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Rheumatology Advance Access originally published online on September 20, 2005
Rheumatology 2006 45(1):76-78; doi:10.1093/rheumatology/kei106
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Healthy children have a significantly increased skin score assessed with the modified Rodnan skin score

I. Foeldvari and A. Wierk

Pediatric Rheumatologic Clinic, Allgemeines Krankenhaus Eilbek, Hamburg, Germany.

Correspondence to: I. Foeldvari, Pediatric Rheumatologic Clinic, Allgemeines Krankenhaus Eilbek, Friedrichsbergerstr. 60, 22081 Hamburg, Germany. E-mail: Sprechstunde{at}kinderrheumatologie.de


   Abstract
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 Abstract
Introduction
 Methods
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Objectives. The modified Rodnan skin score (MRSS) is used as a primary outcome measure in most therapeutic trials in systemic sclerosis (SSc) in adults. Before we can apply this outcome measure in trials in juvenile patients with SSc, we need to evaluate this assessment method in children without sclerodermatous skin changes, to establish values for the normal paediatric population.

Methods. To determine the MRSS in healthy paediatric population, patients of the paediatric rheumatology out-patient clinic with mechanical pain or with juvenile idiopathic arthritis at the age of 16 yr or under were assessed between 1 January and 31 March 2004. Patients with any sign of connective tissue disease or skin disorders, such as psoriasis or ectopic dermatitis, were excluded. The MRSS was determined at a standardized location and with a standardized pinching method.

Results. Two hundred and seventeen patients, including 100 females, were assessed. The mean age of the patients was 10.5 yr (2.9–16), the mean body mass index (BMI) was 18.3 (9.3–35.7), and the mean MRSS was 13.92 (range 4–25). The MRSS score showed a difference between males and females at every Tanner stage. There was a linear correlation between MRSS and body mass index independently of age and Tanner stage.

Conclusion. The mean MRSS in healthy children is 13.92 units and this range would be expected in a patient with a diffuse form of SSc. The MRSS score in children correlates with the body mass index and the Tanner stage, so it should be corrected to these parameters, according to this pilot study.

KEY WORDS: Modified Rodnan skin score, Children, Juvenile systemic sclerosis, Systemic sclerosis


   Introduction
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 Abstract
 Introduction
Methods
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The modified Rodnan skin score (MRSS) [1, 2] is used as a primary outcome measure in most of the therapeutic trials in systemic sclerosis (SSc) in adults. It is recommended as the core assessment technique for all international descriptive investigations in systemic sclerosis [3] and it shows correlation with survival [4]. It is a simple bedside examination, and is cost-effective in measuring skin thickening (but not tethering). It is already validated in an adult population with SSc for face, content, criterion, discrimination and construct validity, and has shown good feasibility [5] despite significant interobserver variability [6]. MRSS does not discriminate between the contributions of fibrosis, oedema and inflammation.

There are currently no data regarding the MRSS in paediatric patients with SSc. Before we can use MRSS in patients with disease involvement, it is interesting to see how this measure performs in a healthy population, especially because a part of the skin in patients with juvenile SSc is unaffected, and the site where unaffected skin stops and affected skin begins is often difficult to determine. Children are not just small adults and during childhood development they have natural changes in skin thickening [7], which may influence the MRSS. To assess the applicability of MRSS in children, we conducted a study of MRSS in a healthy control population.


   Methods
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 Abstract
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 Methods
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To determine the MRSS in a healthy paediatric population, patients of the out-patient clinic with mechanical pain (non-inflammatory joint pain) or with juvenile idiopathic arthritis (JIA) at the age of 16 yr or under were assessed by a single trained observer between 1 January and 31 March 2004. Patients with any sign of connective tissue disease other than scleroderma, or skin disorders, which could influence the MRSS, such as psoriasis, ectopic dermatitis or vasculitic changes associated with systemic JIA, were excluded. The MRSS was determined at a standardized location of the 17 areas of the body with a standardized pinching method and was scored from 0 to 3 [3].

All patients and or their parents, depending on the patient's age, gave informed consent to participation in the study.


   Results
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 Abstract
 Introduction
 Methods
 Results
Discussion
 References
 
Two hundred and seventeen patients with mechanical pain or JIA, including 100 females, were assessed. The mean age of the patients was 10.5 yr (2.9–16), the mean body mass index (BMI) was 18.3 (9.3–35.7), and the mean MRSS was 13.92 (range 4–25). There was mostly a linear correlation between MRSS and BMI (Fig. 1). We looked at the correlation of the mean value of MRSS with the mean value of the BMI in males and females, and there was no difference (Fig. 2). We then analysed the data regarding the mean MRSS score for females and males. The mean score for MRSS in males was 12.89 compared with 15.11 in females, a significant difference (Fig. 2). There was a lower MRSS score in males than in females when each Tanner stage was compared (Fig. 3); this difference was significant for Tanner stage 1 and 4. None of the single-area MRSS scores was higher than 2.



