A Cross-sectional Comparison of Magnetic Resonance Imaging Findings and Clinical Assessment in Patients With Morphea | Dermatology | JAMA Dermatology | JAMA Network
[Skip to Navigation]
Sign In
Figure.  Clinical and Magnetic Resonance Imaging (MRI) Findings in Patients With Suspected Deep Morphea
Clinical and Magnetic Resonance Imaging (MRI) Findings in Patients With Suspected Deep Morphea

A, A clinical photograph of subtle morphea of the left thigh (red box demonstrates location of scan). B, An axial fat saturated T2 weighted image, showing significant and subclinical subcutaneous involvement of this morphea lesion after MRI. This underscores the potential utility of MRI in evaluation of morphea because making the distinction of morphea activity vs damage is critical for management and can be challenging to assess in deep lesions. C, A clinical photograph of morphea involving the right lower extremity of another patient (red box demonstrates location of scan). D, An axial 3-dimensional subtracted postcontrast image indicating MRI findings of fascial involvement, which can be difficult to determine on clinical examination alone. The pink arrows indicate fascial enhancement; the yellow arrows indicate normal fascia of the contralateral extremity.

Table.  Comparison of Clinical Assessment With Magnetic Resonance Imaging Findings
Comparison of Clinical Assessment With Magnetic Resonance Imaging Findings
1.
Klimas  NK, Shedd  AD, Bernstein  IH, Jacobe  H.  Health-related quality of life in morphoea.   Br J Dermatol. 2015;172(5):1329-1337. doi:10.1111/bjd.13572PubMedGoogle ScholarCrossref
2.
Laxer  RM, Zulian  F.  Localized scleroderma.   Curr Opin Rheumatol. 2006;18(6):606-613. doi:10.1097/01.bor.0000245727.40630.c3PubMedGoogle ScholarCrossref
3.
Arkachaisri  T, Pino  S.  Localized scleroderma severity index and global assessments: a pilot study of outcome instruments.   J Rheumatol. 2008;35(4):650-657.PubMedGoogle Scholar
4.
Eutsler  EP, Horton  DB, Epelman  M, Finkel  T, Averill  LW.  Musculoskeletal MRI findings of juvenile localized scleroderma.   Pediatr Radiol. 2017;47(4):442-449. doi:10.1007/s00247-016-3765-xPubMedGoogle ScholarCrossref
5.
Horger  M, Fierlbeck  G, Kuemmerle-Deschner  J,  et al.  MRI findings in deep and generalized morphea (localized scleroderma).   AJR Am J Roentgenol. 2008;190(1):32-39. doi:10.2214/AJR.07.2163PubMedGoogle ScholarCrossref
6.
Schanz  S, Fierlbeck  G, Ulmer  A,  et al.  Localized scleroderma: MR findings and clinical features.   Radiology. 2011;260(3):817-824. doi:10.1148/radiol.11102136PubMedGoogle ScholarCrossref
Limit 200 characters
Limit 25 characters
Conflicts of Interest Disclosure

Identify all potential conflicts of interest that might be relevant to your comment.

Conflicts of interest comprise financial interests, activities, and relationships within the past 3 years including but not limited to employment, affiliation, grants or funding, consultancies, honoraria or payment, speaker's bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued.

Err on the side of full disclosure.

If you have no conflicts of interest, check "No potential conflicts of interest" in the box below. The information will be posted with your response.

Not all submitted comments are published. Please see our commenting policy for details.

Limit 140 characters
Limit 3600 characters or approximately 600 words
    Research Letter
    April 1, 2020

    A Cross-sectional Comparison of Magnetic Resonance Imaging Findings and Clinical Assessment in Patients With Morphea

    Author Affiliations
    • 1Department of Dermatology, University of Texas Southwestern Medical Center, Dallas
    • 2Department of Radiology, University of Texas Southwestern Medical Center, Dallas
    • 3Department of Musculoskeletal Radiology, University of Washington, Seattle
    JAMA Dermatol. 2020;156(5):590-592. doi:10.1001/jamadermatol.2020.0036

    Morphea is an inflammatory disorder that produces sclerosis of the skin and subcutaneous tissues. Accurate assessment of disease depth, extent, and activity (inflammation) is critical for management but can be difficult to discern on clinical examination alone. This presents a challenge to care.1 The objective of the present study was to compare clinical assessment of deep morphea with magnetic resonance imaging (MRI) findings. We hypothesized that MRI would be a useful clinical adjunct, demonstrating subclinical morphea activity and expansion.

