{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T03:35:37Z","timestamp":1789961737381,"version":"4.0.1"},"reference-count":41,"publisher":"American Medical Association (AMA)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Dermatol"],"published-print":{"date-parts":[[2023,3,1]]},"abstract":"<jats:sec id=\"ab-doi220073-4\"><jats:title>Importance<\/jats:title><jats:p>Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi220073-5\"><jats:title>Objective<\/jats:title><jats:p>To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi220073-6\"><jats:title>Design, Setting, and Participants<\/jats:title><jats:p>This pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi220073-7\"><jats:title>Main Outcomes and Measures<\/jats:title><jats:p>During the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and \u03ba statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman \u03c1 coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi220073-8\"><jats:title>Results<\/jats:title><jats:p>Among 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman \u03c1\u2009=\u20090.747; <jats:italic>P<\/jats:italic>\u2009&amp;amp;lt;\u2009.001) and the MAM and the Physician Global Assessment of Activity (Spearman \u03c1\u2009=\u20090.729; <jats:italic>P<\/jats:italic>\u2009&amp;amp;lt;\u2009.001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi220073-9\"><jats:title>Conclusions and Relevance<\/jats:title><jats:p>In this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamadermatol.2022.6365","type":"journal-article","created":{"date-parts":[[2023,2,8]],"date-time":"2023-02-08T16:05:23Z","timestamp":1675872323000},"page":"299","source":"Crossref","is-referenced-by-count":18,"title":["Development and Validation of the Morphea Activity Measure in Patients With Pediatric Morphea"],"prefix":"10.1001","volume":"159","author":[{"given":"Maria Teresa","family":"Garc\u00eda-Romero","sequence":"first","affiliation":[{"name":"Department of Dermatology, National Institute of Pediatrics, Mexico City, Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megha","family":"Tollefson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mayo Clinic and Mayo Clinic Children\u2019s Center, Rochester, Minnesota"},{"name":"Department of Dermatology, Mayo Clinic and Mayo Clinic Children\u2019s Center, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Pope","sequence":"additional","affiliation":[{"name":"Dermatology Section, Hospital for Sick Children, Toronto, Ontario, Canada"},{"name":"Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather A.","family":"Brandling-Bennett","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Department of Pediatrics, Seattle Children\u2019s Hospital, Seattle, Washington"},{"name":"Department of Medicine, School of Medicine, University of Washington, Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy S.","family":"Paller","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Northwestern University, Chicago, Illinois"},{"name":"Department of Pediatrics, Northwestern University, Chicago, Illinois"},{"name":"Department of Dermatology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Keimig","sequence":"additional","affiliation":[{"name":"VitalSkin Dermatology, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Arkin","sequence":"additional","affiliation":[{"name":"Department of Dermatology, School of Medicine and Public Health, University of Wisconsin, Madison, Madison"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolyn A.","family":"Wanat","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Medical College of Wisconsin, Milwaukee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen R.","family":"Humphrey","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Medical College of Wisconsin, Milwaukee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victoria P.","family":"Werth","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia"},{"name":"Department of Dermatology, Corporal Michael J. Crescenz VA Medical Center, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikash","family":"Oza","sequence":"additional","affiliation":[{"name":"Ronald O. Perelman Department of Dermatology, Grossman School of Medicine, New York University, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Jacobe","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Texas Southwestern Medical Center, Dallas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Fett","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Oregon