title: Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment.
abstract: Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic disorder with prototypical clinical presentations. Its pathophysiology is complex and not fully explained. Recent information regarding the genetic basis of PG and the role of auto-inflammation provides a better understanding of the disease and new therapeutic targets. PG equally affects patients of both sexes and of any age. Uncontrolled cutaneous neutrophilic inflammation is the cornerstone in a genetically predisposed individual. Multimodality management is often required to reduce inflammation, optimize wound healing, and treat underlying disease. A gold standard for the management of PG does not exist and high-level evidence is limited. Multiple factors must be taken into account when deciding on the optimum treatment for individual patients: location, number and size of lesion/ulceration(s), extracutaneous involvement, presence of associated disease, cost, and side effects of treatment, as well as patient comorbidities and preferences. Refractory and rapidly progressive cases require early initiation of systemic therapy. Newer targeted therapies represent a promising pathway for the management of PG, and the main focus of this review is the management and evidence supporting the role of new targeted therapies in PG.
authors.affiliation: Division of Dermatology, Department of Medicine, Women's College Hospital, University of Toronto, 76 Grenville St, 5th Floor, Toronto, ON, M5S 1B2, Canada. afsaneh.alavi@utoronto.ca.
authors.forename: Afsaneh
authors.initials: A
authors.lastname: Alavi
authors.affiliation: Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Lars E
authors.initials: LE
authors.lastname: French
authors.affiliation: Department of Dermatology, Mayo Clinic, MN, Rochester, USA.
authors.forename: Mark D
authors.initials: MD
authors.lastname: Davis
authors.affiliation: Department of Dermatology, University of Alberta, Edmonton, AB, Canada.
authors.forename: Alain
authors.initials: A
authors.lastname: Brassard
authors.affiliation: Department of Dermatology, University of Miami Miller School of Medicine, Miami, FL, USA.
authors.forename: Robert S
authors.initials: RS
authors.lastname: Kirsner
mesh_terms: D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D058990:Molecular Targeted Therapy; D009504:Neutrophils / Q000378:metabolism; D017511:Pyoderma Gangrenosum / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D014945:Wound Healing