Cutaneous Outcome Measures of Adult Dermatomyositis

  • Rebecca G. Gaffney
  • Victoria P. WerthEmail author
Living reference work entry


Dermatomyositis is an autoimmune skin disease marked by inflammation of the skin and muscle. The skin manifestations significantly impact quality of life and are difficult to treat. Therefore, clinical trials for effective therapies to treat the skin involvement in dermatomyositis are critical. There are many validated outcome measures that allow physicians to objectively evaluate response to treatment in clinical trials and in the clinic. This chapter reviews the following popular tools to assess skin severity in dermatomyositis: the Cutaneous Dermatomyositis Disease Area and Severity Index, the Physician Global Assessment, and the Cutaneous Assessment Tool-Binary Method. Based on validation studies outlined in this chapter, it appears that the Cutaneous Dermatomyositis Disease Area and Severity Index demonstrates superiority over the other tools and is a useful outcome measure for dermatomyositis.


Dermatomyositis Clinical trials Validated tools CDASI PGA CAT-BM 


  1. Anyanwu CO, Fiorentino DF, Chung L, Dzuong C, Wang Y, Okawa J, et al. Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index: characterizing disease severity and assessing responsiveness to clinical change. Br J Dermatol. 2015;173(4):969–74.CrossRefGoogle Scholar
  2. Anyanwu CO, Chansky PB, Feng R, Carr K, Okawa J, Werth VP. The systemic management of cutaneous dermatomyositis: Results of a stepwise strategy. Int J Women’s Derm. 2017;3(4):189–94.CrossRefGoogle Scholar
  3. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006;24(5):363–73.CrossRefGoogle Scholar
  4. Chansky PB, Olazagasti JM, Feng R, Werth VP. Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI). J Am Acad Dermatol. 2017;79(3):464–469.CrossRefGoogle Scholar
  5. Gaines E, Werth VP. Development of outcome measures for autoimmune dermatoses. Arch Dermatol Res. 2008;300(1):3–9.CrossRefGoogle Scholar
  6. Goreshi R, Okawa J, Rose M, Feng R, Lee LA, Hansen CB, et al. Evaluation of reliability, validity, and responsiveness of the CDASI and the CAT-BM. J Invest Dermatol. 2012;132(4):1117–24.CrossRefGoogle Scholar
  7. Huard C, Gulla SV, Bennett DV, Coyle AJ, Vleugels RA, Greenberg SA. Correlation of cutaneous disease activity with type 1 interferon gene signature and interferon beta in dermatomyositis. Br J Dermatol. 2017;176(5):1224–30.CrossRefGoogle Scholar
  8. Huber AM, Dugan EM, Lachenbruch PA, Feldman BM, Perez MD, Zemel LS, et al. Preliminary validation and clinical meaning of the Cutaneous Assessment Tool in juvenile dermatomyositis. Arthritis Rheum. 2008a;59(2):214–21.CrossRefGoogle Scholar
  9. Huber AM, Lachenbruch PA, Dugan EM, Miller FW, Rider LG. Juvenile Dermatomyositis Disease Activity Collaborative Study G. Alternative scoring of the Cutaneous Assessment Tool in juvenile dermatomyositis: results using abbreviated formats. Arthritis Rheum. 2008b;59(3):352–6.CrossRefGoogle Scholar
  10. Hundley JL, Carroll CL, Lang W, Snively B, Yosipovitch G, Feldman SR, et al. Cutaneous symptoms of dermatomyositis significantly impact patients’ quality of life. J Am Acad Dermatol. 2006;54(2):217–20.CrossRefGoogle Scholar
  11. Klein RQ, Bangert CA, Costner M, Connolly MK, Tanikawa A, Okawa J, et al. Comparison of the reliability and validity of outcome instruments for cutaneous dermatomyositis. Br J Dermatol. 2008;159(4):887–94.CrossRefGoogle Scholar
  12. Rider LG, Giannini EH, Harris-Love M, Joe G, Isenberg D, Pilkington C, et al. Defining Clinical Improvement in Adult and Juvenile Myositis. J Rheumatol. 2003;30(3):603–17.PubMedGoogle Scholar
  13. Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, et al. Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res (Hoboken). 2011;63(Suppl 11):S118–57.CrossRefGoogle Scholar
  14. Robinson ES, Feng R, Okawa J, Werth VP. Improvement in the cutaneous disease activity of patients with dermatomyositis is associated with a better quality of life. Br J Dermatol. 2015;172(1):169–74.CrossRefGoogle Scholar
  15. Singer AJ, Thode HC Jr, Hollander JE. Research fundamentals: selection and development of clinical outcome measures. Acad Emerg Med. 2000;7(4):397–401.CrossRefGoogle Scholar
  16. Tiao J, Feng R, Berger EM, Brandsema JF, Coughlin CC, Khan N, et al. Evaluation of the reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index and the Cutaneous Assessment Tool-Binary Method in juvenile dermatomyositis among paediatric dermatologists, rheumatologists and neurologists. Br J Dermatol. 2017a;177(4):1086–92.CrossRefGoogle Scholar
  17. Tiao J, Feng R, Bird S, Choi JK, Dunham J, George M, et al. The reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) among dermatologists, rheumatologists and neurologists. Br J Dermatol. 2017b;176(2):423–30.CrossRefGoogle Scholar
  18. Wolstencroft PW, Chung L, Li S, Casciola-Rosen L, Fiorentino DF. Factors associated with clinical remission of skin disease in dermatomyositis. JAMA Dermatol. 2018;154(1):44–51.CrossRefGoogle Scholar
  19. Yassaee M, Fiorentino D, Okawa J, Taylor L, Coley C, Troxel AB, et al. Modification of the cutaneous dermatomyositis disease area and severity index, an outcome instrument. Br J Dermatol. 2010;162(3):669–73.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of DermatologyCorporal Michael J. Crescenz VAMCPhiladelphiaUSA
  2. 2.Department of DermatologyUniversity of Pennsylvania, Perelman School of MedicinePhiladelphiaUSA
  3. 3.Rutgers Robert Wood Johnson Medical SchoolNew BrunswickUSA

Personalised recommendations