Autoinflammatory Disorders: Under-Discussed Pathologies in Dermatology

Autoinflammatory diseases are the rare disorders characterized by recurrent/persistent systemic inflammation in the form of recurrent fever, mucocutaneous lesions, serositis, osteoarticular, and various other symptoms in the absence of infectious, neoplastic, or autoimmune triggers [1]. Though these disorders are known for quite a long time, their detailed understanding has been of recent interest. These unprovoked episodes of systemic inflammation are caused by the defective innate immune response unlike autoimmune disorders where acquired immunity is the culprit. Majority of these disorders are monogenic, seen predominantly during childhood, and caused by mutation/polymorphism in genes of innate immune response. There is excessive production of acute phase reactants and inflammatory cytokines like IL-1 β, IL-6 in autoinflammatory disorders [2].