{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T11:26:20Z","timestamp":1767180380575,"version":"build-2238731810"},"reference-count":17,"publisher":"Public Library of Science (PLoS)","issue":"6","license":[{"start":{"date-parts":[[2021,6,28]],"date-time":"2021-06-28T00:00:00Z","timestamp":1624838400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:sec id=\"sec001\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin biopsies in SS patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec002\">\n                    <jats:title>Materials and methods<\/jats:title>\n                    <jats:p>We studied skin biopsies from patients with a clinical diagnosis of SS or isolated LR. We also studied controls with vitiligo or normal skin. Biopsies were considered standardized if 3 biopsies were taken from the white centre of the livedo and reached until the dermis-subcutis border. Biopsies were scored for features of an occlusive microangiopathy without knowledge of the clinical features. Sensitivity and specificity of the biopsy findings were calculated with the clinical criteria as the reference standard.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec003\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We included 34 SS patients, 14 isolated LR patients and 41 control patients. Biopsies of 17 patients with SS (50%), 4 with isolated LR (31%) and 10 control patients (24%) showed at least one artery in the deep dermis with a thickened vessel wall combined with recanalization or neovascularization (sensitivity 50% and specificity 69% with LR as reference). Standardized biopsies increased the sensitivity to 70%. In a post hoc analysis the combination of an occlusive microangiopathy and the presence of a livedo pattern in the superficial dermis increased the specificity to 92%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec004\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Standardized skin biopsies can support the clinical diagnosis of SS. An occlusive microangiopathy as the only positive criterion for the diagnosis of SS had insufficient specificity for a definite diagnosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1371\/journal.pone.0253365","type":"journal-article","created":{"date-parts":[[2021,6,28]],"date-time":"2021-06-28T13:31:03Z","timestamp":1624887063000},"page":"e0253365","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":4,"title":["The diagnostic value of skin biopsies in Sneddon syndrome"],"prefix":"10.1371","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8033-7126","authenticated-orcid":true,"given":"N. L. P.","family":"Starmans","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Zoetemeyer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. R.","family":"van Dijk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L. J.","family":"Kappelle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. J. M.","family":"Frijns","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2021,6,28]]},"reference":[{"key":"pone.0253365.ref001","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1111\/j.1365-2133.1965.tb14628.x","article-title":"Cerebro-vascular lesions and livedo reticularis","volume":"77","author":"I Sneddon","year":"1965","journal-title":"Br J Dermatol"},{"key":"pone.0253365.ref002","article-title":"Sneddon\u2019s syndrome: A comprehensive review of the literature","author":"S Wu","year":"2014","journal-title":"Orphanet J Rare Dis"},{"key":"pone.0253365.ref003","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1097\/00005792-199907000-00001","article-title":"Sneddon syndrome with or without antiphospholipid antibodies: A comparative study in 46 patients","volume":"78","author":"C Franc\u00e8s","year":"1999","journal-title":"Medicine (Baltimore)"},{"key":"pone.0253365.ref004","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1002\/ana.24382","article-title":"Strokes in Sneddon syndrome without antiphospholipid antibodies","volume":"77","author":"L Bottin","year":"2015","journal-title":"Ann Neurol"},{"key":"pone.0253365.ref005","article-title":"Sneddon syndrome: a comprehensive clinical review of 53 patients","author":"NLP Starmans","year":"2021","journal-title":"J Neurol"},{"key":"pone.0253365.ref006","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1046\/j.1365-2133.2001.04348.x","article-title":"Diagnostic impact and sensitivity of skin biopsies in Sneddon\u2019s syndrome. A report of 15 cases","volume":"145","author":"J Wohlrab","year":"2001","journal-title":"Br J Dermatol"},{"key":"pone.0253365.ref007","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1016\/0046-8177(92)90323-U","article-title":"Life history of cutaneous vascular lesions in Sneddon\u2019s syndrome","volume":"23","author":"B Zelger","year":"1992","journal-title":"Hum Pathol"},{"key":"pone.0253365.ref008","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1097\/00000478-199504000-00006","article-title":"Sneddon\u2019s syndrome\u2013An inflammatory disorder of small arteries followed by smooth muscle proliferation","volume":"19","author":"N Sepp","year":"1995","journal-title":"Am J Surg Pathol"},{"key":"pone.0253365.ref009","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.jaad.2010.11.061","article-title":"Vitiligo: A comprehensive overview","volume":"65","author":"A Alikhan","year":"2011","journal-title":"J Am Acad Dermatol"},{"key":"pone.0253365.ref010","doi-asserted-by":"crossref","first-page":"2276","DOI":"10.1161\/STROKEAHA.108.192218","article-title":"Definition and evaluation of transient ischemic attack","volume":"40","author":"JD Easton","year":"2009","journal-title":"Stroke"},{"key":"pone.0253365.ref011","first-page":"59","article-title":"Sneddon\u2019s syndrome: Clinical and laboratory analysis of 10 cases","volume":"58","author":"E Bolayir","year":"2004","journal-title":"Acta Med Okayama"},{"key":"pone.0253365.ref012","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/0303-8467(85)90115-5","article-title":"Livedo racemosa generalisata and stroke","volume":"87","author":"R De Reus","year":"1985","journal-title":"Clin Neurol Neurosurg"},{"key":"pone.0253365.ref013","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/0303-8467(94)90120-1","article-title":"Sneddon\u2019s syndrome: Clinical and immunohistochemical findings","volume":"96","author":"V Fetoni","year":"1994","journal-title":"Clin Neurol Neurosurg"},{"key":"pone.0253365.ref014","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1212\/WNL.40.3_Part_1.464","article-title":"Anticardiolipin antibodies in Sneddon\u2019s syndrome","volume":"40","author":"LA Kalashnikova","year":"1990","journal-title":"Neurology"},{"key":"pone.0253365.ref015","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1161\/01.STR.24.5.685","article-title":"Sneddon\u2019s syndrome: Diagnosis by skin biopsy and MRI in 17 patients","volume":"24","author":"G Stockhammer","year":"1993","journal-title":"Stroke"},{"key":"pone.0253365.ref016","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s100720070091","article-title":"Clinical and neuroradiological aspects of Sneddon\u2019s syndrome and primary antiphospholipid antibody syndrome. A follow-up study","volume":"21","author":"V Fetoni","year":"2000","journal-title":"Neurol Sci"},{"key":"pone.0253365.ref017","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1007\/s00415-005-0967-9","article-title":"The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review","volume":"252","author":"M Kraemer","year":"2005","journal-title":"J Neurol"}],"updated-by":[{"DOI":"10.1371\/journal.pone.0314784","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2024,11,27]],"date-time":"2024-11-27T00:00:00Z","timestamp":1732665600000}}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0253365","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,28]],"date-time":"2021-06-28T13:31:19Z","timestamp":1624887079000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0253365"}},"subtitle":[],"editor":[{"given":"Feroze","family":"Kaliyadan","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2021,6,28]]},"references-count":17,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2021,6,28]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0253365","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,28]]}}}