    Methods

    This was a cross-sectional analysis of 20 consecutive patients from the Morphea in Adults and Children cohort with suspected deep morphea, defined as deeply sclerotic, poorly circumscribed plaques, indicating possible involvement of soft tissue (Figure). The University of Texas Southwestern Institutional Review Board approved this study, and written informed consent was obtained from all participants in the Morphea in Adults and Children cohort on inclusion.

    Patients were assigned a morphea subtype and given a clinical assessment score by a single dermatologist (H.T.J.) using the validated Localized Scleroderma Assessment Tool (including modified Localized Skin Severity Index [mLoSSI], Physician Global Assessment of Disease Activity [PGA-A], Localized Scleroderma Damage Index, and Physician Global Assessment of Disease Damage).2,3 Pain and function were assessed using validated questionnaires and clinical examination. The clinical margins of the lesion were marked to be visible on imaging. Subsequently, patients underwent high-resolution MRI using a dedicated musculoskeletal protocol. Two musculoskeletal radiologists (P.P. and M.C.) blinded to the clinical assessment reviewed the images independently for lesion depth, extent, and activity (inflammation), resolving discrepancies by discussion with a senior musculoskeletal radiologist (A.C.).

    Sample size calculations were not performed for this pilot study. Fisher exact tests, Wilcoxon-Mann-Whitney tests, and Cohen κ tests were used when appropriate. Two-tailed P values were used, and P values less than .05 were considered significant. All analyses were performed with SAS, version 9.4 (SAS Institute Inc).

    Results

    Of the 20 patients included in this analysis, 13 patients (65%) were white, 15 (75%) were women, and 14 (70%) had linear morphea. There were a total of 27 MRI examinations. At time of examination, 12 patients (44%) were receiving methotrexate therapy, 3 (11%) were receiving mycophenolate mofetil, 1 (4%) was receiving topical triamcinolone, and 11 (41%) underwent no therapy. Findings from MRI confirmed clinical suspicion of soft tissue involvement in 26 of 27 scans. A total of 7 scans showed involvement of muscle and/or deep fascia. Induration, a component of the Localized Scleroderma Assessment Tool score, was present more often in those patients with fascia/muscle involvement (6 of 7 MRI scans [86%], vs 5 of 20 [25%]; P = .005), and they had higher PGA-A scores (median [interquartile range], 37 [22-48] vs 0 [0-23]; P = .02).

    Clinically active disease was suspected in 15 patients, while MRI results demonstrated active disease in 17 cases. In 5 patients, MRI findings showed disease activity where there was none apparent clinically. Patients with active disease seen on MRI results had higher clinical disease activity scores using the PGA-A (median [interquartile range], 25 [2-37] vs 0 [0-2]; P = .007) but not with the mLoSSI. Details of the comparison of clinical evaluation with MRI assessment are summarized in the Table. Results from the MRI showed that lesions extended beyond clinical margins by a mean of 6.3 cm. Cohen κ ranged from 0.780 to 1.000, indicating substantial to near-perfect agreement.

    Discussion

    This cross-sectional analysis compared the clinical assessment of morphea with MRI evaluation. The findings corroborate those of previous retrospective studies, which showed that MRI results reveal clinically occult musculoskeletal involvement.4-6 We also found that clinical examination alone underestimated lesion activity and extent. Specifically, the validated clinical parameters used to determine activity as part of the mLoSSI score did not correspond to features of activity on MRI findings, and in 5 patients (19%) MRI results demonstrated occult active disease. These discrepancies highlight potential misclassification of lesions as inactive when using the mLoSSI alone, particularly in deep morphea, in which activity is difficult to detect. Interestingly, palpable peripheral induration, a component of the mLoSSI, best reflected inflammation in MRI results, which suggests induration is a sign of activity in deeper tissues where erythema is not visible. We also observed subclinical extension of lesions on MRI results, which indicates that pathology extends beyond clinical margins. The presence of activity on MRI results changed management with continuation or escalation of treatment and subsequent control of activity.

