Supplementary List S1:

List of full text scored papers (n=53; 26 for diagnosis assessment, 27 for therapy)

JLS assessment

1    Arkachaisri T, Vilaiyuk S, Li S, O'Neil KM, Pope E, Higgins GC, 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.

2    Arkachaisri T,Vilaiyuk S, Torok KS, Medsger TA Jr. 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;49:373–381.

3    Chiu YE, Vora S, Kwon EKM, Maheshwari M. A Significant Proportion of Children with Morphea En Coup De Sabre and Parry-Romberg Syndrome Have Neuroimaging Findings. Pediatr Dermatol 2012; 29:738–748.

4    Guariso G, Conte S, Galeazzi F,Vettorato MG, Martini G, Zulian F. Esophageal involvement in juvenile localized scleroderma: a pilot study. Clin Exp Rheumatol 2007;25:786-789.

5    Hedrich CM, Fiebig B, Hahn G, Suttorp M, Gahr M. Presentations and Treatment of Childhood Scleroderma: Localized Scleroderma, Eosinophilic Fasciitis, Systemic Sclerosis, and Graft-Versus-Host Disease. Clin Pediatr(Phila) 2011;50:604–614.

6    Kaliyadan F, Biswas K, Dharmaratnam AD. Progressive Facial Hemiatrophy – A Case Series. Indian J Dermatol 2011;56:557–560.

7    Kelsey CE, Torok KS. The Localized Scleroderma Cutaneous Assessment Tool: responsiveness to change in a pediatric clinical population. J Am Acad Dermatol 2013;69:214-20.

8    Li SC, Liebling MS, Haines KA. Ultrasonography is a sensitive tool for monitoring localized Scleroderma. Rheumatology 2007;46:1316–9.

9    Martini G, Murray KJ, Howell KJ, Harper J, Atherton D, Woo P, et al. Juvenile-onset localized scleroderma activity detection by infrared thermography. Rheumatology 2002;41:1178–82.

10  Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, Caputo R. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 2003;13:171-6.

11  Orozco-Covarrubias L, Guzmán-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R. Scleroderma ‘en coup de sabre’ and progressive facial emiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol2002;16:361–6.

12  Prinz JC, Kutasi Z, Weisenseel P, Poto L, Battyani Z, Ruzicka T. ‘‘Borrelia-associated early-onset morphea’’: A particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases. J Am Acad Dermatol 2009;60:248-55.

13  Schanz S, Henes J, Ulmer A, Kotter I, Fierlbeck G, Claussen CD, Horger M. Response Evaluation of Musculoskeletal Involvement in Patients With Deep Morphea Treated With Methotrexate and Prednisolone: A Combined MRI and Clinical Approach. AJR Am J Roentgenol 2013;200:W376-W382.

14  Schanz S, Fierlbeck G , Ulmer A, Schmalzing M, Kümmerle-Deschner J, Claussen CD, Horger M. Localized Scleroderma: MR Findings and Clinical Features. Radiology 2011;260:817-24.

15  Shaw LJ, Shipley J, Newell EL, Harris N, Clinch JG, Lovell CR. Scanning laser Doppler imaging may predict disease progression of localized scleroderma in children and young adults. Br J Dermatol 2013;169:152-5.

16  Shim WH, Jwa SW, Song M, Kim HS, Ko HC, Kim MB, Kim BS. Diagnostic usefulness of dermatoscopy in differentiating lichen sclerous et atrophicus from morphea. J Am Acad Dermatol 2012;66:690-1.

17  Sommer A, Gambichler T, Bacharach-Buhles M, Von Rothenburg T, Altmeyer P, Kreuter A. Clinical and serological characteristics of progressive facial hemiatrophy: A case series of 12 patients. J Am Acad Dermatol 2006;54:227-33.

18  Szymanska E, Nowicki A, Mlosek K, Litniewski J, Lewandowski M, Secomski W, Tymkiewicz R. Skin imaging with high frequency ultrasound — preliminary results. Eur J of Ultrasound 2000;12(1):9–16.

19  Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-63.

20  Trainito S, Favero L, Martini G, Pedersen TK, Favero V, Herlin T, Zulian F. Odontostomatologic involvement in juvenile localized scleroderma of the face. J Paediatr Child Health 2012;48:572–6.

21  L Weibel, Howell KJ, Visentin MT, Rudiger A, Denton CP, Zulian F, et al. Laser Doppler Flowmetry for Assessing Localized Scleroderma in Children. Arthritis Rheum 2007;56:3489–95.

22  Wortsman X, Wortsman J, Sazunic I, L Carreno L. Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol 2011;65:942-8.

23  Zannin ME, Martini G, Athreya BH, Russo R, Higgins GC, Vittadello F, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol 2007;91(10):1311–1314.

24  Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J, et al. Localized Scleroderma in childhood is not just a skin disease. Arthritis Rheum 2005;52:2873–81.

25  Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology 2006;45:614–620.

26  Zulian F, Meneghesso D, Grisan E, Vittadello F, Belloni Fortina A, Pigozzi B, et al. A new computerized method for the assessment of skin lesions in localized scleroderma. Rheumatology 2007;46:856–60.

JLS treatment

27  Cox D, O' Regan G, Collins S, Byrne A, Irvine A, Watson R.Juvenile localised scleroderma: a retrospective review of response to systemic treatment. Ir J Med Sci 2008;177:343–6.

