{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,27]],"date-time":"2025-04-27T11:10:03Z","timestamp":1745752203209,"version":"3.40.4"},"reference-count":12,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2018,5,25]]},"abstract":"<jats:p>Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2\u2009years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud\u2019s phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema.<\/jats:p>","DOI":"10.1136\/bcr-2017-224148","type":"journal-article","created":{"date-parts":[[2018,6,16]],"date-time":"2018-06-16T05:54:58Z","timestamp":1529128498000},"page":"bcr-2017-224148","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Lymphedema secondary to limited cutaneous systemic sclerosis"],"prefix":"10.1136","volume":"2018","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3363-0589","authenticated-orcid":false,"given":"S\u00f3nia","family":"Moreira","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jorge","family":"Crespo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L\u00e8lita","family":"Santos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2018,6,15]]},"reference":[{"key":"2025042703541597000_2018.jun14_2.bcr-2017-224148.1","doi-asserted-by":"crossref","unstructured":"Varga J , Denton CP . et al Systemic sclerosis and the scleroderma spectrum disorders. In: Firestein GS , Budd RC , Harris ED , McInnes IB , Ruddy S , Sergent JS , . eds. Kelley\u2019s textbook of rheumatology. 8th edn. Philadelphia: W.B. 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