Affiliation:
1. Dermatology Department, Unidade Local de Saúde Alto Minho, Viana do Castelo, Portugal
2. Dermatology Department, Centro Hospitalar Porto-EPE, Porto, Portugal
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.