Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone

• Verfasst von: Staub, Janina [VerfasserIn]
• Verfasst von: Goerdt, Sergij [VerfasserIn]
• Verfasst von: Leverkus, Martin [VerfasserIn]
• Titel: Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone
• Verf.angabe: J. Staub, N. Pfannschmidt, R. Strohal, M. Braun-Falco, P. Lohse, S. Goerdt, M. Leverkus
• E-Jahr: 2015
• Jahr: 28 October 2014
• Jahr des Originals: 2014
• Umfang: 5 S.
• Fussnoten: Gesehen am 10.01.2018
• Titel Quelle: Enthalten in: European Academy of Dermatology and VenereologyJournal of the European Academy of Dermatology and Venereology
• Ort Quelle: Oxford [u.a.] : Wiley-Blackwell, 1991
• Jahr Quelle: 2015
• Band/Heft Quelle: 29(2015), 11, Seite 2243-2247
• ISSN Quelle: 1468-3083
• DOI: doi:10.1111/jdv.12765
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1567020275

Abstract

Background The group of autoinflammatory syndromes associated with Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis are poorly defined and difficult to control with currently available treatment modalities. Objectives We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone. Methods A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for PSTPIP1 mutations associated with PAPA syndrome. Results A 22-year-old woman presented to our department with pyoderma gangrenosum, concomitant acne, and suppurative hidradenitis. She had previously been treated unsuccessfully with etanercept, adalimumab, fumaric acid and the IL-1 receptor antagonist (IL-1RA) anakinra without prolonged remission. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms that we classified as PASH syndrome. We review the literature about this group of diseases and report the third case of PASH syndrome to date. Conclusion PASH syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about PASH syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of TNF or IL-1.