{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T16:38:23Z","timestamp":1789749503122,"version":"4.0.1"},"reference-count":20,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,10,28]],"date-time":"2014-10-28T00:00:00Z","timestamp":1414454400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2015,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats: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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>We describe a patient with <jats:styled-content style=\"fixed-case\">PASH<\/jats:styled-content> syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for <jats:styled-content style=\"fixed-case\">PSTPIP<\/jats:styled-content>1 mutations associated with <jats:styled-content style=\"fixed-case\">PAPA<\/jats:styled-content> syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A 22\u2010year\u2010old 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 <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20101 receptor antagonist (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20101<jats:styled-content style=\"fixed-case\">RA<\/jats:styled-content>) 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 <jats:styled-content style=\"fixed-case\">PASH<\/jats:styled-content> syndrome. We review the literature about this group of diseases and report the third case of <jats:styled-content style=\"fixed-case\">PASH<\/jats:styled-content> syndrome to date.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">PASH<\/jats:styled-content> syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about <jats:styled-content style=\"fixed-case\">PASH<\/jats:styled-content> syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of <jats:styled-content style=\"fixed-case\">TNF<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20101.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.12765","type":"journal-article","created":{"date-parts":[[2014,10,29]],"date-time":"2014-10-29T02:15:55Z","timestamp":1414548955000},"page":"2243-2247","source":"Crossref","is-referenced-by-count":67,"title":["Successful treatment of <scp>PASH<\/scp> syndrome with infliximab, cyclosporine and dapsone"],"prefix":"10.1111","volume":"29","author":[{"given":"J.","family":"Staub","sequence":"first","affiliation":[{"name":"Department of Dermatology University Medical Center Mannheim Medical Faculty Mannheim University of Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Pfannschmidt","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Federal Academic Teaching Hospital Feldkirch Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Strohal","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Federal Academic Teaching Hospital Feldkirch Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Braun\u2010Falco","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy Ludwig\u2010Maximilian University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Lohse","sequence":"additional","affiliation":[{"name":"Institute for Laboratory Medicine and Human Genetics Singen 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Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,10,28]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2010.12.025"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.8.961"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2005.22320.x"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/RHU.0b013e318278b84c"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2013.2907"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34332"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/2013.jamadermatol.717"},{"key":"e_1_2_4_9_1","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1684\/ejd.2013.2018","article-title":"Pyoderma gangrenosum and concomitant hidradenitis suppurativa \u2010 rapid response to canakinumab 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