Skip to main content
Top
Published in: Current Rheumatology Reports 2/2013

01-02-2013 | SCLERODERMA (J VARGA, SECTION EDITOR)

Recent Developments in Diagnosis and Assessment of Morphea

Authors: Sarah Nouri, Heidi Jacobe

Published in: Current Rheumatology Reports | Issue 2/2013

Login to get access

Abstract

There is huge variation in the evaluation, diagnosis, and treatment of patients with morphea (localized scleroderma). In part, this variability results from the lack of validated methods to assess severity and outcomes with a consequent lack of adequate therapeutic trials. Evaluation is also hindered by lack of information regarding the impact of morphea on patients. Recent studies are addressing this gap in knowledge and include: development of clinical outcome measures, validation of imaging studies, publication of consensus treatment plans, and increased understanding of the impact of morphea on patients and parents. The purpose of this review is to summarize the results of these studies and to synthesize the information into a rational approach to the diagnosis and assessment of patients with morphea.
Literature
1.
go back to reference • Johnson W, Jacobe H: Morphea in adults and children (MAC) cohort II: Patients with morphea experience delay in diagnosis and large variation in treatment. J Am Acad Dermatol 2012 Feb 28. [Epub ahead of print]. This cross sectional survey of patients enrolled in the Morphea in Adults and Children Cohort underscores often significant delays in diagnosis and treatment of patients with morphea. Further, treatments prescribed for patients varied largely dependent on the specialty of treating physician. The results of this study support the need for treatment plans accepted across specialties. • Johnson W, Jacobe H: Morphea in adults and children (MAC) cohort II: Patients with morphea experience delay in diagnosis and large variation in treatment. J Am Acad Dermatol 2012 Feb 28. [Epub ahead of print]. This cross sectional survey of patients enrolled in the Morphea in Adults and Children Cohort underscores often significant delays in diagnosis and treatment of patients with morphea. Further, treatments prescribed for patients varied largely dependent on the specialty of treating physician. The results of this study support the need for treatment plans accepted across specialties.
2.
go back to reference Weibel L, Laguda B, Atherton D, Harper JI. Misdiagnosis and delay in referral of children with localized scleroderma. Br J Dermatol. 2011;165:1308–13.PubMedCrossRef Weibel L, Laguda B, Atherton D, Harper JI. Misdiagnosis and delay in referral of children with localized scleroderma. Br J Dermatol. 2011;165:1308–13.PubMedCrossRef
3.
go back to reference Li SC, Torok KS, Pope E, et al. Development of consensus treatment plans for juvenile localized scleroderma. Arthritis Care Res. 2012;64:1175–85. Li SC, Torok KS, Pope E, et al. Development of consensus treatment plans for juvenile localized scleroderma. Arthritis Care Res. 2012;64:1175–85.
4.
go back to reference Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614–20.CrossRef Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614–20.CrossRef
5.
go back to reference • Saxton-Daniels S, Jacobe HT: An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol 2010, 146:1044-5. This cross-sectional survey of adults with pediatric onset morphea in the Morphea in Adults and Children Cohort is one of the first to report continued episodes of active morphea well into adulthood of these patients. The study also reports significant impact on life quality in these patients which was associated with the number of lesions and presence of functional impairment. These findings indicate children with morphea will require close follow-up into adulthood. PubMedCrossRef • Saxton-Daniels S, Jacobe HT: An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol 2010, 146:1044-5. This cross-sectional survey of adults with pediatric onset morphea in the Morphea in Adults and Children Cohort is one of the first to report continued episodes of active morphea well into adulthood of these patients. The study also reports significant impact on life quality in these patients which was associated with the number of lesions and presence of functional impairment. These findings indicate children with morphea will require close follow-up into adulthood. PubMedCrossRef
7.
go back to reference Skobieranda K, Helm KF. Decreased expression of the human progenitor cell antigen (CD34) in morphea. Am J Dermatopathol. 1995;17:471–5.PubMedCrossRef Skobieranda K, Helm KF. Decreased expression of the human progenitor cell antigen (CD34) in morphea. Am J Dermatopathol. 1995;17:471–5.PubMedCrossRef
8.
