title: What do autoinflammatory syndromes teach about common cutaneous diseases such as pyoderma gangrenosum? A commentary.
abstract: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerating skin lesions with rapid onset and recalcitrant treatment course. PG treatment targets an array of inflammatory pathways with variable success. One of the hallmark features of PG is its association with a broad spectrum of systemic disorders. The authors hypothesize that there are common inflammatory pathways linking these systemic disorders to neutrophilic dermatoses. Rare autoinflammatory diseases offer insights into the understanding of inflammatory skin conditions. This article explores observations of the natural history of PG that illuminate aspects of PG pathogenesis, highlighting the role of autoinflammatory mediators.
authors.affiliation: University of Arizona School of Medicine, 1510 N Campbell Avenue, PO Box 245017, Tucson, AZ 85724, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Butler
authors.forename: Kanade
authors.initials: K
authors.lastname: Shinkai
mesh_terms: D001327:Autoimmune Diseases / Q000188:drug therapy / Q000473:pathology*; D003937:Diagnosis, Differential; D056660:Hereditary Autoinflammatory Diseases / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009503:Neutropenia / Q000188:drug therapy / Q000473:pathology; D017511:Pyoderma Gangrenosum / Q000188:drug therapy / Q000473:pathology*; D013577:Syndrome
chemical_list: D007166:Immunosuppressive Agents
keywords: Autoinflammation; Cryopyrin-associated periodic syndrome; Deficiency of interleukin 1 receptor agonist; Familial Mediterranean fever; Neutrophilic dermatoses; Pyoderma gangrenosum; Pyogenic arthritis syndrome