Titre
Pyoderma gangrenosum.
Type
synthèse (review)
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Maverakis, E.
Auteure/Auteur
Marzano, A.V.
Auteure/Auteur
Le, S.T.
Auteure/Auteur
Callen, J.P.
Auteure/Auteur
Brüggen, M.C.
Auteure/Auteur
Guenova, E.
Auteure/Auteur
Dissemond, J.
Auteure/Auteur
Shinkai, K.
Auteure/Auteur
Langan, S.M.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
2056-676X
Statut éditorial
Publié
Date de publication
2020-10-08
Volume
6
Numéro
1
Première page
81
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Review ; Research Support, Non-U.S. Gov't
Publication Status: epublish
Publication Status: epublish
Résumé
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that presents with rapidly developing, painful skin ulcers hallmarked by undermined borders and peripheral erythema. Epidemiological studies indicate that the average age of PG onset is in the mid-40s, with an incidence of a few cases per million person-years. PG is often associated with a variety of other immune-mediated diseases, most commonly inflammatory bowel disease and rheumatoid arthritis. The cause of PG is not well understood, but PG is generally considered an autoinflammatory disorder. Studies have focused on the role of T cells, especially at the wound margin; these cells may support the destructive autoinflammatory response by the innate immune system. PG is difficult to diagnose as several differential diagnoses are possible; in addition to clinical examination, laboratory tests of biopsied wound tissue are required for an accurate diagnosis, and new validated diagnostic criteria will facilitate the process. Treatment of PG typically starts with fast-acting immunosuppressive drugs (corticosteroids and/or cyclosporine) to reduce inflammation followed by the addition of more slowly acting immunosuppressive drugs with superior adverse event profiles, including biologics (in particular, anti-tumour necrosis factor (TNF) agents). Appropriate wound care is also essential. Future research should focus on PG-specific outcome measures and PG quality-of-life studies.
PID Serval
serval:BIB_E49FEE4ADF26
PMID
Date de création
2020-10-26T06:31:10.208Z
Date de création dans IRIS
2025-05-21T05:11:50Z