Cutaneous Sarcoidosis Outcome Instruments: Reliability and Validity Among Dermatologists, Pulmonologists, and Rheumatologists

  • William FixEmail author
  • Misha Rosenbach
Living reference work entry


Valid and reliable cutaneous sarcoidosis outcome instruments are required for the rigorous clinical research that will lead to evidence-based treatments for cutaneous sarcoidosis. Cutaneous sarcoidosis outcome instruments may provide an accessible, low-cost, low-risk tool to measure disease activity and should be accessible and comprehensible to non-dermatologist investigators. The sarcoidosis activity and severity index (SASI) and the cutaneous sarcoidosis activity and morphology instrument (CSAMI) both demonstrate validity and reliability among dermatologists and non-dermatologists alike. The CSAMI correlates strongly with patient-reported quality of life (QOL) metrics, as well as histologic measures of disease activity. The SASI is a simpler tool initially developed for the assessment of facial sarcoidosis, which has been successfully utilized as an outcome instrument in clinical trials. Both of these instruments demonstrated markedly decreased reliability when tested using store-and-forward teledermatology technology. High-frequency ultrasonography (HFU) is an emerging technology that could provide a valid, reliable, and objective measure of cutaneous sarcoidosis activity. Further research is needed to establish standardized imaging protocols and confirm the results of existing studies.


Cutaneous Sarcoidosis Outcome Instruments SASI CSAMI High-frequency ultrasound Reliability Validity 


