{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T05:06:50Z","timestamp":1772168810139,"version":"3.50.1"},"update-to":[{"DOI":"10.12688\/f1000research.13604.1","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2018,3,26]],"date-time":"2018-03-26T00:00:00Z","timestamp":1522022400000}}],"reference-count":9,"publisher":"F1000 Research Ltd","license":[{"start":{"date-parts":[[2018,3,26]],"date-time":"2018-03-26T00:00:00Z","timestamp":1522022400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["f1000research.com"],"crossmark-restriction":false},"short-container-title":["F1000Res"],"abstract":"<ns4:p>A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission.<\/ns4:p>\n                  <ns4:p>Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute, clinically amyopathic dermatomyositis in adults. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).<\/ns4:p>","DOI":"10.12688\/f1000research.13604.2","type":"journal-article","created":{"date-parts":[[2018,3,26]],"date-time":"2018-03-26T04:00:14Z","timestamp":1522036814000},"page":"60","update-policy":"https:\/\/doi.org\/10.12688\/f1000research.crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema"],"prefix":"10.12688","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2925-9679","authenticated-orcid":false,"given":"Efthymia","family":"Pappa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marina","family":"Gkeka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asimina","family":"Protogerou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonidas","family":"Marinos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chariclia","family":"Loupa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Constantinos","family":"Christopoulos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2560","published-online":{"date-parts":[[2018,3,26]]},"reference":[{"key":"ref-1","doi-asserted-by":"publisher","first-page":"971-82","DOI":"10.1016\/S0140-6736(03)14368-1","article-title":"Polymyositis and dermatomyositis.","volume":"362","author":"M Dalakas","year":"2003","journal-title":"Lancet."},{"key":"ref-2","doi-asserted-by":"publisher","first-page":"275-85","DOI":"10.1007\/s11926-012-0249-3","article-title":"Morbidity and Mortality in Adult Polymyositis and Dermatomyositis.","volume":"14","author":"I Marie","year":"2012","journal-title":"Curr Rheumatol Rep."},{"key":"ref-3","doi-asserted-by":"publisher","first-page":"597-613","DOI":"10.1016\/j.jaad.2005.10.041","article-title":"A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sin\u00e9 myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.","volume":"54","author":"P Gerami","year":"2006","journal-title":"J Am Acad Dermatol."},{"key":"ref-4","doi-asserted-by":"publisher","first-page":"466-7","DOI":"10.1046\/j.1365-4362.2003.01696.x","article-title":"Periorbital edema as the presenting sign of dermatomyositis.","volume":"42","author":"V Hall","year":"2003","journal-title":"Int J Dermatol."},{"key":"ref-5","doi-asserted-by":"publisher","first-page":"228-33","DOI":"10.1016\/j.semarthrit.2014.04.004","article-title":"Dermatomyositis presenting with severe subcutaneous edema: five additional cases and review of the literature.","volume":"44","author":"J Milisenda","year":"2014","journal-title":"Semin Arthritis Rheum."},{"key":"ref-6","first-page":"21244","article-title":"Increasing recognition of dermatomyositis with subcutaneous edema - is this a poorer prognostic marker?","volume":"20","author":"J Tu","year":"2014","journal-title":"Dermatol Online J."},{"key":"ref-7","doi-asserted-by":"publisher","first-page":"J276-86","DOI":"10.1016\/j.jaut.2009.11.014","article-title":"The complement system in systemic autoimmune disease.","volume":"34","author":"M Chen","year":"2010","journal-title":"J Autoimmun."