Abstract
Autoinflammatory diseases have become an important differential diagnostic entity along with other diagnoses accompaniednot only by fever, such as infections, immunodeficiencies, idiopathic inflammatory conditions, and hemato-oncologic diseases.The term autoinflammatory characterizes the etiologic background of these diseases in which episodic activation of proinflammatorymechanisms is caused by impaired regulation of innate immunity processes. This review article describes the individualconditions with an emphasis on their skin manifestations.
The skin manifestations of several of the above-mentioned diseasescan be a significant clue in diagnosing them correctly.