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Current medical treatments often show limited and inconsistent effectiveness, highlighting the need to better define the role of alternative approaches. To present a case of extensive dermatomyositis-associated calcinosis successfully managed with surgical excision and flap reconstruction, and to systematically review the role of surgical management in autoimmune-related calcinosis. A case report is presented together with a literature review conducted according to PRISMA guidelines. PubMed, Scopus, DOAJ and the Cochrane Library were searched from inception to June 21, 2026 for studies reporting surgical management of calcinosis in autoimmune connective tissue diseases. A total of 13 case reports and 3 case series were identified, including predominantly female patients with a median age of 55.3\u00a0years. Surgical intervention was mainly performed in symptomatic cases, such as pain, infection, or functional impairment. Overall, surgery was associated with pain relief and functional improvement, although recurrence and complications, including wound healing issues, were reported. Pharmacological therapies demonstrated variable and generally limited efficacy. Data on reconstructive techniques were scarce; however, available evidence suggests that flap reconstruction and grafting may improve outcomes in complex cases. Surgical management may represent a valuable treatment option for selected patients with calcinosis associated with autoimmune diseases, particularly in refractory or symptomatic cases. Reconstructive techniques may further enhance functional outcomes, although evidence remains limited. An individualized, multidisciplinary approach is recommended, and further studies are needed to establish optimal management strategies.\n                    <jats:italic>Registration<\/jats:italic>\n                    PROSPERO (CRD420261359925).\n                  <\/jats:p>","DOI":"10.1007\/s00296-026-06232-y","type":"journal-article","created":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T02:50:39Z","timestamp":1783997439000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Surgical management of autoimmune-associated calcinosis: a dermatomyositis case and case-based review"],"prefix":"10.1007","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-6325-8649","authenticated-orcid":false,"given":"Melina","family":"Yerolatsite","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-7637-7317","authenticated-orcid":false,"given":"Lamprina","family":"Magkou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1844-1264","authenticated-orcid":false,"given":"Konstantinos","family":"Seretis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-9528-0583","authenticated-orcid":false,"given":"Nikolaos","family":"Zintziovas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-8134-9128","authenticated-orcid":false,"given":"Anastasia K.","family":"Zikou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5193-2284","authenticated-orcid":false,"given":"Paraskevi V.","family":"Voulgari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,14]]},"reference":[{"issue":"4","key":"6232_CR1","doi-asserted-by":"publisher","first-page":"527","DOI":"10.1016\/S0190-9622(98)70001-5","volume":"39","author":"DM Touart","year":"1998","unstructured":"Touart DM, Sau P (1998) Cutaneous deposition diseases. 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This study was conducted in accordance with the Declaration of Helsinki and was approved by the Institutional Review Board of the University Hospital of Ioannina (Protocol code: 283\/2-4-2026).","order":2,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Informed consent was obtained from the participant included in the study.","order":3,"name":"Ethics","label":"Consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Participant provided consent for publication of the study findings.","order":4,"name":"Ethics","label":"Consent for publication","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"203"}}