Pyoderma Gangrenosum: A Mini-Review

Abstract

Pyoderma gangrenosum (PG) is a rare, chronic neutrophilic dermatosis of unknown aetiology that usually presents with necrotising ulcers, although the evolution of the disease can be variable and is not always progressive. Its pathogenesis is poorly understood but an underlying immunological abnormality seems to be implicated in the genesis of the lesions. This hypothesis is supported by its frequent association with inflammatory bowel disease, malignancies, and rheumatological disorders. The diagnosis is challenging even for dermatologists as there are no specific tests or histological features. There are no clinical trials evaluating the efficacy of the different drugs used to treat the disease due to its rarity, and therefore there is no ’gold standard’ therapy. In this mini-review we describe the main clinical aspects of PG, its pathophysiology, association with underlying diseases, diagnosis, treatment options, and prognosis.

Tyrosine kinase inhibitors-associated pyoderma gangrenosum, a systematic review of published case reports

“…Generally, ulcerative and bullous subtypes have a worse outcome. Rapid response to treatment suggests a good prognosis [1].…”

“…Pyoderma gangrenosum is a rare, chronic, recurrent, noninfectious, difficult-to-treat ulcerative dermatosis. It is characterized by neutrophil dysfunction and is now classified within the neutrophilic disease spectrum [1]. Contrary to its name, pyoderma gangrenosum is neither infectious nor cause gangrene.…”

“…More than half of the cases of pyoderma gangrenosum are associated with the underlying inflammatory condition, including inflammatory bowel disease, rheumatoid arthritis, myelodysplastic syndrome, or hematologic malignancies [1].…”

Treatment of Pyoderma Gangrenosum in Pediatric Inflammatory Bowel Disease

“…The worldwide incidence of PG is estimated to be 3-10 cases per 1,000,000 population per year 11 and 4% are in children. 12 In adults, approximately 60% of reported PG cases occur in association with a systemic disorder, including rheumatoid arthritis, autoimmune hepatitis, and malignancy. Up to 50% are associated with IBD [11][12][13] ; however, <5% of patients with IBD develop PG.…”

“…12 In adults, approximately 60% of reported PG cases occur in association with a systemic disorder, including rheumatoid arthritis, autoimmune hepatitis, and malignancy. Up to 50% are associated with IBD [11][12][13] ; however, <5% of patients with IBD develop PG. 11,13 In IBD patients, PG is diagnosed most frequently in association with UC, but it is also reported in CD.…”