The term “autoinflammatory illnesses” refers to a group of conditions defined by uncontrolled activation of the innate immune system. Whereas adaptive immunity abnormalities have long been identified in ‘autoimmune’ disorders, the concept of ‘autoinflammation’ emerged relatively recently, first describing a group of clinical disorders characterized by spontaneous episodes of systemic inflammation without the manifestations typical of autoimmune disorders. 

Improved understanding of innate immune systems, along with amazing advances in genomics and a growing number of clinical cases, has resulted in an increase in the number of illnesses categorized as autoinflammatory or including an autoinflammatory component. Biologic medicines that target particular components of the innate immune system have produced significant therapeutic benefits and shed light on the function of innate immunity in autoinflammatory diseases.

This article discusses the fundamental processes of autoinflammation, followed by an update on the pathophysiology and management of monogenic and multifactorial autoinflammatory disorders, as well as prevalent dermatologic ailments in which autoinflammation plays a significant role.