AUTOSOMAL DOMINANT HYPER IGESYNDROME (JOB’S SYNDROME): A CASE REPORT OF TWO PATIENTS

Abstract

Recently we came across two male patients with Hyper IgE type immune deficiency (HIES/Job’s syndrome), a primary immune deficiency disorder characterized by abnormally raised serum levels of IgE and repeated childhood infections. In our hospital in pediatrics and nursery, acquired immunodeficiency secondary to severe malnutrition, prolonged steroid therapy, nephrotic syndrome, aplastic anemia, leishmaniasis, leukemias and other malignancies is more common as compared to inherited causes.