  1. Baughman RP, Judson MA, Teirstein A, Lower EE, Lo K, Schlenker-Herceg R, Barnathan ES. Chronic facial sarcoidosis including lupus pernio. Am J Clin Dermatol. 2008;9(3):155–61.CrossRefGoogle Scholar
  2. Baughman RP, Judson MA, Ingledue R, Craft NL, Lower EE. Efficacy and safety of apremilast in chronic cutaneous sarcoidosis. Arch Dermatol. 2012;148(2):262–4.CrossRefGoogle Scholar
  3. Berg SA, Yeung H, English JC, Keimig EL, Kim EJ, Micheletti RG, Wanat KA, Judson MA, Baughman RP, Rosenbach M. Inter-rater reliability of cutaneous sarcoidosis assessment tools via remote photographic assessment. Sarcoidosis Vasc Diffuse Lung Dis. 2016;33(3):315–29.Google Scholar
  4. Drake WP, Oswald-Richter K, Richmond BW, Ison J, Burke VE, Algood H, Braun N, Taylor T, Pandit KV, Aboud C, Yu C, Kaminski N, Boyd AS, King LE. Oral antimycobacterial therapy in chronic cutaneous sarcoidosis a randomized, single-masked, placebo-controlled study. JAMA Dermatol. 2013;149(9):1040–9.CrossRefGoogle Scholar
  5. Droicourt C, Rybojad M, Porcher R, Julliard C, Cosnes A, Joly P, Lacour J, D’Incan M, Dupin N, Sassolas B, Laurent M, Chevrant-Breton J, Lebrun-Vignes B, Dessaux K, Valeyre D, Revux J, Tazi A, Chosidow O, Dupuy A. A randomized, investigator-masked, double-blind, placebo-controlled trial on alidomide in severe cutaneous sarcoidosis. Chest. 2014;146(4):1046–54.CrossRefGoogle Scholar
  6. Haimovic A, Sanchez MD, Judson MA, Prystowsky S. Sarcoidosis: a comprehensive review and update for the dermatologist. J Am Acad Dermaol. 2012;66(5):699.e1.Google Scholar
  7. Honari G. Clinical scoring of atopic dermatitis. In: Humbert P, et al., editors. Measuring the skin. Cham: Springer International Publishing; 2015.Google Scholar
  8. Judson MA, Baughman RP, Costabel U, Flavin S, Lo KH, Kavuru MS, Drent M. Efficacy of infliximab in extrapulmonary sarcoidosis: results from a randomized trial. Eur Respir J. 2008;31:1189–96.CrossRefGoogle Scholar
  9. Judson MA, Baughman RP, Costabel U, Drent M, Gibson KF, Raghu F, Shigemitsu H, Barney JB, Culver DA, Hamzeh NY, Wijsenbeek MS, Albera C, Huizar I, Agarwal P, Brodmerkel C, Watt R, Barnathan ES. Safety and efficacy of ustekinumab or golimumab in patients with chronic sarcoidosis. Eur Respir J. 2014;44:1296–307.CrossRefGoogle Scholar
  10. Lacarrubba F, Pellacani G, Gurgone S, Verzi AE, Micali G. Advances in non-invasive techniques as aids to the diagnosis and monitoring of therapeutic response in plaque psoriasis: a review. Int J Dermatol. 2015;54:626–34.CrossRefGoogle Scholar
  11. Li Y, Berenji GR. Cutaneous sarcoidosis evaluated by FDG PET. Clin Nucl Med. 2011;36(7): 584–6.CrossRefGoogle Scholar
  12. Mlosek RK, Malinowska S. Ultrasound image of the skin, apparatus, and imaging basics. J Ultrason. 2013;13:212–21.CrossRefGoogle Scholar
  13. Noe MH, Rodriguez O, Taylor L, Schultz S, Gelfand JM, Judson MA, Rosenbach M. High frequency ultrasound: a novel measure of granuloma burden in cutaneous sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2018;34(2):136–141.Google Scholar
  14. Pariser RJ, Paul J, Hirano S, Torosky C, Smith M. A double-blind, randomized, placebo-controlled trial of adalimumab in the treatment of cutaneous sarcoidosis. J Am Acad Dermatol. 2012;68(5):765–73.CrossRefGoogle Scholar
  15. Pascoe VL, Enamandram M, Corey KC, Cheng CE, Javorsky EJ, Sung SM, Donahue KR, Kimball AB. Using the physician global assessment in a clinical setting to measure and track patient outcomes. JAMA Dermatol. 2015;151(4):375–81.CrossRefGoogle Scholar
  16. Rosenbach M, Murrell DF, Bystryn J, Dulay S, Dick S, Farkharzadeh S, Hall R, Korman NJ, Lin J, Okawa J, Pandya AG, Payne AS, Rose M, Rubenstein D, Woodley D, Vittorio C, Werth BB, Williams EA, Taylor L, Troxel AB, Werth VP. Reliability and convergent validity of two outcome instruments for pemphigus. J Invest Dermatol. 2009;129:2404–10.CrossRefGoogle Scholar
  17. Rosenbach M, Yeung H, Chu EY, Kim EJ, Payne AS, Takeshita J, Vittorio CC, Wanat KA, Werth VP, Gelfand JM. Reliability and convergent validity of the cutaneous sarcoidosis activity and morphology instrument for assessing cutaneous sarcoidosis. JAMA Dermatol. 2013;149(5): 550–6.CrossRefGoogle Scholar
  18. Stokkel MPM, van der Hiel B, Olmos RV, Hoefnagel K. Fluorodeoxyglucose PET/CT in subcutaneous sarcoidosis mimicking cutaneous lymphoma. Eur J Nucl Med Mol Imaging. 2012;39:919–20.CrossRefGoogle Scholar
  19. Wanat KA, Rosenbach M. Cutaneous sarcoidosis. Clin Chest Med. 2015;36:685–702.CrossRefGoogle Scholar
  20. Yeung H, Farber S, Birnbaum BK, Dunham J, Ogdie A, Patterson KC, Payne AS, Porteous MK, Rossman MD, Sharim R, Takeshita J, Werth VP, Shin DB, Price S, Rosenbach M. Reliability and validity of cutaneous sarcoidosis outcome instruments among dermatologists, pulmonologists, and rheumatologists. JAMA Dermatol. 2015;151(12):1317–1322.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA
  2. 2.Department of DermatologyPerelman School of Medicine, University of PennsylvaniaPhiladelphiaUSA

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