Health and Science University, Portland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly M.","family":"Cordoro","sequence":"additional","affiliation":[{"name":"Department of Dermatology, School of Medicine, University of California, San Francisco, San Francisco"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Medina-Vera","sequence":"additional","affiliation":[{"name":"Department of Research Methodology, National Institute of Pediatrics, Mexico City"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvonne E.","family":"Chiu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Medical College of Wisconsin, Milwaukee"},{"name":"Department of Pediatrics, Medical College of Wisconsin, Milwaukee"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10","reference":[{"issue":"3","key":"doi220073r1","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/j.jaad.2008.05.005","article-title":"Pediatric morphea (localized scleroderma): review of 136 patients.","volume":"59","author":"Christen-Zaech","year":"2008","journal-title":"J Am Acad Dermatol"},{"issue":"7","key":"doi220073r2","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1016\/j.autrev.2018.02.004","article-title":"Disease course and long-term outcome of juvenile localized scleroderma: experience from a single pediatric rheumatology centre and literature review.","volume":"17","author":"Martini","year":"2018","journal-title":"Autoimmun Rev"},{"issue":"5","key":"doi220073r3","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1001\/jamadermatol.2020.0034","article-title":"Changes in disease activity and damage over time in patients with morphea.","volume":"156","author":"O\u2019Brien","year":"2020","journal-title":"JAMA Dermatol"},{"issue":"5","key":"doi220073r4","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1001\/jamadermatol.2020.0033","article-title":"At the leading edge in morphea\u2014new insights into disease course and management options.","volume":"156","author":"Cruz-Diaz","year":"2020","journal-title":"JAMA Dermatol"},{"issue":"3","key":"doi220073r5","doi-asserted-by":"publisher","first-page":"756","DOI":"10.3390\/jcm9030756","article-title":"Associations between disease activity\/severity and damage and health-related quality of life in adult patients with localized scleroderma\u2014a comparison of LoSCAT and visual analogue scales.","volume":"9","author":"Lis-Swiety","year":"2020","journal-title":"J Clin Med"},{"issue":"8","key":"doi220073r6","doi-asserted-by":"publisher","first-page":"1663","DOI":"10.1016\/j.jid.2017.04.008","article-title":"Transcriptional and cytokine profiles identify CXCL9 as a biomarker of disease activity in morphea.","volume":"137","author":"O\u2019Brien","year":"2017","journal-title":"J Invest Dermatol"},{"issue":"1","key":"doi220073r7","doi-asserted-by":"publisher","first-page":"20218","DOI":"10.1038\/s41598-020-76995-2","article-title":"Circulating miRNA-181b-5p, miRNA-223-3p, miRNA-210-3p, let 7i-5p, miRNA-21-5p and miRNA-29a-3p in patients with localized scleroderma as potential biomarkers.","volume":"10","author":"Wolska-Gawron","year":"2020","journal-title":"Sci Rep"},{"issue":"2","key":"doi220073r8","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1111\/srt.2019.25.issue-2","article-title":"Detecting and quantifying activity\/inflammation in localized scleroderma with thermal imaging.","volume":"25","author":"Ranosz-Janicka","year":"2019","journal-title":"Skin Res Technol"},{"key":"doi220073r9","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1186\/1546-0096-12-27","article-title":"A cross-sectional electromyography assessment in linear scleroderma patients.","volume":"12","author":"Saad Magalh\u00e3es","year":"2014","journal-title":"Pediatr Rheumatol Online J"},{"issue":"1","key":"doi220073r10","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1111\/ijd.2017.56.issue-1","article-title":"The role of local temperature and other clinical characteristics of localized scleroderma as markers of disease activity.","volume":"56","author":"Garcia-Romero","year":"2017","journal-title":"Int J Dermatol"},{"issue":"1","key":"doi220073r11","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1111\/jdv.13790","article-title":"A systematic review of tools for determining activity of localized scleroderma in paediatric and adult patients.","volume":"31","author":"Lis-Swiety","year":"2017","journal-title":"J Eur Acad Dermatol