{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T18:14:47Z","timestamp":1778696087301,"version":"3.51.4"},"reference-count":11,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T00:00:00Z","timestamp":1724716800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Med Surg"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:p>Xanthoma disseminatum (XD) is a rare normolipidemic mucocutaneous xanthomatosis within the spectrum of cutaneous non-Langerhans histiocytosis. Managing XD poses substantial challenges, with limited available data. This study aims to comprehensively evaluate existing literature on clinical features of XD and treatment outcomes. A systematic search of MEDLINE, Embase, and PubMed was performed, using \u201cxanthoma disseminatum\u201d and \u201cMontgomery syndrome\u201d as search terms, without restrictions. Screening was performed in duplicate by 2 reviewers. One hundred fifty-one studies met the inclusion criteria, yielding 166 cases of XD (106 females, 60 males), mean age at diagnosis 35.3 years (range: 9 months-87 years). XD typically presented as yellow-to-brown coalescing papules\/plaques and nodules. Distribution affects mainly the face (n = 116\/166), flexures (n = 45\/166), trunk (n = 65\/166), and genitalia\/inguinal areas (n = 63\/166). Most cases (99.4%; n = 165\/166) exhibited extracutaneous manifestations, including the pituitary gland and the oropharynx. Treatment options rendered low complete response rates (CRRs). Treatments with reported outcomes included surgical resection (n = 17\/99), systemic steroids (n = 40\/99), immunosuppressants\/immunomodulators (n = 73\/99), energy-based devices (n = 7\/99), lipid-lowering agents (n = 24\/99), cryotherapy (n = 6\/99), lasers (n = 10\/99), topical steroids (n = 6\/99), oral retinoids (n = 2\/99), and radiotherapy (n = 5\/99), with CCRs of 23.5% (n = 4\/17), 5.0% (n = 2\/40), 9.6% (n = 7\/73), 14.3% (n = 1\/7), 4.2% (n = 1\/24), 16.7% (n = 1\/6), 10.0% (n = 1\/10), 0% (n = 0\/6), 0% (n = 0\/2), and 0% (n = 0\/5), respectively. The most promising therapy is cladribine, with the highest CRR of 27.1% (n = 6\/22) and the lowest no response rate (9.1%; n = 2\/22) of all reported treatments. This review confirms the high prevalence of systemic manifestations in XD. Treatment options vary widely; thus, further research is needed to establish management strategies for this challenging condition.<\/jats:p>","DOI":"10.1177\/12034754241274356","type":"journal-article","created":{"date-parts":[[2024,8,28]],"date-time":"2024-08-28T01:39:51Z","timestamp":1724809191000},"page":"572-576","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":6,"title":["A Systematic Review of Clinical Features and Treatment Outcomes of Xanthoma Disseminatum"],"prefix":"10.1177","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-0273-0321","authenticated-orcid":false,"given":"Lorena Alexandra","family":"Mija","sequence":"first","affiliation":[{"name":"Department of Medicine, Universit\u00e9 de Montr\u00e9al, Montr\u00e9al, QC, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6253-847X","authenticated-orcid":false,"given":"Catherine Keying","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Medicine, McGill University, Montr\u00e9al, QC, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1058-9413","authenticated-orcid":false,"given":"Ilya","family":"Mukovozov","sequence":"additional","affiliation":[{"name":"Toronto Dermatology Centre, North York, ON, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2024,8,27]]},"reference":[{"key":"bibr1-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1016\/s0140-6736(17)31934-7"},{"key":"bibr2-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1159\/000246628"},{"key":"bibr3-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1962.01590110018003"},{"key":"bibr4-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1159\/000328137"},{"key":"bibr5-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-4362.2002.01680_1.x"},{"key":"bibr6-12034754241274356","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.13523"},{"key":"bibr7-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1684\/ejd.2023.4502"},{"key":"bibr8-12034754241274356","doi-asserted-by":"publisher","DOI":"10.4103\/ajm.ajm_177_19"},{"key":"bibr9-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2018-228464"},{"key":"bibr10-12034754241274356","volume-title":"StatPearls","author":"Majeed H","year":"2023"},{"key":"bibr11-12034754241274356","doi-asserted-by":"publisher","DOI":"10.1111\/bcp.13066"}],"container-title":["Journal of Cutaneous Medicine and Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/12034754241274356","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/12034754241274356","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/12034754241274356","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T15:51:05Z","timestamp":1777391465000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/12034754241274356"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,27]]},"references-count":11,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["10.1177\/12034754241274356"],"URL":"https:\/\/doi.org\/10.1177\/12034754241274356","relation":{},"ISSN":["1203-4754","1615-7109"],"issn-type":[{"value":"1203-4754","type":"print"},{"value":"1615-7109","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,27]]}}}