},{"key":"ref-8","doi-asserted-by":"publisher","first-page":"3224-38","DOI":"10.1002\/art.23884","article-title":"Vascular endothelial growth factor is highly expressed in muscle tissue of patients with polymyositis and patients with dermatomyositis.","volume":"58","author":"C Grundtman","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref-9","doi-asserted-by":"publisher","first-page":"L678-84","DOI":"10.1152\/ajplung.1998.274.5.L678","article-title":"VEGF induces hyperpermeability by a direct action on endothelial cells.","volume":"274","author":"S Hippenstiel","year":"1998","journal-title":"Am J Physiol Lung Cell Mol Physiol."}],"container-title":["F1000Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/f1000research.com\/articles\/7-60\/v2\/xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/7-60\/v2\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/f1000research.com\/articles\/7-60\/v2\/iparadigms","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,12,6]],"date-time":"2018-12-06T06:08:01Z","timestamp":1544076481000},"score":1,"resource":{"primary":{"URL":"https:\/\/f1000research.com\/articles\/7-60\/v2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,3,26]]},"references-count":9,"URL":"https:\/\/doi.org\/10.12688\/f1000research.13604.2","relation":{"has-review":[{"id-type":"doi","id":"10.5256\/f1000research.15685.r32410","asserted-by":"subject"},{"id-type":"doi","id":"10.5256\/f1000research.15685.r32410","asserted-by":"object"}]},"ISSN":["2046-1402"],"issn-type":[{"value":"2046-1402","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,3,26]]},"assertion":[{"value":"Indexed","URL":"https:\/\/f1000research.com\/articles\/7-60\/v2#article-reports","order":0,"name":"referee-status","label":"Referee status","group":{"name":"current-referee-status","label":"Current Referee Status"}},{"value":"10.5256\/f1000research.15685.r32410, Lyubomir\u00a0A. Dourmishev, Department of Dermatology and Venereology, Medical University, Sofia, Sofia, Bulgaria, 03 Apr 2018, version 2, indexed","URL":"https:\/\/f1000research.com\/articles\/7-60\/v2#referee-response-32410","order":0,"name":"referee-response-32410","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"10.5256\/f1000research.14778.r31365, Albert Selva-O'Callaghan, Department\u00a0of\u00a0Internal Medicine, Autonomous University of Barcelona, Barcelona, Spain, 05 Mar 2018, version 1, 1 approved, 1 approved with reservations","URL":"https:\/\/f1000research.com\/articles\/7-60\/v1#referee-response-31365","order":1,"name":"referee-response-31365","label":"Referee Report","group":{"name":"article-reports","label":"Article Reports"}},{"value":"<b>Efthymia Pappa<\/b>; \n<i>Posted: 22 Mar 2018<\/i>; We thank Dr. Selva-O\u2019Callaghan for critically reviewing our manuscript. Our response is as follows:  We have used the term \u201cclinically amyopathic dermatomyositis\u201d in the revised version, as suggested by both reviewers. We have also clarified that the skin biopsy findings were compatible with but not diagnostic of DM. Among the autoantibodies specific for myositis, we only had access to anti-Mi2 and anti-Jo1. Anti-Mi2 tends to be positive in classical DM with typical skin manifestations and high muscle enzyme levels, both of which were absent in our case. Positive anti-Mi2 was reported in 38.8% of DM patients from the Indian subcontinent (P Srivastava \n<i>et al<\/i>. Rheumatol Int 2016, DOI 10.1007\/s00296-016-3494-3). In the same ethnic group, Jo1 was detected in 5.4% of DM patients. Although the face is frequently the only area affected in dermatomyositis, particularly in the early stages, we could find no published cases of adult DM &nbsp;presenting with isolated facial edema without rash or muscle enzyme abnormalities.&nbsp;","URL":"https:\/\/f1000research.com\/articles\/7-60\/v2#referee-comment-3539","order":2,"name":"referee-comment-3539","label":"Referee Comment","group":{"name":"article-reports","label":"Article Reports"}},{"value":"The author(s) declared that no grants were involved in supporting this work","order":3,"name":"grant-information","label":"Grant Information"},{"value":"This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Data associated with the article are available under the terms of the Creative Commons Zero \"No rights reserved\" data waiver (CC0 1.0 Public domain dedication).","order":0,"name":"copyright-info","label":"Copyright"}]}}