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It may occur in rare syndromes: follicular occlusion, Bazex\u2013Dupr\u00e9\u2013Christol, Down's, <jats:styled-content style=\"fixed-case\">KID<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> PAPASH<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> PASS<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> PASH<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">SAPHO<\/jats:styled-content> syndromes, as well as Dowling\u2010Degos disease. An overview of syndromic <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content> may inform the search for aetiological factors in <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>. PubMed, Ovid and Web of Science were systematically searched using \u2018(hidradenitis <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> acne invers*) <jats:styled-content style=\"fixed-case\">AND<\/jats:styled-content> (syndrome <jats:styled-content style=\"fixed-case\">OR KID OR PASS OR PAPA OR PASH OR SAPHO OR<\/jats:styled-content> bazex\u2010dupre <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> \u2018dowling degos\u2019 <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> triad <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> tetrad)\u2019 and Cochrane Library using \u2018hidradenitis <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> acne invers*\u2019. A total of 82 articles were included in the final review. We summarize 134 cases collected from the 82 included articles. The syndromes are discussed, focusing on etiopathogenesis, clinical presentation and treatment. This study is based on case reports; therefore, conclusions may be subject to the selection bias. These syndromes are rare; however, it is important to recognize them, as treating them may require a different approach. Three subtypes of syndromic hidradenitis are suggested: syndromes with known genetic abnormalities, syndromes characterized by follicular plugging or structural defects, and syndromes with possible autoinflammatory pathogenesis. There was no universally effective treatment for syndromic <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>, and treatment was individualized.<\/jats:p>","DOI":"10.1111\/jdv.14464","type":"journal-article","created":{"date-parts":[[2017,7,11]],"date-time":"2017-07-11T09:39:41Z","timestamp":1499765981000},"page":"1809-1816","source":"Crossref","is-referenced-by-count":76,"title":["Recognizing syndromic hidradenitis suppurativa: a review of the literature"],"prefix":"10.1111","volume":"31","author":[{"given":"J.","family":"Gasparic","sequence":"first","affiliation":[{"name":"Department of Dermatology Zealand University Hospital Roskilde Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Theut Riis","sequence":"additional","affiliation":[{"name":"Department of Dermatology Zealand University Hospital Roskilde Denmark"},{"name":"Health Sciences Faculty University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.B.","family":"Jemec","sequence":"additional","affiliation":[{"name":"Department of Dermatology Zealand University Hospital Roskilde Denmark"},{"name":"Health Sciences Faculty University of Copenhagen Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,9,7]]},"reference":[{"key":"e_1_2_14_2_1","first-page":"481","volume-title":"Dermatology","author":"Pillsbury DM","year":"1956"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pmed.1000097"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-642-96246-2_11"},{"key":"e_1_2_14_5_1","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1001\/jama.198.1.201","article-title":"Hidradenitis suppurativa. 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