Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management

Abstract

Pyoderma gangrenosum (PG) is a rare entity that is characterized by infiltration of neutrophils into the dermis, causing the formation of rapidly enlarging, painful and necrotic skin ulcers. The pathophysiology of PG is still poorly understood. However, genetic, autoimmune and autoinflammatory mechanisms have been proposed that could potentially explain the etiology of this ulcerating skin disorder. Early diagnosis and treatment are key, as the disease course is rapidly progressive and can leave disfiguring, cribriform scars. However, the diagnosis of PG proves difficult, firstly because there are multiple variants of the disease and secondly because it is a clinical diagnosis and can appear similar to that of other diseases such as vasculitis, skin/soft tissue infections and malignancy. Additionally, there are no official diagnostic criteria to aid in the recognition of PG, which often leads to significant delays in diagnosis. The treatment of PG consists in immunosuppression. However, due to a lack of standardized guidelines, therapeutic regimens are usually dependent upon the individual clinician’s experience and are based on little evidence. Knowledge of the clinical features and pathophysiology of PG can aid in early diagnosis and targeted treatment strategies, which in turn results in improved patient outcomes.

Pyoderma Gangrenosum of the Breast: A Rare Location of Neutrophilic Dermatoses

“…It has been well documented in the literature that pyoderma gangnosum is usually associated to many different diseases such as inflammatory bowel disease, autoimmune diseases, and hematologic malignancies [1,4,7,8] but in our case we did not find any other diseases associated to PG.…”

“…, the international Delphi Criteria and PARACELSUS score in 2018, all had major and minor criteria but in 2021 a cross-sectional retrospective cohort study found that the PARACELSUS score was the most accurate and precise tool to use in the diagnosis of PG [7].…”

Pyoderma gangrenosum following vaccination against coronavirus disease‐2019: a case report

“…PG is often associated with systemic diseases, such as rheumatoid arthritis, inflammatory bowel disease, and other autoimmune and inflammatory diseases 2 . While the exact pathophysiologic mechanisms of PG remain unclear, autoimmune processes driven by abnormalities in the innate immune system are implicated 2 . Unlike conventional PG, vaccination‐associated PG presents without associated systemic diseases 3 .…”

“… 2 While the exact pathophysiologic mechanisms of PG remain unclear, autoimmune processes driven by abnormalities in the innate immune system are implicated. 2 Unlike conventional PG, vaccination‐associated PG presents without associated systemic diseases. 3 In our case, it is highly likely that COVID‐19 vaccination could have contributed to the development of PG due to their close temporal relationship.…”

An aggressive course of pyoderma gangrenosum mimicking bacterial osteomyelitis after open reduction and internal fixation of a distal radius fracture with a titanium plate

“…Reumatologia 2022; 60/4 cular occlusive or venous disease, skin cancer, infection, or drug reaction, are excluded. There are 3 systems of criteria -Mayo, Delphi Consensus, and PARACELSUS-score, among which the latter appears to be the most valuable [10].…”