Autoinflammatory syndromes: A review

Abstract

Autoinflammatory syndromes (AIS) are disorders of innate immunity which present with recurrent episodes of fever and skin lesions, such as urticaria, pustules, maculopapular rash, oral ulcers, generalized pustular psoriasis, or pyoderma gangrenosum-like lesions. The different entities that come under AIS are familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, and cryopyrin-associated periodic syndromes. Many new entities are also described. As many of them present with skin lesions, dermatologists should be aware of myriad of clinical features associated with these disorders. Childhood onset, positive family history, and elevated laboratory markers of systemic inflammation during acute episodes are the clues to diagnosis. Infections, connective tissue diseases, and malignancies should be excluded before diagnosing AIS.