{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T19:22:42Z","timestamp":1789932162916,"version":"4.0.1"},"reference-count":39,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2017,3,27]],"date-time":"2017-03-27T00:00:00Z","timestamp":1490572800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Dermatology"],"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Autoinflammatory syndromes associated with hidradenitis suppurativa (\n                    <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>\n                    ) and\/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (\n                    <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\n                    )\u20101 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with\n                    <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>\n                    , acne, and pyoderma gangrenosum (\n                    <jats:styled-content style=\"fixed-case\">PG<\/jats:styled-content>\n                    ) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence\u2010based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.\n                  <\/jats:p>","DOI":"10.1111\/ijd.13603","type":"journal-article","created":{"date-parts":[[2017,3,27]],"date-time":"2017-03-27T02:43:14Z","timestamp":1490582594000},"page":"811-818","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":77,"title":["Autoinflammatory syndromes associated with hidradenitis suppurativa and\/or acne"],"prefix":"10.1111","volume":"56","author":[{"given":"Caroline","family":"Vinkel","sequence":"first","affiliation":[{"name":"Department of Dermatology Bispebjerg Hospital Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon F.","family":"Thomsen","sequence":"additional","affiliation":[{"name":"Department of Dermatology Bispebjerg Hospital Copenhagen Denmark"},{"name":"Department of Biomedical Sciences University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,3,27]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13634"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/QCO.0b013e3283428d07"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.2174\/138920210793175921"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2010.12.025"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1159\/000443648"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2013.2907"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.10.002"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12585"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.03.002"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2015.07.040"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00384-014-2110-9"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2015.12.037"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000000187"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11154-016-9328-5"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.12966"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4632.2004.02536.x"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2015.08.002"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13090"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.14038"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.12765"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-157-12-201212180-00004"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2011.10339.x"},{"key":"e_1_2_8_24_1","article-title":"Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation","author":"Marzano AV","journal-title":"Br J Dermatol"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.12631"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2009.09404.x"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keh479"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34332"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms17050725"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1001\/2013.jamadermatol.717"},{"key":"e_1_2_8_31_1","article-title":"PSTPIP1 Gene mutation in a pyoderma gangrenosum, acne and suppurative hidradenitis (PASH) Syndrome","author":"Calder\u00f3n\u2010Castrat X","year":"2015","journal-title":"Br J Dermatol"},{"key":"e_1_2_8_32_1","article-title":"Pyoderma gangrenosum, acne, suppurative hidradenitis (PASH) and polycystic ovary syndrome: coincidentally or aetiologically connected?","author":"Zivanovic D","year":"2016","journal-title":"Australas J Dermatol"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2010.328"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1159\/000343602"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13668"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1097\/RHU.0b013e318278b84c"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/194864"},{"key":"e_1_2_8_38_1","unstructured":"ISSAID \u2013 The International Society of Systemic Auto\u2010Inflammatory Diseases[Internet]. 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