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Development of minimum standards of care for juvenile localized scleroderma

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Abstract

Juvenile localized scleroderma (jLS), also known as morphea, is an orphan disease. Pediatric guidelines regarding diagnosis, assessment, and management are lacking.

Our objective was to develop minimum standards of care for diagnosis, assessment, and management of jLS. A systematic review was undertaken to establish the pediatric evidence for assessment and monitoring of jLS. An expert panel, including members of the Pediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and, where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement. Recommendations for diagnosis, assessment, and management were developed. Due to a lack of pediatric evidence, these were primarily consensus driven. Careful assessment for extra-cutaneous manifestations including synovitis, brain involvement, and uveitis were key features together with joint assessments between Dermatology and Rheumatology to improve and standardize care.

Conclusion: Management of jLS is varied. These recommendations should help provide standardization of assessment and care for those with this rare and potentially debilitating condition.

What is Known:

• Children with juvenile localized scleroderma (jLS) are managed by a number of specialties including pediatric rheumatologists and dermatologists, sometimes in shared clinics. Studies have shown that management varies considerably and that there are notable differences between specialties [1].

• There is very little published guidance on management of jLS.

What is new:

• These recommendations aim to standardize diagnosis, assessment, and management through review of pediatric evidence and consensus agreement.

• Joint review of patients by both pediatric rheumatologists and dermatologists is recommended.

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Abbreviations

AHA:

Anti-histone antibodies

ANA :

Antinuclear antibody

CARRA:

Childhood Arthritis and Rheumatology Alliance

CDLQI:

Children’s Dermatology Life Quality Index

CPK:

Creatinine phosphokinase

CRP:

C-reactive protein

DIET:

Dyspigmentation, Induration, Erythema, Telengiectasias

DMARD:

Disease-modifying anti-rheumatic drug

ECDS:

En coup de saber

EEG:

Electro-encephalogram

ESR:

Erythrocyte sedimentation rate

EULAR:

European League Against Rheumatism

JLS:

Juvenile localized scleroderma

LOCUS:

Localized Scleroderma Clinical and Ultrasound Study

LoSCAT:

Localized Scleroderma Cutaneous Assessment Tool

LoSDI:

Localized Scleroderma Damage Index

LS:

Localized scleroderma

mLoSSI:

Modified Localized Scleroderma Severity Skin Index

MMF :

Mycophenolate mofetil

mRSS:

Modified Rodnan Skin Score

MTX:

Methotrexate

PRES:

Pediatric Rheumatology European Society

PUVA therapy :

Psoralen ultraviolet A

QoL:

Quality of life

SSc:

Systemic sclerosis

UV:

Ultraviolet

References

  1. Johnson W, Jacobe H (2012) Morphea in adults and children cohort II: patients with morphea experience delay in diagnosis and large variation in treatment. J Am Acad Dermatol 67:881–889

    Article  PubMed  Google Scholar 

  2. Weibel L, Laguda B, Atherton D, Harper JI (2011) Misdiagnosis and delay in referral of children with localized scleroderma. Br J Dermatol 165:1308–1313

    Article  PubMed  CAS  Google Scholar 

  3. Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, Rabinovich CE, Laxer RM, Higgins GC, Ferguson PJ, Lasky A, Baszis K, Becker M, Campillo S, Cartwright V, Cidon M, Inman CJ, Jerath R, O'Neil KM, Vora S, Zeft A, Wallace CA, Ilowite NT, Fuhlbrigge RC (2012) Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res 64:1175–1185

    CAS  Google Scholar 

  4. Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, Cuttica R, Higgins GC, Van Suijlekom-Smit LW, Moore TL, Lindsley C, Garcia-Consuegra J, Esteves Hilario MO, Lepore L, Silva CA, Machado C, Garay SM, Uziel Y, Martini G, Foeldvari I, Peserico A, Woo P, Harper J, Juvenile Scleroderma Working Group of the Pediatric Rheumatology European S (2006) Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford, England) 45:614–620

