v24i9575Abstract
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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