{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T13:22:08Z","timestamp":1784035328834,"version":"3.55.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,7,25]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and systemic complications, including malignancy and interstitial lung disease (ILD). Myositis-specific autoantibodies (MSAs) define distinct disease subtypes, but significant clinical heterogeneity can still occur. Here, we report a unique case of smouldering myeloma-associated anti-nuclear matrix protein 2 (anti-NXP2) antibody-positive DM mimicking clinical features of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive DM, including digital ischemia and palmar papules (i.e. inverse Gottron\u2019s papules), and severe synovitis without clinically evident myopathy. Additionally, the patient exhibited a rare scrotal rash. This case expands the known spectrum of cutaneous and systemic manifestations in anti-NXP2 antibody-positive DM and emphasises the heterogeneous nature of DM, where significant clinical mimicry can occur despite the presence of a well-defined MSA. It highlights the need for further research into the immune mechanisms and biomarker profiles driving these mimicking DM phenotypes, which could improve early diagnosis, risk stratification, and targeted therapeutic strategies.<\/jats:p>","DOI":"10.1093\/mrcr\/rxaf047","type":"journal-article","created":{"date-parts":[[2025,7,18]],"date-time":"2025-07-18T14:08:59Z","timestamp":1752847739000},"source":"Crossref","is-referenced-by-count":2,"title":["Anti-nuclear matrix protein 2 antibody-positive dermatomyositis associated with smouldering myeloma mimicking anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: A case of digital gangrene, palmar papules, and scrotal rash"],"prefix":"10.1093","volume":"9","author":[{"given":"Saloni","family":"Patel","sequence":"first","affiliation":[{"name":"Department of Dermatology, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziga","family":"Vodusek","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaroslaw J","family":"Jedrych","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jemima","family":"Albayda","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-7883-0798","authenticated-orcid":false,"given":"Jun","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Johns Hopkins University School of Medicine , Baltimore, MD ,","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,7,18]]},"reference":[{"key":"2025081214065284100_ref1","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-540-79313-7","volume-title":"Dermatomyositis","author":"Dourmishev","year":"2009"},{"key":"2025081214065284100_ref2","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1002\/acr.22498","article-title":"Cutaneous ulceration in dermatomyositis: association with anti-melanoma differentiation-associated gene 5 antibodies and interstitial lung disease","volume":"67","author":"Narang","year":"2015","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2025081214065284100_ref3","doi-asserted-by":"crossref","first-page":"773352","DOI":"10.3389\/fimmu.2021.773352","article-title":"Dermatomyositis with anti-MDA5 antibodies: bioclinical features, pathogenesis and emerging therapies","volume":"12","author":"Nombel","year":"2021","journal-title":"Front Immunol"},{"key":"2025081214065284100_ref4","doi-asserted-by":"crossref","first-page":"426","DOI":"10.5114\/ada.2020.99754","article-title":"Scrotum involvement in dermatomyositis","volume":"39","author":"Bag\u0142aj-Oleszczuk","year":"2022","journal-title":"Postepy Dermatol Alergol"},{"key":"2025081214065284100_ref5","volume":"69","author":"Albayda","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2025081214065284100_ref6","volume":"12","author":"Rajkumar","journal-title":"Blood Cancer J"},{"key":"2025081214065284100_ref7","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1196\/annals.1361.047","article-title":"Cancer-associated myositis: clinical features and prognostic signs","volume":"1051","author":"Ponyi","year":"2005","journal-title":"Ann N Y Acad Sci"},{"key":"2025081214065284100_ref8","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.ad.2013.11.007","article-title":"Paraneoplastic dermatomyositis: a study of 12 cases","volume":"105","author":"Requena","year":"2014","journal-title":"Actas Dermosifiliogr"},{"key":"2025081214065284100_ref9","doi-asserted-by":"crossref","first-page":"e8884","DOI":"10.1002\/ccr3.8884","article-title":"Anti-nuclear matrix protein 2 antibody-positive amyopathic dermatomyositis presenting in a patient with prostate cancer: a case report","volume":"12","author":"Shan","year":"2024","journal-title":"Clin Case Rep"},{"key":"2025081214065284100_ref10","first-page":"1","article-title":"NXP-2 positive dermatomyositis: a unique clinical presentation","volume":"2017","author":"Butt","year":"2017","journal-title":"Case Rep Rheumatol"},{"key":"2025081214065284100_ref11","first-page":"e42118","article-title":"Anti-Ro52\/anti-nuclear matrix protein 2 positive clinically amyopathic dermatomyositis presented only with interstitial lung disease","volume":"15","author":"Skrimizeas","year":"2023","journal-title":"Cureus."