The Skin as a Window into Primary Immune Deficiency Diseases: Atopic Dermatitis and Chronic Mucocutaneous Candidiasis.

Abstract

Primary immune deficiency diseases characteristically present with recurrent, severe, or unusual infections. These infections may often involve the skin, with mucocutaneous candidal infections seen in a variety of different primary immune deficiencies. Primary immune deficiencies may also present with noninfectious cutaneous complications, of which eczema is the most common. In a patient with suspected primary immune deficiency, the presence of eczema or candidal skin infections offers critical information about the underlying immune defect, either the presence of atopy or defect in the T_H17 pathway, respectively. These skin manifestations also are often early or heralding findings of the underlying immunologic disease. Therefore, awareness of associations between these skin findings and specific immune deficiencies may aide in the early detection and treatment of serious or life-threatening immunologic defects. This review specifically will focus on the primary immune deficiencies commonly associated with eczema or mucocutaneous candidiasis.