    Article  CAS  Google Scholar 

  5. Nouri S, Jacobe H (2013) Recent developments in diagnosis and assessment of morphea. Curr Rheumatol Rep 15:308

    Article  PubMed  Google Scholar 

  6. Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM (2010) Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res 62:213–218

    Article  Google Scholar 

  7. Weibel L (2012) Localized scleroderma (morphea) in childhood. Der Hautarzt; Zeitschrift Dermatol, Venerol Verwandte Geb 63:89–96

    Article  CAS  Google Scholar 

  8. Kreuter A, Krieg T, Worm M, Wenzel J, Gambichler T, Kuhn A, Aberer E, Scharffetter-Kochanek K, Hunzelmann N (2009) AWMF guideline no. 013/066. Diagnosis and therapy of circumscribed scleroderma. J Deutschen Dermatol Ges = J German Soc Dermatol: JDDG 7(Suppl 6):S1–S14

  9. Peterson LS, Nelson AM, Su WP (1995) Classification of morphea (localized scleroderma). Mayo Clin Proc 70:1068–1076

    Article  PubMed  CAS  Google Scholar 

  10. Hawley DP, Baildam EM, Amin TS, Cruikshank MK, Davidson JE, Dixon J, Martin NS, Ohlsson V, Pilkington C, Rangaraj S, Riley P, Sundaramoorthy C, Walsh J, Foster HE (2012) Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK. Rheumatology (Oxford, England) 51:1235–1239

    Article  Google Scholar 

  11. Hawley DP, Pain CE, Baildam EM, Murphy R, Taylor AE, Foster HE (2014) United Kingdom survey of current management of juvenile localized scleroderma. Rheumatology (Oxford, England) 53:1849–1854

    Article  CAS  Google Scholar 

  12. Baildam EM, Ennis H, Foster HE, Shaw L, Chieng AS, Kelly J, Herrick AL, Richards HL (2011) Influence of childhood scleroderma on physical function and quality of life. J Rheumatol 38:167–173

    Article  PubMed  Google Scholar 

  13. Orzechowski NM, Davis DM, Mason TG 3rd, Crowson CS, Reed AM (2009) Health-related quality of life in children and adolescents with juvenile localized scleroderma. Rheumatology (Oxford, England) 48:670–672

    Article  Google Scholar 

  14. Das S, Bernstein I, Jacobe H (2014) Correlates of self-reported quality of life in adults and children with morphea. J Am Acad Dermatol 70:904–910

    Article  PubMed  PubMed Central  Google Scholar 

  15. Kreuter A, Krieg T, Worm M, Wenzel J, Moinzadeh P, Kuhn A, Aberer E, Scharffetter-Kochanek K, Horneff G, Reil E, Weberschock T, Hunzelmann N (2016) German guidelines for the diagnosis and therapy of localized scleroderma. J Deutschen Dermatol Ges = J German Soc Dermatol: JDDG 14:199–216

    Article  Google Scholar 

  16. Dougados M, Betteridge N, Burmester GR, Euller-Ziegler L, Guillemin F, Hirvonen J, Lloyd J, Ozen S, Da Silva JA, Emery P, Kalden JR, Kvien T, Matucci-Cerinic M, Smolen J (2004) EULAR standardised operating procedures for the elaboration, evaluation, dissemination, and implementation of recommendations endorsed by the EULAR standing committees. Ann Rheum Dis 63:1172–1176

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  17. Zhang W, Doherty M, Pascual E, Bardin T, Barskova V, Conaghan P, Gerster J, Jacobs J, Leeb B, Liote F, McCarthy G, Netter P, Nuki G, Perez-Ruiz F, Pignone A, Pimentao J, Punzi L, Roddy E, Uhlig T, Zimmermann-Gorska I (2006) EULAR evidence based recommendations for gout. Part I: diagnosis. Report of a task force of the standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). Ann Rheum Dis 65:1301–1311

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  18. Zhang W, Doherty M, Bardin T, Pascual E, Barskova V, Conaghan P, Gerster J, Jacobs J, Leeb B, Liote F, McCarthy G, Netter P, Nuki G, Perez-Ruiz F, Pignone A, Pimentao J, Punzi L, Roddy E, Uhlig T, Zimmermann-Gorska I (2006) EULAR evidence based recommendations for gout. Part II: management. Report of a task force of the EULAR standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). Ann Rheum Dis 65:1312–1324