},{"key":"2025081214065284100_ref12","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/s41584-023-01045-w","article-title":"International guideline for idiopathic inflammatory myopathy-associated cancer screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative","volume":"19","author":"Oldroyd","year":"2023","journal-title":"Nat Rev Rheumatol"},{"key":"2025081214065284100_ref13","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1093\/rheumatology\/keab177","article-title":"Palmar erythema and palmar papules as predictors for dermatomyositis-related acute\/subacute interstitial lung disease: a retrospective study","volume":"61","author":"Lu","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"2025081214065284100_ref14","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.jaad.2010.09.016","article-title":"The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study","volume":"65","author":"Fiorentino","year":"2011","journal-title":"J Am Acad Dermatol"},{"key":"2025081214065284100_ref15","doi-asserted-by":"crossref","first-page":"436","DOI":"10.21037\/atm-20-5252","article-title":"Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis","volume":"9","author":"Castillo","year":"2021","journal-title":"Ann Transl Med"},{"key":"2025081214065284100_ref16","doi-asserted-by":"crossref","first-page":"1209282","DOI":"10.3389\/fimmu.2023.1209282","article-title":"Predictors of rapidly progressive interstitial lung disease and prognosis in Chinese patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis","volume":"14","author":"Li","year":"2023","journal-title":"Front 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patients","volume":"26","author":"Jayaraman","year":"2025","journal-title":"J Autoimmun"},{"key":"2025081214065284100_ref20","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1001\/jamadermatol.2013.10416","article-title":"Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis","volume":"150","author":"Valenzuela","year":"2014","journal-title":"JAMA Dermatol"},{"key":"2025081214065284100_ref21","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1093\/rheumatology\/key183","article-title":"Scrotal calcinosis in an adult patient with amyopathic dermatomyositis","volume":"58","author":"Steel","year":"2019","journal-title":"Rheumatology (Oxford)"},{"key":"2025081214065284100_ref22","first-page":"303","article-title":"Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options","volume":"35","author":"Fredi","year":"2017","journal-title":"Clin Exp Rheumatol"},{"key":"2025081214065284100_ref23","first-page":"382","article-title":"Scrotum and testicular calcinosis in juvenile dermatomyositis (JDM): a report of two cases","volume":"27","author":"Sallum","year":"2009","journal-title":"Clin Exp Rheumatol"},{"key":"2025081214065284100_ref24","first-page":"176","article-title":"Penile and scrotum swelling in juvenile dermatomyositis","volume":"36","author":"Sallum","year":"2011","journal-title":"Acta Reumatol Port"},{"key":"2025081214065284100_ref25","doi-asserted-by":"crossref","first-page":"bcr2017222915","DOI":"10.1136\/bcr-2017-222915","article-title":"Extensive skin ulcers in a child with juvenile dermatomyositis","volume":"2018","author":"Gupta","year":"2018","journal-title":"BMJ Case Rep"},{"key":"2025081214065284100_ref26","doi-asserted-by":"crossref","first-page":"810785","DOI":"10.3389\/fped.2021.810785","article-title":"Is anti-NXP2 autoantibody a risk factor for calcinosis and poor outcome in juvenile dermatomyositis 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dermatomyositis shed insight into the cancer-myositis connection","volume":"64","author":"Fiorentino","year":"2012","journal-title":"Arthritis Rheum"},{"key":"2025081214065284100_ref30","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1016\/j.jaad.2014.12.009","article-title":"Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1\u03b3 antibodies in adults with dermatomyositis","volume":"72","author":"Fiorentino","year":"2015","journal-title":"J Am Acad Dermatol"},{"key":"2025081214065284100_ref31","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.3899\/jrheum.200399","article-title":"Ulcerative paraneoplastic dermatomyositis in the setting of positive transcriptional intermediary factor 1-\u03b3 antibody","volume":"48","author":"Al Awqati","year":"2021","journal-title":"J Rheumatol"}],"container-title":["Modern Rheumatology Case 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