Pyoderma gangraenosum als diagnostische und therapeutische interdisziplinäre Herausforderung

Zusammenfassung

Das Pyoderma gangraenosum (PG) ist eine neutrophile Dermatose unklarer Genese, die sowohl in Assoziation zu hämatologischen und neoplastischen Systemerkrankungen, chronisch-entzündlichen Darmerkrankungen und autoinflammatorischen Syndromen als auch idiopathisch auftreten kann. Sowohl die Diagnosestellung wie auch die Therapie des PG stellen aufgrund seiner Seltenheit, des Fehlens großer randomisierter kontrollierter Studien und der unzureichend verstandenen Pathogenese eine Herausforderung in der klinischen Praxis dar. Diese Übersichtsarbeit beschreibt und diskutiert aktuelle Erkenntnisse, die das PG als autoinflammatorische Erkrankung beschreiben. Durch eine Dysregulation von T-Lymphozyten und myeloiden Zellen wie den neutrophilen Granulozyten kommt es zur Entstehung von Pusteln und großflächigen Ulzera. Klassische Therapieansätze umfassen eine anti-inflammatorische topische Therapie, eine Analgesie sowie die systemische Gabe von Immunmodulantien oder -suppressiva. Neuere, bisher nicht zugelassene Therapieoptionen sind der Einsatz von Biologika und JAK-Inhibitoren.

Abstract

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown aetiology, which occurs idiopathically or in association with inflammatory bowel disease, haemato-oncological diseases and autoinflammatory syndromes. Both diagnosis and treatment of PG are challenging in clinical practice due to its rarity, lack of large randomised controlled trials and its poorly understood pathogenesis. This review discusses novel evidence regarding the pathophysiology of PG as an autoinflammatory disease and its treatment. Activation of the inflammasome, dysregulation of myeloid cells such as neutrophil granulocytes as well as T cells lead to the development of painful ulcerations. Traditional therapeutic approaches include anti-inflammatory topical therapy, analgesia and immunomodulatory or immunosuppressive drugs. Novel therapeutic options include biologics and JAK inhibitors, which are not yet approved.

Schlüsselwörter

Pyoderma gangraenosum - neutrophile Dermatose - chronische Wunde - Biologicals - Small-Molecular-Weight Inhibitoren

Keywords

chronic ulceration - pyoderma gangrenosum - neutrophilic dermatosis - biologicals - small-molecular-weight inhibitors

Publication History

Received: 14 February 2022

Accepted after revision: 15 March 2022

Article published online:
20 July 2023

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