go back to reference McNiff JM, Glusac EJ, Lazova RZ, Carroll CB. Morphea limited to the superficial reticular dermis: an underrecognized histologic phenomenon. Am J Dermatopathol. 1999;21:315–9.PubMedCrossRef McNiff JM, Glusac EJ, Lazova RZ, Carroll CB. Morphea limited to the superficial reticular dermis: an underrecognized histologic phenomenon. Am J Dermatopathol. 1999;21:315–9.PubMedCrossRef
9.
go back to reference Sung JJ, Chen TS, Gilliam AC, et al. Clinicohistopathological correlations in juvenile localized scleroderma: studies on a subset of children with hypopigmented juvenile localized scleroderma due to loss of epidermal melanocytes. J Am Acad Dermatol. 2011;65:364–73.PubMedCrossRef Sung JJ, Chen TS, Gilliam AC, et al. Clinicohistopathological correlations in juvenile localized scleroderma: studies on a subset of children with hypopigmented juvenile localized scleroderma due to loss of epidermal melanocytes. J Am Acad Dermatol. 2011;65:364–73.PubMedCrossRef
10.
go back to reference Stewart L, Nuara A, Anand D, et al. Perifollicular papules and hyperkeratotic plaques on the back in a blaschkoid distribution. Morphea with features of lichen sclerosus et atrophicus (LS). Arch Dermatol. 2011;147:857–62.PubMedCrossRef Stewart L, Nuara A, Anand D, et al. Perifollicular papules and hyperkeratotic plaques on the back in a blaschkoid distribution. Morphea with features of lichen sclerosus et atrophicus (LS). Arch Dermatol. 2011;147:857–62.PubMedCrossRef
11.
go back to reference Sherber NS, Boin F, Hummers LK, Wigley FM. The "tank top sign": a unique pattern of skin fibrosis seen in pansclerotic morphea. Ann Rheum Dis. 2009;68:1511–2.PubMedCrossRef Sherber NS, Boin F, Hummers LK, Wigley FM. The "tank top sign": a unique pattern of skin fibrosis seen in pansclerotic morphea. Ann Rheum Dis. 2009;68:1511–2.PubMedCrossRef
12.
go back to reference Li SC, Liebling MS. The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep. 2009;11:205–11.PubMedCrossRef Li SC, Liebling MS. The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep. 2009;11:205–11.PubMedCrossRef
13.
go back to reference Bendeck SE, Jacobe HT. Ultrasound as an outcome measure to assess disease activity in disorders of skin thickening: an example of the use of radiologic techniques to assess skin disease. Dermatol Ther. 2007;20:86–92.PubMedCrossRef Bendeck SE, Jacobe HT. Ultrasound as an outcome measure to assess disease activity in disorders of skin thickening: an example of the use of radiologic techniques to assess skin disease. Dermatol Ther. 2007;20:86–92.PubMedCrossRef
14.
go back to reference Lott JP, Girardi M. Practice gaps. The hard task of measuring cutaneous fibrosis: comment on "14-MHz ultrasonography as an outcome measure in morphea (localized scleroderma)". Arch Dermatol. 2011;147:1115–6.PubMedCrossRef Lott JP, Girardi M. Practice gaps. The hard task of measuring cutaneous fibrosis: comment on "14-MHz ultrasonography as an outcome measure in morphea (localized scleroderma)". Arch Dermatol. 2011;147:1115–6.PubMedCrossRef
15.
go back to reference Nezafati KA, Cayce RL, Susa JS, et al. 14-MHz ultrasonography as an outcome measure in morphea (localized scleroderma). Arch Dermatol. 2011;147:1112–5.PubMedCrossRef Nezafati KA, Cayce RL, Susa JS, et al. 14-MHz ultrasonography as an outcome measure in morphea (localized scleroderma). Arch Dermatol. 2011;147:1112–5.PubMedCrossRef
16.
go back to reference •• Wortsman X, Wortsman J, Sazunic I, Carreno L: Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol 2011, 65:942-8. This study reports ultrasound features associated with disease activity in morphea. This is helpful reading when considering referring patients for ultrasound evaluation, as the radiologists performing the scan should be familiar with these changes. PubMedCrossRef •• Wortsman X, Wortsman J, Sazunic I, Carreno L: Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol 2011, 65:942-8. This study reports ultrasound features associated with disease activity in morphea. This is helpful reading when considering referring patients for ultrasound evaluation, as the radiologists performing the scan should be familiar with these changes. PubMedCrossRef
17.
go back to reference Li SC, Liebling MS, Ramji FG, et al. Sonographic evaluation of pediatric localized scleroderma: preliminary disease assessment measures. Pediatr Rheumatol Online J. 2010;8:14.PubMedCrossRef Li SC, Liebling MS, Ramji FG, et al. Sonographic evaluation of pediatric localized scleroderma: preliminary disease assessment measures. Pediatr Rheumatol Online J. 2010;8:14.PubMedCrossRef
18.
go back to reference Li SC, Liebling MS, Haines KA, et al. Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma. Arthritis Care Res (Hoboken). 2011;63:735–42.CrossRef Li SC, Liebling MS, Haines KA, et al. Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma. Arthritis Care Res (Hoboken). 2011;63:735–42.CrossRef