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  19. Piram M, McCuaig CC, Saint-Cyr C, Marcoux D, Hatami A, Haddad E, Powell J (2013) Short- and long-term outcome of linear morphoea in children. Br J Dermatol 169:1265–1271

    Article  PubMed  CAS  Google Scholar 

  20. Blaszczyk M, Janniger CK, Jablonska S (1996) Childhood scleroderma and its peculiarities. Cutis 58:141–144, 148–152

  21. (2009) Weedon’s skin pathology. Churchill Livingstone

  22. Wortsman X, Wortsman J, Sazunic I, Carreno L (2011) Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol 65:942–948

    Article  PubMed  Google Scholar 

  23. Beltramelli M, Vercellesi P, Frasin A, Gelmetti C, Corona F (2010) Localized severe scleroderma: a retrospective study of 26 pediatric patients. Pediatr Dermatol 27:476–480

    Article  PubMed  Google Scholar 

  24. Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM Clinical features of childhood localized scleroderma in an incidence cohort. Rheumatology (Oxford, England) 50:1865–1868

  25. Wu EY, Li SC, Torok KS, Virkud Y, Fuhlbrigge R, Rabinovich CE (2014) A28: Description of the Juvenile Localized Scleroderma Subgroup of the CARRA Registry. Arthritis Rheumatol (Hoboken, NJ) 66(Suppl 11):S43–S44

    Article  Google Scholar 

  26. Uziel Y, Krafchik BR, Silverman ED, Thorner PS, Laxer RM (1994) Localized scleroderma in childhood: a report of 30 cases. Semin Arthritis Rheum 23:328–340

    Article  PubMed  CAS  Google Scholar 

  27. Vancheeswaran R, Black CM, David J, Hasson N, Harper J, Atherton D, Trivedi P, Woo P (1996) Childhood-onset scleroderma: is it different from adult-onset disease. Arthritis Rheum 39:1041–1049

    Article  PubMed  CAS  Google Scholar 

  28. Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, Caputo R (2003) Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Europ J Dermatol: EJD 13:171–176

    Google Scholar 

  29. Rosenberg AM, Uziel Y, Krafchik BR, Hauta SA, Prokopchuk PA, Silverman ED, Laxer RM (1995) Antinuclear antibodies in children with localized scleroderma. J Rheumatol 22:2337–2343

    PubMed  CAS  Google Scholar 

  30. Takehara K, Moroi Y, Nakabayashi Y, Ishibashi Y (1983) Antinuclear antibodies in localized scleroderma. Arthritis Rheum 26:612–616

    Article  PubMed  CAS  Google Scholar 

  31. Christen-Zaech S, Hakim MD, Afsar FS, Paller AS (2008) Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 59:385–396

    Article  PubMed  Google Scholar 

  32. Laxer RM, Zulian F (2006) Localized scleroderma. Curr Opin Rheumatol 18:606–613

    Article  PubMed  Google Scholar 

  33. Takehara K, Sato S (2005) Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford, England) 44:274–279

    Article  CAS  Google Scholar 

  34. Sato S, Fujimoto M, Ihn H, Kikuchi K, Takehara K (1994) Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma. J Am Acad Dermatol 31:567–571

    Article  PubMed  CAS  Google Scholar 

  35. Lo CY, Shyur SD, Chu SH, Huang LH, Kao YH, Lei WT, Cheng CH, Lee KH, Chen CK, Liu LC Juvenile scleroderma: experience in one institution. Asian Pac J Allergy Immunol/ Launched Allergy Immunol Soc Thailand 28:279–286

  36. Parodi A, Drosera M, Barbieri L, Rebora A (1995) Antihistone antibodies in scleroderma. Dermatology (Basel, Switzerland) 191:16–18

    Article  CAS  Google Scholar 

  37. el-Azhary RA, Aponte CC, Nelson AM, Weaver AL, Homburger HA (2006) Antihistone antibodies in linear scleroderma variants. Int J Dermatol 45:1296–1299

