Leandro Costa, Faculdade de Medicina, Universidade de Coimbra, Coimbra, Portugal André Aparício-Martins, Department of Dermatology and Venereology, Hospital da Universidade de Coimbra, Unidade Local de Saúde de Coimbra. Coimbra, Portugal Margarida Gonçalo, Faculdade de Medicina, Universidade de Coimbra; Department of Dermatology and Venereology, Hospital da Universidade de Coimbra, Unidade Local de Saúde de Coimbra. Coimbra, Portugal
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis, often associated with systemic diseases, including inflammatory bowel disease, rheumatoid arthritis, and hematological disorders. Its etiopathogenesis is complex, including neutrophil dysfunction, immunological dysregulation, and genetic alterations. PG lesions can be single or multiple, with varying sizes and morphologies, affecting different anatomical areas. The most common clinical variant is the ulcerative/classic, but bullous, pustular, vegetative, peristomal, and post-surgical variants are also described. Indeed, the diagnosis of PG is challenging due to the heterogeneous clinical presentation and the absence of specific analytical and histopathological findings. Moreover, its lesions can mimic cutaneous ulcers of other etiologies. A wide array of complementary tests may be required not only to rule out alternative diagnoses but also to investigate underlying systemic diseases and extracutaneous manifestations. PG has a significant impact on the quality of life. Its treatment relies on a multimodal approach aimed to reduce disease activity, optimize wound healing, manage pain, and treat associated comorbidities. Topical and systemic immunomodulatory drugs, analgesics, and appropriate wound care are the available therapeutic options. Therefore, this review provides a narrative synthesis of the published literature, contributing to a better understanding of the disease and an optimized approach to these patients.
Keywords: Pyoderma gangrenosum. Neutrophilic dermatosis. Autoinflammation. Autoimmunity. Immunosuppression.