Autoinflammatory diseases were initially assigned to the hereditary recurrent fevers that were characterized by unprovoked episodes of inflammation without antigen-specific T cells or high titers of auto-antibodies, in contrast to the autoimmune diseases in which acquired immunity played an essential role. Except for Blau syndrome and early-onset sarcoidosis that are associated with granuloma due to NOD2 mutations and classified as NF-κB activation disorders, the major types of autoinflammatory diseases are defined as IL-1β activating disorders or inflammasomopathies. This is based on accumulating evidence for the efficacy of anti-IL-1 therapy. These diseases include intrinsic cryopyrin-associated periodic syndrome (CAPS), extrinsic familial Mediterranean fever, hyper IgD syndrome, pyogenic sterile arthritis pyoderma gangrenosum and acne syndrome, and deficiency of an IL-1 receptor antagonist. Knowledge obtained from these autoinflammatory disorders should also be pertinent to a number of common disorders. For example, neutrophil migration is observed in autoinflammatory CAPS and common inflammatory keratoses represented by psoriasis. Abnormal regulation of the innate immune response and Th17 cell differentiation via IL-1 signaling may be associated with the molecular pathogenesis of these conditions.