An Unusual Association of Hyper-IgE Syndrome with Celiac Disease: A Case Report
Ghita Hachim *
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Jihane El Mahi
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Abdelilah Radi
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Abdelhakim Ourrai
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Rachid Abilkassem
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Amal Hassani
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
Aomar Agadr
Department of Pediatrics, Mohammed V Military Instruction Hospital, Rabat, Morocco.
*Author to whom correspondence should be addressed.
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.
Keywords: HyperIgE syndrome, celiac disease, immune deficiency, infection