Common variable immunodeficiency (CVID) is a rare primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections especially in respiratory and gastrointestinal systems. The authors present here a case of CVID with uncommon gastrointestinal manifestation in the form of CVID enteropathy. Based upon the clinical presentation and histopatholic findings the patient was diagnosed as CVID enteropathy and treated with intravenous antibiotics and 3-4 wkly intravenous immunoglobulin infusion and he had responded poorly to gastrointestinal symptoms but the frequency of respiratory and skin infection reduced. CVID has heterogenous gastrointestinal manifestations; among them the CVID enteropathy is an uncommon presentation which responds poorly to the mainstay therapy of intravenous immunoglobulin infusion and represents a diagnostic as well as therapeutic challenge for treating physician.
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