Treatment of Pyodermatitis-Pyostomatitis Vegetans: A Systematic Review and Meta-analysis

Background: Pyodermatitis-pyostomatitis vegetans (PDV-PSV) is a rare muco-cutaneous disorder characterized by vegetating and pustular plaques and is often associated with inflammatory bowel disease (IBD). The purpose of this study was to systematically identify and analyze reports of PDV-PSV to determine the most effective treatment. Methods: Reports of PDV-PSV were identified using the OVID-Medline database from inception through November 2019. Publications were excluded if no new patient case was included, if there was not clinical and histological evidence of PDV, PSV, or PDV-PSV, or if no treatment was discussed. Results: The final sample was comprised of 74 publications plus an additional patient from the authors’ institution, corresponding to 95 total patients. The basis of the review and analysis is limited to case reports and case series, which likely only report the cases with positive outcomes. Statistical analysis revealed that oral corticosteroids (OCS), 6-mercaptopurine/azathioprine, oral calcineurin inhibitors (OCNI), 5-aminosalicylic-acid (5-ASA), and biologics (BIO) were the most effective treatments for PDV-PSV. Topical medications, colchicine, oral dapsone, and other antibiotics were ineffective treatments, with topical medications being the least effective option. When OCS are used, they work best when used as initial treatment to induce remission. 5-ASA and BIO are most effective when used as maintenance therapies after initial remission. Conclusions: Thus, first line therapy for PDV-PSV should begin with OCS with transition to steroid-sparing agents including OCNI, BIO, and 5-ASA if indicated.