The autoinflammatory syndromes represent a newly classified heterogenous subset of familiar or sporadic diseases, that are characterized by seemingly unprovoked episodes of inflammation due to excessive interleukin-1β activation. They manifest mainly in childhood with skin lesions, recurrent attacks of fever and joint symptoms.
Theis immunopathonegic mechanisms are gradually being revealed due to increasing level of knowledge and it appears that even common dermatoses might have present autoinflammatory signs. Recognition of the pathogenetic role of inflammasomes and kex mediators of innate immune system opens the possibility for new targeted therapies.