Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. (28th October 2014)

• Record Type: Journal Article
• Authors: Staub, J.; Pfannschmidt, N.; Strohal, R.; Braun‐Falco, M.; Lohse, P.; Goerdt, S.; Leverkus, M.
• Is Part Of: Journal of the European Academy of Dermatology and Venereology. Volume 29:Number 11(2015:Nov.)
• Page Start: 2243
• Page End: 2247
• Publication Date: 2014-10-28
• Subjects: Dermatology -- Periodicals; Sexually transmitted diseases -- Periodicals; 616.5
• DOI: 10.1111/jdv.12765
• Languages: English
• ISSNs: 0926-9959

Abstract

<abstract abstract-type="main" id="jdv12765-abs-0001"> <title>Abstract</title> <sec id="jdv12765-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="jdv12765-sec-0002" sec-type="section"> <title>Objectives</title> <p>We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone.</p> </sec> <sec id="jdv12765-sec-0003" sec-type="section"> <title>Methods</title> <p>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.</p> </sec> <sec id="jdv12765-sec-0004" sec-type="section"> <title>Results</title> <p>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.</p> </sec> <sec id="jdv12765-sec-0005" sec-type="section"> <title>Conclusion</title> <p>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.</p> </sec> </abstract>