    Article  PubMed  CAS  Google Scholar 

  38. Dharamsi JW, Victor S, Aguwa N, Ahn C, Arnett F, Mayes MD, Jacobe H (2013) Morphea in adults and children cohort III: nested case-control study—the clinical significance of autoantibodies in morphea. JAMA dermatology 149:1159–1165

    Article  PubMed  PubMed Central  Google Scholar 

  39. Falanga V, Medsger TA Jr, Reichlin M, Rodnan GP (1986) Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med 104:849–857

    Article  PubMed  CAS  Google Scholar 

  40. Arkachaisri T, Fertig N, Pino S, Medsger TA Jr (2008) Serum autoantibodies and their clinical associations in patients with childhood- and adult-onset linear scleroderma. A single-center study. J Rheumatol 35:2439–2444

    Article  PubMed  CAS  Google Scholar 

  41. Weber K (2009) Is juvenile localized scleroderma related to Lyme borreliosis? J Am Acad Dermatol 61:901

    Article  PubMed  Google Scholar 

  42. Weide B, Walz T, Garbe C (2000) Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol 142:636–644

    Article  PubMed  CAS  Google Scholar 

  43. Fan W, Leonardi CL, Penneys NS (1995) Absence of Borrelia burgdorferi in patients with localized scleroderma (morphea). J Am Acad Dermatol 33:682–684

    Article  PubMed  CAS  Google Scholar 

  44. Dillon WI, Saed GM, Fivenson DP (1995) Borrelia burgdorferi DNA is undetectable by polymerase chain reaction in skin lesions of morphea, scleroderma, or lichen sclerosus et atrophicus of patients from North America. J Am Acad Dermatol 33:617–620

    Article  PubMed  CAS  Google Scholar 

  45. Raguin G, Boisnic S, Souteyrand P, Baranton G, Piette JC, Godeau P, Frances C (1992) No evidence for a spirochaetal origin of localized scleroderma. Br J Dermatol 127:218–220

    Article  PubMed  CAS  Google Scholar 

  46. Vaillant L, Goudeau A (1992) Localized scleroderma is not a Borrelia burgdorferi infection in France. Dermatology (Basel, Switzerland) 184:286–288

    Article  CAS  Google Scholar 

  47. Lupoli S, Cutler SJ, Stephens CO, Wright DJ, Black CM (1991) Lyme disease and localized scleroderma—no evidence for a common aetiology. Br J Rheumatol 30:154–156

    Article  PubMed  CAS  Google Scholar 

  48. Eisendle K, Zelger B (2009) The expanding spectrum of cutaneous borreliosis. G Ital Dermatol Venereol 144:157–171

    PubMed  CAS  Google Scholar 

  49. Ozkan S, Atabey N, Fetil E, Erkizan V, Gunes AT (2000) Evidence for Borrelia burgdorferi in morphea and lichen sclerosus. Int J Dermatol 39:278–283

    Article  PubMed  CAS  Google Scholar 

  50. Schempp C, Bocklage H, Lange R, Kolmel HW, Orfanos CE, Gollnick H (1993) Further evidence for Borrelia burgdorferi infection in morphea and lichen sclerosus et atrophicus confirmed by DNA amplification. J Invest Dermatol 100:717–720

    Article  PubMed  CAS  Google Scholar 

  51. Akimoto S, Ishikawa O, Miyachi Y (1996) The absence of antibodies against Borrelia burgdorferi in the sera of Japanese patients with localized scleroderma. J Rheumatol 23:573–574

    PubMed  CAS  Google Scholar 

  52. Espinoza-Leon F, Hassanhi-Hassanhi M, Arocha-Sandoval F, Urbina-Lopez M (2006) Absence of Borrelia burgdorferi antibodies in the sera of Venezuelan patients with localized scleroderma (morphea). Investig Clin 47:283–288

    Google Scholar 

  53. Zollinger T, Mertz KD, Schmid M, Schmitt A, Pfaltz M, Kempf W (2010) Borrelia in granuloma annulare, morphea and lichen sclerosus: a PCR-based study and review of the literature. J Cutan Pathol 37:571–577

