Pyoderma gangrenosum as an interdisciplinary diagnostic and therapeutic challenge

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.