title: Are neutrophilic dermatoses autoinflammatory disorders?
abstract: Neutrophils constitute essential players in inflammatory responses and are the first line of defence against harmful stimuli. However, dysregulation of neutrophil homeostasis can result in excessive inflammation and subsequent tissue damage. Neutrophilic dermatoses are a spectrum of inflammatory disorders characterized by skin lesions resulting from a neutrophil-rich inflammatory infiltrate in the absence of infection. The exact molecular pathophysiology of neutrophilic dermatoses has long been poorly understood. Interestingly, neutrophil-rich cutaneous inflammation is also a cardinal feature of several autoinflammatory diseases with skin involvement, the latter being caused by aberrant innate immune responses. Overactivation of the innate immune system leading to increased production of interleukin-1 family members and 'sterile' neutrophil-rich cutaneous inflammation are features of both inherited autoinflammatory syndromes with skin involvement and an increasing number of neutrophilic dermatoses. Therefore, we propose that autoinflammation may be a cause of neutrophilic dermatoses.
authors.affiliation: Department of Dermatology, University Hospital Zurich, Gloriastraße 31, Zurich, 8091, Switzerland.
authors.forename: T K
authors.initials: TK
authors.lastname: Satoh
authors.affiliation: Department of Dermatology, University Hospital Zurich, Gloriastraße 31, Zurich, 8091, Switzerland.
authors.forename: M
authors.initials: M
authors.lastname: Mellett
authors.affiliation: Department of Dermatology, University Hospital Zurich, Gloriastraße 31, Zurich, 8091, Switzerland.
authors.forename: E
authors.initials: E
authors.lastname: Contassot
authors.affiliation: Department of Dermatology, University Hospital Zurich, Gloriastraße 31, Zurich, 8091, Switzerland.
authors.forename: L E
authors.initials: LE
authors.lastname: French
mesh_terms: D000152:Acne Vulgaris / Q000473:pathology; D001170:Arthritis, Infectious / Q000473:pathology; D001327:Autoimmune Diseases / Q000473:pathology; D001528:Behcet Syndrome / Q000473:pathology; D053468:CARD Signaling Adaptor Proteins / Q000502:physiology; D003872:Dermatitis / Q000473:pathology*; D006162:Guanylate Cyclase / Q000502:physiology; D017497:Hidradenitis Suppurativa / Q000473:pathology; D006801:Humans; D058847:Inflammasomes / Q000502:physiology; D007375:Interleukin-1 / Q000502:physiology; D008565:Membrane Proteins / Q000502:physiology; D009504:Neutrophils / Q000502:physiology*; D011565:Psoriasis / Q000473:pathology; D017511:Pyoderma Gangrenosum / Q000473:pathology; D016463:Sweet Syndrome / Q000473:pathology
chemical_list: D053468:CARD Signaling Adaptor Proteins; C508588:IL36B protein, human; D058847:Inflammasomes; D007375:Interleukin-1; D008565:Membrane Proteins; C426243:CARD14 protein, human; D006162:Guanylate Cyclase