    Article  PubMed  Google Scholar 

  54. Goodlad JR, Davidson MM, Gordon P, Billington R, Ho-Yen DO (2002) Morphoea and Borrelia burgdorferi: results from the Scottish highlands in the context of the world literature. Mol Pathol: MP 55:374–378

    Article  PubMed  CAS  Google Scholar 

  55. Weide B, Schittek B, Klyscz T, Schuz K, Stark M, Rassner G, Wilske B, Garbe C (2000) Morphoea is neither associated with features of Borrelia burgdorferi infection, nor is this agent detectable in lesional skin by polymerase chain reaction. Br J Dermatol 143:780–785

    Article  PubMed  CAS  Google Scholar 

  56. Wienecke R, Schlupen EM, Zochling N, Neubert U, Meurer M, Volkenandt M (1995) No evidence for Borrelia burgdorferi-specific DNA in lesions of localized scleroderma. J Invest Dermatol 104:23–26

    Article  PubMed  CAS  Google Scholar 

  57. Ranki A, Aavik E, Peterson P, Schauman K, Nurmilaakso P (1994) Successful amplification of DNA specific for Finnish Borrelia burgdorferi isolates in erythema chronicum migrans but not in circumscribed scleroderma lesions. J Invest Dermatol 102:339–345

    Article  PubMed  CAS  Google Scholar 

  58. Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J, Espada G, Corona F, Mukamel M, Vesely R, Musiej-Nowakowska E, Chaitow J, Ros J, Apaz MT, Gerloni V, Mazur-Zielinska H, Nielsen S, Ullman S, Horneff G, Wouters C, Martini G, Cimaz R, Laxer R, Athreya BH, Juvenile Scleroderma Working Group of the Pediatric Rheumatology European S (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52:2873–2881

    Article  PubMed  Google Scholar 

  59. Weber P, Ganser G, Frosch M, Roth J, Hulskamp G, Zimmer KP (2000) Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol 27:2692–2695

    PubMed  CAS  Google Scholar 

  60. Nelson SP, Chen EH, Syniar GM, Christoffel KK (2000) Prevalence of symptoms of gastroesophageal reflux during childhood: a pediatric practice-based survey. Pediatric Practice Res Group. Arch Pediatr Adolesc Med 154:150–154

    Article  CAS  Google Scholar 

  61. Akinbami LJ, Moorman JE, Garbe PL, Sondik EJ (2009) Status of childhood asthma in the United States, 1980–2007. Pediatrics 123(Suppl 3):S131–S145

    Article  PubMed  Google Scholar 

  62. Wozniak J, Dabrowski R, Luczak D, Kwiatkowska M, Musiej-Nowakowska E, Kowalik I, Szwed H (2009) Evaluation of heart rhythm variability and arrhythmia in children with systemic and localized scleroderma. J Rheumatol 36:191–196

    PubMed  Google Scholar 

  63. Borowiec A, Dabrowski R, Wozniak J, Jasek S, Chwyczko T, Kowalik I, Musiej-Nowakowska E, Szwed H (2011) Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation. Scand J Rheumatol 41:33–38

    Article  PubMed  Google Scholar 

  64. Hayashi M, Ichiki Y, Kitajima Y (2009) Coexistence of recurrent generalized morphea and systemic sclerosis. Acta Derm Venereol 89:329–330

    Article  PubMed  Google Scholar 

  65. Holzmann H, Schlieter A (1994) Coexistence of morphea and systemic sclerosis. Dermatology (Basel, Switzerland) 189:99

    Article  CAS  Google Scholar 

  66. Soma Y, Tamaki T, Kikuchi K, Abe M, Igarashi A, Takehara K, Ishibashi Y (1993) Coexistence of morphea and systemic sclerosis. Dermatology (Basel, Switzerland) 186:103–105

    Article  CAS  Google Scholar 

  67. Maricq HR (1992) Capillary abnormalities, Raynaud’s phenomenon, and systemic sclerosis in patients with localized scleroderma. Arch Dermatol 128:630–632