28  Cunningham BB, Landells ID, Langman C, Sailer DE, Paller AS. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol 1998;39:211-5.

29  De Rie MA, Enomoto DN, De Vries HJ, Bos JD. Evaluation of Medium-Dose UVA1 Phototherapy in Localized Scleroderma with the Cutometer and Fast Fourier Transform Method. Dermatology 2003;207:298–301.

30  Dytoc M, Ting PT, Man J, Sawyer D, Fiorillo L. First case series on the use of imiquimod for morphoea. Br J Dermatol 2005;153:815–20.

31  Dytoc MT, Kossintseva I, Ting PT. First case series on the use of calcipotriol–betamethasone dipropionate for morphoea. Br J Dermatol 2007;157:609–35.

32  El-Mofty M, Mostafa W, El-Darouty M, Bosseila M, Nada H, Yousef R, et al. Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis. A clinico-pathologic study. Photodermatol PhotoimmunolPhotomed 2004;20:148–156.

33  Elst EF, Van Suijlekom-Smit LW, Oranje AP. Treatment of Linear Scleroderma with Oral 1,25-Dihydroxyvitamin D3 (Calcitriol) in Seven Children. Pediatr Dermatol 1999;16:53-8.

34  Kerscher M, Volkenandt M, Gruss C, Reuther T, von Kobyletzki G, Freitag M, et al. Low-dose UVA1 phototherapy for treatment of localized scleroderma. J Am Acad Dermatol 1998;38:21-6.

35  Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol 2006;54:440-7.

36  Kreuter A, Gambichler T, Avermaete A, Jansen T, Hoffmann M, Hoffmann K, et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in Childhood Morphoea. Pediatr Dermatol2001;18:241-5.

37  Kroft EB, Creemers MC, Van Den Hoogen FH, Boezeman JB, De Jong EM, et al. Effectiveness, side-effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study. Br J Dermatol 2009;160:1075–82.

38  Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, et al. and The Childhood Arthritis and Rheumatology Research Alliance (CARRA) Localized Scleroderma Workgroup. Development of Consensus Treatment Plans for Juvenile Localized Scleroderma: A roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res 2012;64:1175–85.

39  Martini G, Ramanan AV, Falcini F, Girschick H, Goldsmith DP, Zulian F. Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford) 2009;48:1410–3.

40  Mirsky L, Chakkittakandiyil A, Laxer RM, O'Brien C, Pope E. Relapse after systemic treatment in paediatric morphoea. Br J Dermatol 2012,166:443–5.

41  Pavlotsky F, Sakka N, Lozinski A, Barzilai A. Bath psoralen–UVA photochemotherapy for localized scleroderma: experience from a single institute. Photodermatol Photoimmunol Photomed 2013; 29:247–52.

42  Pereira N. Santiago F, Oliveira H, Figueiredo A. Low-dose UVA1 phototherapy for scleroderma: what benefit can we expect? J Eur Acad Dermatol Venereol 2012;26:619–26.

43  Pope E, Doria AS, Theriault M, Mohanta A, Laxer RM. Topical Imiquimod 5% cream for pediatric plaque morphea: a prospective, multiple-baseline, open-label pilot study. Dermatology 2011;223:363–9.

44  Sator PG, Radakovic S, Schulmeister K, Hönigsmann H, Tanew A. Medium-dose is more effective than low-dose ultraviolet A1 phototherapy for localized scleroderma as shown by 20-MHz ultrasound assessment. J Am Acad Dermatol 2009;60:786-91.

45  Stege H, Berneburg M, Humke S, Klammer M, Grewe M, Grether-Beck S, et al. High-dose UVA1 radiation therapy for localized scleroderma. J Am Acad Dermatol 1997;36:938-44.

46  Su O, Onsun N, Onay HK, Erdemoglu Y, Ozkaya DB, Cebeci F, Somay A. Effectiveness of medium-dose ultraviolet A1 phototherapy in localized scleroderma. Int J Dermatol 2011;50:1006–13.

47  Tan E, Lim D, Rademaker M. Narrowband UVB phototherapy in children: a New Zealand experience. Australas J Dermatol 2010;51:268–73.

48  Torok KS, Arkachaisri T. Methotrexate and corticosteroids in the treatment of Localized Scleroderma: a standardized prospective longitudinal single-center study. J Rheumatol 2012;39:286–94.

49  Usmani N, Murphy A, Veale D, Goulden V, Goodfield M. Photochemotherapy for localized morphoea: effect on clinical and molecular markers. Clin Exp Dermatol 2008;33:698–704.

50  Uziel Y, Feldman BM, Krafchik BR, Yeung RS, Laxer RM. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr 2000;136:91-5.

51  Weibel L, Sampaio MC, Visentin MT, Howell KJ, Woo P, Harper JI. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol 2006;155:1013–20.

52  Zulian F, Martini G, Vallongo C, Vittadello F, Falcini F, Patrizi A, et al. Methotrexate Treatment in Juvenile Localized Scleroderma. A Randomized, Double-Blind, Placebo-Controlled Trial. Arthritis Rheum 2011;63:1998–2006.

53  Zulian F, Vallongo C, Patrizi A, Belloni-Fortina A, Cutrone M, Alessio M et al. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol 2012;67:1151-6.