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

Abstract

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.

Idiopathic pyostomatitis-pyodermatitis vegetans with nasal obstruction: A case report

“… 1 Tumor necrosis factor (TNF)-alpha blocking biologics have been useful in some refractory cases. 3 As seen in this case, patients may require long-term suppressive therapy and steroid-sparing agents should be preferred. 2 , 4 , 5 Perhaps agents targeting eosinophils such as anti-interleukin (IL)-4, IL-5, and IL-13 blocking agents may also be useful given the predominant eosinophilic infiltrates and peripheral eosinophilia present in most cases.…”

“…4 While not all patients have underlying systemic disease, PSV-PDV is almost always reported in the setting of IBD and most patients have gastrointestinal symptoms prior to its onset. 3,5 We report a rare case of PSV-PDV in an otherwise healthy male without gastrointestinal or other involvement.…”

“… 1 , 2 Although the term PSV-PDV is suggestive of mucosal and/or skin infection, the exact etiology is unknown and no pathogenic agent has been consistently implicated in the disease. 3 PSV-PDV is likely a consequence of immunologic dysfunction given its strong association with immune dysregulation, most notably inflammatory bowel disease (IBD). There may also be associations with chronic liver dysfunction (primary sclerosing cholangitis, chronic hepatitis, cirrhosis) and HIV.…”