    Article  PubMed  CAS  Google Scholar 

  68. Carneiro S, Ramos-e-Silva M, Russi DC, Albuquerque EM, Sousa MA (2011) Coexistence of generalized morphea and lichen sclerosus et atrophicus mimicking systemic disease. Skinmed 9:131–133

    PubMed  Google Scholar 

  69. Fain ET, Mannion M, Pope E, Young DW, Laxer RM, Cron RQ, Brain cavernomas associated with en coup de sabre linear scleroderma (2011) Two case reports. Pediatr Rheumatol Online J 9:18

    Article  PubMed  PubMed Central  Google Scholar 

  70. Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, Alpigiani MG, Alessio M, Paradisi M, Woo P, Zulian F, Juvenile Scleroderma Working Group of the Pediatric Rheumatology European S (2007) Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol 91:1311–1314

    Article  PubMed  PubMed Central  Google Scholar 

  71. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, Lehman TJ, de Oliveira SK, Susic G, Lyskina G, Nemcova D, Sundel R, Falcini F, Girschick H, Lotito AP, Buoncompagni A, Sztajnbok F, Al-Mayouf SM, Orban I, Ferri C, Athreya BH, Woo P, Zulian F, Juvenile Scleroderma Working Group of the Pediatric Rheumatology European S (2006) Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum 54:3971–3978

    Article  PubMed  CAS  Google Scholar 

  72. Foster TD (1979) The effects of hemifacial atrophy on dental growth. Br Dent J 146:148–150

    Article  PubMed  CAS  Google Scholar 

  73. You KH, Baik HS (2011) Orthopedic and orthodontic treatment of Parry–Romberg syndrome. J Craniofac Surg 22:970–973

    Article  PubMed  Google Scholar 

  74. Vig PS, Vig KW (1986) Hybrid appliances: a component approach to dentofacial orthopedics. Am J Orthod Dentofac Orthop 90:273–285

    Article  CAS  Google Scholar 

  75. Grippaudo C, Deli R, Grippaudo FR, Di Cuia T, Paradisi M (2004) Management of craniofacial development in the Parry–Romberg syndrome: report of two patients. Cleft Palate Craniofac J 41:95–104

    Article  PubMed  Google Scholar 

  76. Tzaribachev N, Fritz J, Horger M (2009) Spectrum of magnetic resonance imaging appearances of juvenile temporomandibular joints (TMJ) in non-rheumatic children. Acta Radiologica (Stockholm, Sweden: 1987) 50:1182–1186

    Article  CAS  Google Scholar 

  77. Stoll ML, Guleria S, Mannion ML, Young DW, Royal SA, Cron RQ, Vaid YN (2018) Defining the normal appearance of the temporomandibular joints by magnetic resonance imaging with contrast: a comparative study of children with and without juvenile idiopathic arthritis. Pediatr Rheumatol Online J 16:8

    Article  PubMed  PubMed Central  Google Scholar 

  78. Zulian F, Meneghesso D, Grisan E, Vittadello F, Belloni Fortina A, Pigozzi B, Frigo AC, Martini G, Ruggeri A (2007) A new computerized method for the assessment of skin lesions in localized scleroderma. Rheumatology (Oxford, England) 46:856–860

    Article  CAS  Google Scholar 

  79. Poff S, Li SC, Kelsey CE, Foeldvari I, Torok KS (2016) Durometry as an outcome measure in juvenile localized scleroderma. Br J Dermatol 174:228–230

    Article  PubMed  Google Scholar 

  80. Li SC, Liebling MS (2009) The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep 11:205–211

    Article  PubMed  Google Scholar 

  81. Li SC, Liebling MS, Haines KA (2007) Ultrasonography is a sensitive tool for monitoring localized scleroderma. Rheumatology (Oxford, England) 46:1316–1319

    Article  CAS  Google Scholar 

  82. Li SC, Liebling MS, Haines KA, Weiss JE, Prann A (2011) Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma. Arthritis Care Res 63:735–742

