Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen

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.

Vaginal extrusion of a ventriculo-peritoneal shunt catheter in an adult

“…Behcet's syndrome is an immune-mediated small-vessel vasculitis that disrupts mucosal membranes as well as ocular, gastrointestinal, pulmonary, and genitourinary dysfunction. [ 10 ] Wound healing is known to be generally impaired in this patient population. While it is unknown what caused the vaginal perforation in this particular case, a history of prior hysterectomy and chronic mucosal dysfunction may have put this patient at higher risk.…”