Zusammenfassung
Ulzerationen der Haut und Schleimhäute sind ein häufiges Symptom autoinflammatorischer Erkrankungen. Nicht selten führen diese zu Wundheilungsstörungen und sollten in der Differenzialdiagnose chronischer Wunden berücksichtigt werden. Durch die pathologische Einwanderung und Aktivierung von Entzündungszellen in die Haut kommt es im Rahmen autoinflammatorischer Erkrankungen zur Freisetzung potenter Proteasen, die zu einer unkontrollierten Degradation von Strukturproteinen führen. Eine immunsuppressive/immunmodulierende Therapie führt in der Regel zu einer raschen Abheilung der Gewebedefekte. Bei verzögerter Therapie mit Persistenz des entzündlichen Infiltrates geht die akute Entzündungsreaktion in einen chronischen Prozess über, eine Entwicklung, welche die Aussicht auf Abheilung der Ulzerationen deutlich verschlechtern kann. In diesem Beitrag werden die autoinflammatorischen Erkrankungen dargestellt, die häufig zu Gewebedefekten und Wundheilungsstörungen führen. Zu diesen gehören zum einen Krankheitsbilder, die den autoinflammatorischen Erkrankungen im weiteren Sinne zugerechnet werden wie das Pyoderma gangraenosum und der M. Behçet, und zum anderen klassische autoinflammatorische Erkrankungen, wie z. B. das PAPA-Syndrom und das Hyperimmunglobulin-D-Syndrom. Die Darstellung dieser Erkrankungen wird durch eine Übersicht weiterer Krankheitsbilder aus dem Formenkreis der autoinflammatorischen Erkrankungen ergänzt, die zwar zu Hautveränderungen führen können, aber nicht durch Ulzeration charakterisiert sind.
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.
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Löhrer, R., Eming, R., Wolfrum, N. et al. Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen. Hautarzt 62, 524–533 (2011). https://doi.org/10.1007/s00105-010-2115-4
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DOI: https://doi.org/10.1007/s00105-010-2115-4