19.
go back to reference Horger M, Fierlbeck G, Kuemmerle-Deschner J, et al. MRI findings in deep and generalized morphea (localized scleroderma). AJR Am J Roentgenol. 2008;190:32–9.PubMedCrossRef Horger M, Fierlbeck G, Kuemmerle-Deschner J, et al. MRI findings in deep and generalized morphea (localized scleroderma). AJR Am J Roentgenol. 2008;190:32–9.PubMedCrossRef
20.
go back to reference •• Schanz S, Fierlbeck G, Ulmer A, et al.: Localized scleroderma: MR findings and clinical features. Radiology 2011, 260:817-24. This cross-sectional study of patients with morphea documents the typical finding on MR imaging of morphea lesions with suspected musculoskeletal involvement. Surprisingly, many patients who did not have any symptoms or physical examination findings suggestive of musculoskeletal involvement had MRI changes suggestive of involvement. The clinical significance of these occult changes is uncertain, but the results demonstrate the utility of MRI when musculoskeletal involvement is suspected. PubMedCrossRef •• Schanz S, Fierlbeck G, Ulmer A, et al.: Localized scleroderma: MR findings and clinical features. Radiology 2011, 260:817-24. This cross-sectional study of patients with morphea documents the typical finding on MR imaging of morphea lesions with suspected musculoskeletal involvement. Surprisingly, many patients who did not have any symptoms or physical examination findings suggestive of musculoskeletal involvement had MRI changes suggestive of involvement. The clinical significance of these occult changes is uncertain, but the results demonstrate the utility of MRI when musculoskeletal involvement is suspected. PubMedCrossRef
21.
go back to reference Arkachaisri T, Vilaiyuk S, Li S, et al. The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures. J Rheumatol. 2009;36:2819–29.PubMedCrossRef Arkachaisri T, Vilaiyuk S, Li S, et al. The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures. J Rheumatol. 2009;36:2819–29.PubMedCrossRef
22.
go back to reference Arkachaisri T, Vilaiyuk S, Torok KS, Medsger Jr TA. Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study. Rheumatology (Oxford). 2010;49:373–81.CrossRef Arkachaisri T, Vilaiyuk S, Torok KS, Medsger Jr TA. Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study. Rheumatology (Oxford). 2010;49:373–81.CrossRef
23.
go back to reference Jacobe H, Saxton-Daniels S: Morphea. Chapter 64. In: Fitzpatrick's Dermatology in General Medicine, 8e. Edited by Goldsmith, et al. New York: McGraw-Hill; 2012:692-701. Jacobe H, Saxton-Daniels S: Morphea. Chapter 64. In: Fitzpatrick's Dermatology in General Medicine, 8e. Edited by Goldsmith, et al. New York: McGraw-Hill; 2012:692-701.
24.
go back to reference Dharamsi J: Morphea in adults and children (MAC) cohort III: The prevalence and clinical significance of autoantibodies in morphea: A prospective case-control survey. Texas Dermatological Society annual meeting May 2012 Dharamsi J: Morphea in adults and children (MAC) cohort III: The prevalence and clinical significance of autoantibodies in morphea: A prospective case-control survey. Texas Dermatological Society annual meeting May 2012
25.
go back to reference Baildam EM, Ennis H, Foster HE, et al. Influence of childhood scleroderma on physical function and quality of life. J Rheumatol. 2011;38:167–73.PubMedCrossRef Baildam EM, Ennis H, Foster HE, et al. Influence of childhood scleroderma on physical function and quality of life. J Rheumatol. 2011;38:167–73.PubMedCrossRef
26.
go back to reference Carlomagno R, Russo G, Forni C, et al. Childhood morphea does not impair self-perception. Pediatr Rheumatol. 2011;9:78.CrossRef Carlomagno R, Russo G, Forni C, et al. Childhood morphea does not impair self-perception. Pediatr Rheumatol. 2011;9:78.CrossRef
27.
go back to reference Ennis H, Herrick AL, Baildam EM, Richards HL. Childrens' and parents' beliefs about childhood onset scleroderma are influenced by child age and physical function impairment. Rheumatology (Oxford). 2012;51:1331–3.CrossRef Ennis H, Herrick AL, Baildam EM, Richards HL. Childrens' and parents' beliefs about childhood onset scleroderma are influenced by child age and physical function impairment. Rheumatology (Oxford). 2012;51:1331–3.CrossRef
Metadata
Title
Recent Developments in Diagnosis and Assessment of Morphea
Authors
Sarah Nouri
Heidi Jacobe
Publication date
01-02-2013
Publisher
Current Science Inc.
Published in
Current Rheumatology Reports / Issue 2/2013
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-012-0308-9

Other articles of this Issue 2/2013

Current Rheumatology Reports 2/2013 Go to the issue

CRYSTAL ARTHRITIS (MH PILLINGER, SECTION EDITOR)

Racial and Gender Disparities Among Patients with Gout

Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.