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Primary immunodeficiency syndrome represents a heterogeneous group of inherited disorders caused by mutations in genes encoding functional proteins of the immune cells and characterized by an increased risk of infections, autoimmunity, autoinflammation, malignancy, and allergic disorders. Hereby, we report an 8-year-old-male child with complaints of severe itchy lesions over the body since infancy. Thorough investigations showed he had primary immunodeficiency with underlying hyperimmunoglobulin E syndrome.