TY - CHAP M1 - Book, Section TI - Autoinflammatory Disorders A1 - Satoh, Takashi K. A1 - French, Lars E. A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. PY - 2019 T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCEAutoinflammatory disorders are caused by dysregulation of the innate immune system.Although many of the symptoms of autoinflammatory disorders, such as fever, skin lesions, and joint pain, mimic infection, the inflammation is sterile.Interleukin (IL)-1 plays a prominent role in a large subset of monogenic autoinflammatory disorders.Monogenic autoinflammatory disorders typically present in childhood but sometimes occur neonatally.Monogenic autoinflammatory disorders are mostly inherited in an autosomal-recessive or autosomal-dominant pattern, but there are a considerable number of sporadic cases.Characteristic skin eruptions are associated with a number of these disorders, including neutrophilic urticarial skin lesions, pustulosis, cellulitis-like skin lesions, erythematous and maculopapular eruptions. Skin lesions can be the sole remarkable disease manifestation in some disorders.Specific treatments exist, including IL-1 antagonists for cryopyrin-associated periodic syndromes (CAPS), Schnitzler syndrome, and deficiency of the interleukin (IL)-1 receptor antagonist (DIRA), and colchicine for familial Mediterranean fever (FMF). IL-1 antagonists have also been used with variable efficacy in FMF and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS)/mevalonate kinase deficiency (MKD).An online database for autoinflammatory mutations is available at Infevers (http://fmf.igh.cnrs.fr/ISSAID/infevers/). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - dermatology.mhmedical.com/content.aspx?aid=1162813768 ER -