{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,10]],"date-time":"2026-02-10T18:22:55Z","timestamp":1770747775947,"version":"3.49.0"},"reference-count":42,"publisher":"The Journal of Rheumatology","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2016,9]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>Gottron papules and Gottron sign are characteristic and possibly pathognomonic cutaneous features of classic dermatomyositis and clinically amyopathic dermatomyositis (DM\/CADM). However, the Gottron papules\/Gottron sign with cutaneous ulceration (ulcerative Gottron papules\/Gottron sign) are less common. We aimed to clarify the clinical characteristics of patients with DM\/CADM who have ulcerative Gottron papules\/Gottron sign.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>Clinical features, laboratory findings, and prognosis of patients with DM\/CADM who had Gottron papules\/Gottron sign with or without ulceration were analyzed and compared.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>Occurrences of acute interstitial pneumonia\/subacute interstitial pneumonia (AIP\/SIP) were significantly higher in patients with ulcerative Gottron papules\/Gottron sign (19\/26) versus patients with Gottron papules\/Gottron sign without ulceration (2\/66, p &lt; 0.001). We also observed that the white blood cell counts (mean \u00b1 SD 4.2 \u00b1 1.6 vs 6.9 \u00b1 2.9; p &lt; 0.001) and creatine kinase (CK) levels (198.0 \u00b1 377.7 vs 1364.0 \u00b1 2477.0; p = 0.019) were significantly lower, whereas the positive rate of antimelanoma differentiation-associated gene 5 antibody (anti-MDA5; 88.5% vs 6.1%, p &lt; 0.001) and serum ferritin levels (665.2 \u00b1 433.5 vs 256.2 \u00b1 279.0, p &lt; 0.001) were significantly higher in the patients with ulcerative Gottron papules\/Gottron sign. Moreover, the cumulative survival rate of the group with ulcerative Gottron papules\/Gottron sign was significantly lower (p &lt; 0.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>Patients with DM\/CADM who have ulcerative Gottron papules\/Gottron sign, positive anti-MDA5 antibody, and significantly lower baseline CK level are at increased risk of interstitial lung disease, especially AIP\/SIP. A new designation for this subgroup of patients should be established to draw more attention to this clinical entity.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.160024","type":"journal-article","created":{"date-parts":[[2016,6,16]],"date-time":"2016-06-16T00:57:22Z","timestamp":1466038642000},"page":"1735-1742","source":"Crossref","is-referenced-by-count":50,"title":["Gottron Papules and Gottron Sign with Ulceration: A Distinctive Cutaneous Feature in a Subset of Patients with Classic Dermatomyositis and Clinically Amyopathic Dermatomyositis"],"prefix":"10.3899","volume":"43","author":[{"given":"Hua","family":"Cao","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qunli","family":"Xia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meng","family":"Pan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoqing","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xia","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruofei","family":"Shi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Min","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoyi","family":"Ding","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masataka","family":"Kuwana","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jie","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2262","published-online":{"date-parts":[[2016,6,15]]},"reference":[{"key":"2018112909361027000_43.9.1735.1","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.120621"},{"key":"2018112909361027000_43.9.1735.2","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-009-1152-9"},{"key":"2018112909361027000_43.9.1735.3","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-007-0562-9"},{"key":"2018112909361027000_43.9.1735.4","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-005-1164-z"},{"key":"2018112909361027000_43.9.1735.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.10794"},{"key":"2018112909361027000_43.9.1735.6","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e31819da352"},{"key":"2018112909361027000_43.9.1735.7","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-857X(02)00024-8"},{"key":"2018112909361027000_43.9.1735.8","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-199911000-00006"},{"key":"2018112909361027000_43.9.1735.9","unstructured":"Sontheimer RD . 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