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  		FIG. 1. Correlation of the mean body mass index (BMI) with the mean modified Rodnan skin score and age.

 


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  		FIG. 2. Correlation of the mean body mass index (BMI) with the sex of the healthy controls and the mean Modified Rodnan skin score (MRSS) with the sex of healthy controls. On the y-axis is the value of BMI/MRSS; the number at the top of the column shows mean BMI or MRSS. The P value under the columns shows the difference between the female and male groups.

 


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  		FIG. 3. Correlation of the Tanner stage with the mean modified Rodnan skin score for males and females. The P values under the columns show the difference between the female and male groups.

 

   Discussion
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 Abstract
 Introduction
 Methods
 Results
 Discussion
References
 
In healthy children—those without sclerodermatous skin changes—the mean MRSS is 13.92 and this range would be expected in a patient with mild diffuse involvement of SSc. There was a significant difference in the mean MRSS score in males and females, which would reflect the lower amount of subcutaneous fat in males. The MRSS score seems to correlate to the Tanner stage. None of the single-area MRSS scores was higher than 2. In the involved areas we would also expect scores of 3, so the current application of MRSS in children would differentiate between severe sclerodermatous changes, MRSS score 3 and areas with less or no involvement. Children without SSc achieve MRSS scores of 1 and 2 because of the ‘Natural thickening of the skin’, explained by the increased subcutaneous fat tissue compared to adults [7]. The natural subcutaneous thickening of the skin seems to change with sexual maturation. This is reflected in the tendency for changes to occur with changing Tanner stage, which reflects sexual maturation; especially in the male children this seems to lead to more pronounced changes. The MRSS seems to correlate to BMI; the less impressive correlation after the age of 13 could be explained, by the smaller group of patients, who present the mean value.

In an adult-limited SSc cohort, the mean MRSS score was 6.6 ± 4.0 units and in the diffuse SSc cohort it was 19.1 ± 8.6 units [8]; in another adult cohort with diffuse SSc with a disease course shorter than 3 yr, the mean initial MRSS score was around 23 [4]. In our healthy children the mean score was 13.92, which would be a significantly increased MRSS even for an adult with SSc.

Currently, the MRSS for paediatric patients fulfils only part of the filter criteria for the Outcome Measures in Rheumatology (OMERACT) [9], such as face validity and accuracy; for the other criteria it needs more validation.

The MRSS score in children correlates with the BMI, so if MRSS is used in a paediatric trial the score should be corrected for the BMI for the same sex and age [10] and for the Tanner score, according to this pilot study. It would be reasonable to apply MRSS in a multinational approach in a cross-sectional juvenile SSc cohort to establish the validity of the MRSS after a training session for the participants to decrease the intraobserver variation.

The authors have declared no conflicts of interest.


   References
Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 References
 

  1. Clements PJ, Furst DE. Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 1993;20:1892–6.[Web of Science][Medline]
  2. Brennan P, Silman A, Black C et al. Reliability of skin involvement measures in scleroderma. The UK Scleroderma Study Group. Br J Rheumatol 1992;31:457–60.[Abstract/Free Full Text]
  3. Akesson A, Fiori G, Krieg T et al. Assessment of skin, joint, tendon and muscle involvement. Clin Exp Rheumatol 2003;21(Suppl. 29):S5–8.
  4. Steen VD, Medsger TA Jr. Improvement in skin thickening in systemic sclerosis associated with improved survival. Arthritis Rheum 2001;44:2828–35.[CrossRef][Web of Science][Medline]
  5. Merkel PA, Clements PJ, Reveille JD et al. Current status of outcome measure development for clinical trials in systemic sclerosis. Report from OMERACT 6. J Rheumatol 2003;30:1630–47.[Abstract/Free Full Text]
  6. Clements P, Lachenbruch P, Siebold J et al. Inter and intraobserver variability of total skin score thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 1995;22:1281–5.[Web of Science][Medline]
  7. de Rigal J, Escoffier C, Querleux B et al. Assessment of aging of the human skin by in vivo ultrasound imaging. J Invest Dermatol 1989;93:621–25.[CrossRef][Web of Science][Medline]
  8. Sato S, Hasegawa M, Takehara K. Serum levels of interleukin-6 and interleukin-10 correlate with total skin thickness score in patients with systemic sclerosis. J Dermatol Sci 2001;27:140–6.[CrossRef][Web of Science][Medline]
  9. Boers M, Brooks P, Strand CV et al. The Omeract filter for outcome measures in rheumatology. J Rheumatol 1998;25:198–9.[Web of Science][Medline]
  10. Perrin EM, Flower KB, Ammerman AS. Body mass index charts: useful yet underused. J Pediatr 2004;144:455–60.[CrossRef][Web of Science][Medline]
Submitted 6 March 2005; revised version accepted 9 August 2005.
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