Different types of hereditary immune deficiencies are at risk of severe fungal infections. Such infections can reveal the deficit. The type of micro-organisms involved, the clinical presentation, the immune reaction observed around the germ can then guide the etiological diagnosis. As often in the field of immunodeficiencies, advances in the pathophysiology provide crucial informations on the natural defense mechanisms against these organisms. Therapeutic advances have dramatically improved the prognosis of these potentially serious and disabling infections.
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