title: [Autoinflammatory diseases as cause of wound healing defects].
abstract: Ulcerations of the skin and mucosal membranes are a common feature of autoinflammatory diseases. They can give raise to chronic wound healing defects and should be considered in the differential diagnosis of chronic skin ulcers. The increased activation of the innate immune system in the absence of an apparent provocation for inflammation is a hallmark of autoinflammatory diseases. Mutations and alterations of signaling pathways regulating the innate immune response to physical trauma/tissue damage result into an unrestrained activation of the inflammasome, which leads to increased activation of Interleukin-1. Uncontrolled recruitment and activation of myeloid effector cells within the wound site lead to the release of potent proteases that cause the degradation of structural components of the skin. The majority of these diseases respond well to immunosuppressive and immunomodulatory treatment regimes. Therapeutic resistance converts the acute inflammatory response into a chronic and non-resolving inflammatory process that leads to tissue degeneration. In this article we will focus on the review of those autoinflammatory diseases that often display ulcerative cutaneous and aphthous lesions including pyoderma gangrenosum, Behçet disease, PAPA syndrome and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Furthermore, the article will be complemented by an overview of those inflammatory diseases that are associated with non-ulcerative cutaneous manifestations.
authors.affiliation: Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Kerpenerstr. 62, 50937, Köln, Deutschland.
authors.forename: R
authors.initials: R
authors.lastname: Löhrer
authors.forename: R
authors.initials: R
authors.lastname: Eming
authors.forename: N
authors.initials: N
authors.lastname: Wolfrum
authors.forename: T
authors.initials: T
authors.lastname: Krieg
authors.forename: S A
authors.initials: SA
authors.lastname: Eming
mesh_terms: D000152:Acne Vulgaris / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D001170:Arthritis, Infectious / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D058847:Inflammasomes / Q000097:blood; D007375:Interleukin-1 / Q000097:blood; D054078:Mevalonate Kinase Deficiency / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D017511:Pyoderma Gangrenosum / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D012883:Skin Ulcer / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D014945:Wound Healing / Q000187:drug effects / Q000276:immunology*
chemical_list: D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D058847:Inflammasomes; D007375:Interleukin-1