Venereol"},{"issue":"4","key":"doi220073r12","first-page":"650","article-title":"Localized scleroderma severity index and global assessments: a pilot study of outcome instruments.","volume":"35","author":"Arkachaisri","year":"2008","journal-title":"J Rheumatol"},{"issue":"12","key":"doi220073r13","doi-asserted-by":"publisher","first-page":"2819","DOI":"10.3899\/jrheum.081284","article-title":"The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures.","volume":"36","author":"Arkachaisri","year":"2009","journal-title":"J Rheumatol"},{"issue":"2","key":"doi220073r14","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1093\/rheumatology\/kep361","article-title":"Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study.","volume":"49","author":"Arkachaisri","year":"2010","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"doi220073r15","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/s12969-018-0254-9","article-title":"Reliability of LoSCAT score for activity and tissue damage assessment in a large cohort of patients with juvenile localized scleroderma.","volume":"16","author":"Agazzi","year":"2018","journal-title":"Pediatr Rheumatol Online J"},{"issue":"2","key":"doi220073r16","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1016\/j.jaad.2013.02.007","article-title":"The Localized Scleroderma Cutaneous Assessment Tool: responsiveness to change in a pediatric clinical population.","volume":"69","author":"Kelsey","year":"2013","journal-title":"J Am Acad Dermatol"},{"issue":"4","key":"doi220073r17","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1016\/j.jclinepi.2013.12.002","article-title":"Defining consensus: a systematic review recommends methodologic criteria for reporting of Delphi studies.","volume":"67","author":"Diamond","year":"2014","journal-title":"J Clin Epidemiol"},{"issue":"4","key":"doi220073r18","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1016\/j.eujim.2015.07.002","article-title":"Delphi methodology in health research: how to do it?","volume":"7","author":"Trevelyan","year":"2015","journal-title":"Eur J Integr Med"},{"issue":"2","key":"doi220073r20","doi-asserted-by":"publisher","first-page":"165","DOI":"10.15171\/jcs.2015.017","article-title":"Design and implementation content validity study: development of an instrument for measuring patient-centered communication.","volume":"4","author":"Zamanzadeh","year":"2015","journal-title":"J Caring Sci"},{"issue":"4","key":"doi220073r21","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1002\/(ISSN)1098-240X","article-title":"Is the CVI an acceptable indicator of content validity? appraisal and recommendations.","volume":"30","author":"Polit","year":"2007","journal-title":"Res Nurs Health"},{"issue":"12","key":"doi220073r22","first-page":"614","article-title":"Biostatistics 104: correlational analysis.","volume":"44","author":"Chan","year":"2003","journal-title":"Singapore Med J"},{"issue":"2","key":"doi220073r24","first-page":"127","article-title":"Developing criteria for establishing interrater reliability of specific items: applications to assessment of adaptive behavior.","volume":"86","author":"Cicchetti","year":"1981","journal-title":"Am J Ment Defic"},{"issue":"2","key":"doi220073r25","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1590\/bjpt-rbf.2014.0143","article-title":"The Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) and how to select an outcome measurement instrument.","volume":"20","author":"Mokkink","year":"2016","journal-title":"Braz J Phys Ther"},{"issue":"2","key":"doi220073r29","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.jcm.2016.02.012","article-title":"A guideline of selecting and reporting intraclass correlation coefficients for reliability research.","volume":"15","author":"Koo","year":"2016","journal-title":"J Chiropr Med"},{"issue":"4","key":"doi220073r30","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1159\/000335560","article-title":"Topical imiquimod 5% cream for pediatric plaque morphea: a prospective, multiple-baseline, open-label pilot study.","volume":"223","author":"Pope","year":"2011","journal-title":"Dermatology"},{"issue":"2 Pt 1","key":"doi220073r31","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1016\/S0190-9622(98)70077-5","article-title":"Topical calcipotriene for morphea\/linear scleroderma.","volume":"39","author":"Cunningham","year":"1998","journal-title":"J Am Acad