title: Autoinflammatory pustular neutrophilic diseases.
abstract: This article provides a new categorization of inflammatory pustular dermatoses in the context of recent genetic and biological insights. Monogenic diseases with pustular phenotypes are discussed, including deficiency of interleukin 1 receptor antagonist, deficiency of the interleukin 36 receptor antagonist, CARD14-associated pustular psoriasis, and pyogenic arthritis, pyoderma gangrenosum, and acne. How these new genetic advancements may inform how previously described pustular diseases are viewed, including pustular psoriasis and its clinical variants, with a focus on historical classification by clinical phenotype, is also discussed.
authors.affiliation: Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 12N238, 10 Center Drive, MSC 1908, Bethesda, MD 20892-1908, USA.
authors.forename: Haley B
authors.initials: HB
authors.lastname: Naik
authors.forename: Edward W
authors.initials: EW
authors.lastname: Cowen
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D003937:Diagnosis, Differential; D006801:Humans; D009504:Neutrophils / Q000276:immunology*; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology
keywords: Deficiency of IL-1 receptor antagonist (DIRA); PAPA syndrome; Palmoplantar pustulosis; Pustular psoriasis; SAPHO syndrome; Subcorneal pustular dermatosis