CASE 25 Immune Dysregulation, Polyendocrinopathy, Enteropathy X-linked Disease

The primary role of the immune system is to recognize pathogens and eliminate them from the body. However, an equally important task is to distinguish potentially dangerous antigens from those that are harmless. Countless innocuous foreign antigens are encountered every day by the lungs, gut, and skin, the interfaces between the body and the environment. Similarly, the body contains numerous self antigens that might bind to the specific antigen receptors on B and T cells. Activation of the immune system by such innocuous antigens is unnecessary and may lead to unwanted inflammation. Allergic and autoimmune diseases are well-known examples of such unwanted and potentially destructive responses.