    This analysis was limited by its small size and lack of reference standard. However, the results underscore the potential utility of MRI as an adjunct to clinical examination in morphea. Further studies are needed to define indications for MRI.

    Back to top
    Article Information

    Accepted for Publication: February 4, 2020.

    Corresponding Author: Heidi T. Jacobe, MD, MSCS, Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390-9069 (heidi.jacobe@utsouthwestern.edu).

    Published Online: April 1, 2020. doi:10.1001/jamadermatol.2020.0036

    Author Contributions: Dr Jacobe had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

    Concept and design: Abbas, O’Brien, Goldman, Chalian, Chhabra, Jacobe.

    Acquisition, analysis, or interpretation of data: All authors.

    Drafting of the manuscript: Abbas, Goldman, Chalian, Chhabra.

    Critical revision of the manuscript for important intellectual content: Abbas, O’Brien, Pezeshk, Chalian, Chhabra, Jacobe.

    Statistical analysis: Abbas, Goldman, Jacobe.

    Administrative, technical, or material support: All authors.

    Supervision: Chalian, Chhabra, Jacobe.

    Conflict of Interest Disclosures: Dr Chhabra reported personal fees from ICON Medical and Treace Medical Concepts for unrelated consultant work and book royalties from Jaypee Brothers Medical Publishers and Wolters Kluwer outside the submitted work. No other disclosures were reported.

    Funding/Support: This research was supported by grant AR073516-01A1 from the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health.

    Role of the Funder/Sponsor: The funder had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

    Disclaimer: The content of this article is solely the responsibility of the authors and does not necessarily represent the official views of the University of Texas Southwestern Medical Center at Dallas and its affiliated academic and health care centers, the National Center for Research Resources, and the National Institutes of Health.

    Additional Contributions: We thank Robert N. Joodi, MD, previously affiliated with University of Texas Southwestern Medical Center, Department of Radiology, currently in private practice in San Antonio, TX, for help in creation of figures. We also acknowledge Yin Xi, PhD, and Heng Chen, BS, both with the University of Texas Southwestern Medical Center, Department of Radiology, for their help with statistics, as well as Elaine Kunzler, MD, and Stephanie Pollack, MD, both with University of Texas Southwestern Medical Center, Department of Dermatology, for their contributions. None of the contributors was compensated for their contributions.

    References
    1.
    Klimas  NK, Shedd  AD, Bernstein  IH, Jacobe  H.  Health-related quality of life in morphoea.   Br J Dermatol. 2015;172(5):1329-1337. doi:10.1111/bjd.13572PubMedGoogle ScholarCrossref
    2.
    Laxer  RM, Zulian  F.  Localized scleroderma.   Curr Opin Rheumatol. 2006;18(6):606-613. doi:10.1097/01.bor.0000245727.40630.c3PubMedGoogle ScholarCrossref
    3.
    Arkachaisri  T, Pino  S.  Localized scleroderma severity index and global assessments: a pilot study of outcome instruments.   J Rheumatol. 2008;35(4):650-657.PubMedGoogle Scholar
    4.
    Eutsler  EP, Horton  DB, Epelman  M, Finkel  T, Averill  LW.  Musculoskeletal MRI findings of juvenile localized scleroderma.   Pediatr Radiol. 2017;47(4):442-449. doi:10.1007/s00247-016-3765-xPubMedGoogle ScholarCrossref
    5.
    Horger  M, Fierlbeck  G, Kuemmerle-Deschner  J,  et al.  MRI findings in deep and generalized morphea (localized scleroderma).   AJR Am J Roentgenol. 2008;190(1):32-39. doi:10.2214/AJR.07.2163PubMedGoogle ScholarCrossref
    6.
    Schanz  S, Fierlbeck  G, Ulmer  A,  et al.  Localized scleroderma: MR findings and clinical features.   Radiology. 2011;260(3):817-824. doi:10.1148/radiol.11102136PubMedGoogle ScholarCrossref
    ×