Dermatol"},{"issue":"3","key":"doi220073r32","doi-asserted-by":"publisher","first-page":"181","DOI":"10.2165\/00128071-200910030-00004","article-title":"Efficacy of topical tacrolimus 0.1% in active plaque morphea: randomized, double-blind, emollient-controlled pilot study.","volume":"10","author":"Kroft","year":"2009","journal-title":"Am J Clin Dermatol"},{"issue":"2","key":"doi220073r33","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1111\/bjd.14001","article-title":"Correlation of clinical tools to determine activity of localized scleroderma in paediatric patients.","volume":"174","author":"Garcia-Romero","year":"2016","journal-title":"Br J Dermatol"},{"issue":"2","key":"doi220073r34","doi-asserted-by":"publisher","first-page":"398","DOI":"10.1111\/bjd.v182.2","article-title":"Using the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) to classify morphoea by severity and identify clinically significant change.","volume":"182","author":"Teske","year":"2020","journal-title":"Br J Dermatol"},{"issue":"7","key":"doi220073r35","doi-asserted-by":"publisher","first-page":"516","DOI":"10.1080\/07434610902744074","article-title":"Objective assessment of psoriasis erythema for PASI scoring.","volume":"33","author":"Ahmad Fadzil","year":"2009","journal-title":"J Med Eng Technol"},{"issue":"4","key":"doi220073r36","doi-asserted-by":"publisher","first-page":"760","DOI":"10.1016\/j.jaad.2014.05.044","article-title":"Reliability of Clinician Erythema Assessment grading scale.","volume":"71","author":"Tan","year":"2014","journal-title":"J Am Acad Dermatol"},{"issue":"3","key":"doi220073r37","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1111\/exd.v30.3","article-title":"The erythema Q-score, an imaging biomarker for redness in skin inflammation.","volume":"30","author":"Frew","year":"2021","journal-title":"Exp Dermatol"},{"issue":"3","key":"doi220073r38","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1016\/j.ijwd.2015.05.002","article-title":"The reliability and validity of outcome measures for atopic dermatitis in patients with pigmented skin: a grey area.","volume":"1","author":"Zhao","year":"2015","journal-title":"Int J Womens Dermatol"},{"issue":"1","key":"doi220073r39","doi-asserted-by":"publisher","first-page":"11","DOI":"10.5301\/jsrd.5000231","article-title":"Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis.","volume":"2","author":"Khanna","year":"2017","journal-title":"J Scleroderma Relat Disord"},{"issue":"6","key":"doi220073r40","first-page":"968","article-title":"Childhood linear scleroderma: a possible role of thermography for evaluation.","volume":"19","author":"Birdi","year":"1992","journal-title":"J Rheumatol"},{"issue":"10","key":"doi220073r41","doi-asserted-by":"publisher","first-page":"1178","DOI":"10.1093\/rheumatology\/41.10.1178","article-title":"Juvenile-onset localized scleroderma activity detection by infrared thermography.","volume":"41","author":"Martini","year":"2002","journal-title":"Rheumatology (Oxford)"},{"key":"doi220073r23","author":"Carmines","year":"1979"},{"key":"doi220073r26","author":"de Vet","year":"2011"},{"key":"doi220073r19","unstructured":"Massey? DS, Martin? JA. The NIS Skin Color Scale. 2003. Accessed August 3, 2018. https:\/\/nis.princeton.edu\/downloads\/NIS-Skin-Color-Scale.pdf"},{"key":"doi220073r27","unstructured":"Phelan? C, Wren? J. Exploring reliability in academic assessment. Office of Academic Assessment, University of Northern Iowa. 2005-2006. Accessed May 5, 2022. https:\/\/chfasoa.uni.edu\/reliabilityandvalidity.htm"},{"key":"doi220073r28","unstructured":"Glen? S. Average inter-item correlation: definition, example. StatisticsHowTo.com. Accessed April 20, 2021. https:\/\/www.statisticshowto.com\/average-inter-item-correlation"}],"container-title":["JAMA Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/jamanetwork.com\/journals\/jamadermatology\/articlepdf\/2800954\/jamadermatology_garcaromero_2023_oi_220073_1677862977.5701.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,15]],"date-time":"2023-03-15T15:13:33Z","timestamp":1678893213000},"score":1,"resource":{"primary":{"URL":"https:\/\/jamanetwork.com\/journals\/jamadermatology\/fullarticle\/2800954"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,1]]},"references-count":41,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,3,1]]}},"URL":"https:\/\/doi.org\/10.1001\/jamadermatol.2022.6365","relation":{},"ISSN":["2168-6068"],"issn-type":[{"value":"2168-6068","type":"print"}],"subject":[],"published":{"date-parts":[[2023,3,1]]}}}