Pathogenesis of Generalized Pustular Psoriasis-From the Viewpoint of Autoinflammatory Disease

Abstract

Autoinflammatory diseases are a category of illnesses characterized by episodes of inflammation, without high-titer autoantibodies or antigen-specific T cells, in which, as in the autoimmune diseases. Although autoimmunity involves adaptive immune activation, autoinflammation involves innate immune activation. Newly discovered evidences established that innate immunity and autoinflammation play significant roles in pathophysiology of psoriasis. Generalized pustular psoriasis is an uncommon variant of psoriasis, which presents as an acute or subacute, widely distributed eruption of pustules. Although the pathogenesis of generalized pustular psoriasis is not fully understood, there has been increasing knowledge of the genetic background of generalized pustular psoriasis. Currently, two genes, IL36RN and CARD14 are thought to be the causative or susceptibility genes in generalized pustular psoriasis. Recent genetic, clinical, and histopathological insights into generalized pustular psoriasis have revealed autoinflammatory pathways and highlight the potential for new targeted interventions of this condition.