Genetic Immunodeficiency Disorders

Immunodeficiency disorders occur when the body's immune response is reduced or absent due to failure or absence of elements of the immune system, including lymphocytes, phagocytes, and complement system. Immunodeficiencies can be primary or secondary. Primary immunodeficiency genodermatoses is subdivided mainly into types that cause T-cell or/and B-cell deficiency or complement deficiency. The most important example for primary T-cell deficiency is chronic mucocutaneous candidiasis with recurrent or persistent mucocutaneous candidal infection; however, Wiskott-Aldrich syndrome represents primary immunodeficiency of both T- and B-cells and characterized by classic triad of immunodeficiency, thrombocytopenia and eczema. Moreover, hyper-IgE Syndrome is characterized by recurrence of skin abscesses, lung infections and eczema with eosinophilia and high serum levels of IgE.