    Article  CAS  Google Scholar 

  83. Weibel L, Howell KJ, Visentin MT, Rudiger A, Denton CP, Zulian F, Woo P, Harper JI (2007) Laser Doppler flowmetry for assessing localized scleroderma in children. Arthritis Rheum 56:3489–3495

    Article  PubMed  Google Scholar 

  84. Boers M, Brooks P, Strand CV, Tugwell P (1998) The OMERACT filter for outcome measures in rheumatology. J Rheumatol 25:198–199

    PubMed  CAS  Google Scholar 

  85. Cunningham BB, Landells ID, Langman C, Sailer DE, Paller AS (1998) Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol 39:211–215

    Article  PubMed  CAS  Google Scholar 

  86. Pope JE, Baron M, Bellamy N, Campbell J, Carette S, Chalmers I, Dales P, Hanly J, Kaminska EA, Lee P et al (1995) Variability of skin scores and clinical measurements in scleroderma. J Rheumatol 22:1271–1276

    PubMed  CAS  Google Scholar 

  87. Arkachaisri T, Vilaiyuk S, Li S, O'Neil KM, Pope E, Higgins GC, Punaro M, Rabinovich EC, Rosenkranz M, Kietz DA, Rosen P, Spalding SJ, Hennon TR, Torok KS, Cassidy E, Medsger TA Jr, Localized Scleroderma C, Ultrasound Study G (2009) 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 36:2819–2829

    Article  PubMed  PubMed Central  Google Scholar 

  88. Torok KS, Arkachaisri T (2012) Methotrexate and corticosteroids in the treatment of localized scleroderma: a standardized prospective longitudinal single-center study. J Rheumatol 39:286–294

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  89. Kelsey CE, Torok KS (2013) The localized scleroderma cutaneous assessment tool: responsiveness to change in a pediatric clinical population. J Am Acad Dermatol 69:214–220

    Article  PubMed  PubMed Central  Google Scholar 

  90. 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, England) 49:373–381

  91. Lewis-Jones MS, Finlay AY (1995) The Children’s Dermatology Life Quality Index (CDLQI): initial validation and practical use. Br J Dermatol 132:942–949

    Article  PubMed  CAS  Google Scholar 

  92. Li SC, Feldman BM, Higgins GC, Haines KA, Punaro MG, O'Neil KM (2010) Treatment of pediatric localized scleroderma: results of a survey of North American pediatric rheumatologists. J Rheumatol 37:175–181

    Article  PubMed  Google Scholar 

  93. Kreuter A, Gambichler T, Avermaete A, Jansen T, Hoffmann M, Hoffmann K, Altmeyer P, von Kobyletzki G, Bacharach-Buhles M (2001) Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol 18:241–245

    Article  PubMed  CAS  Google Scholar 

  94. Zwischenberger BA, Jacobe HT (2011) A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol 65:925–941

    Article  PubMed  CAS  Google Scholar 

  95. Dytoc MT, Kossintseva I, Ting PT (2007) First case series on the use of calcipotriol-betamethasone dipropionate for morphoea. Br J Dermatol 157:615–618

    Article  PubMed  CAS  Google Scholar 

  96. Mancuso G, Berdondini RM (2005) Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol 152:180–182

    Article  PubMed  CAS  Google Scholar 

  97. Dytoc M, Ting PT, Man J, Sawyer D, Fiorillo L (2005) First case series on the use of imiquimod for morphoea. Br J Dermatol 153:815–820

    Article  PubMed  CAS  Google Scholar 

  98. Pope E, Doria AS, Theriault M, Mohanta A, Laxer RM (2011) Topical imiquimod 5% cream for pediatric plaque morphea: a prospective, multiple-baseline, open-label pilot study. Dermatology (Basel, Switzerland) 223:363–369

    Article  CAS  Google Scholar 

  99. Fett N, Werth VP Update on morphea: part II. Outcome measures and treatment. J Am Acad Dermatol 64:231–242 quiz 243–234

  100. Kreuter A, Hyun J, Stucker M, Sommer A, Altmeyer P, Gambichler T (2006) 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 54:440–447

    Article  PubMed  Google Scholar 

  101. Vilela FA, Carneiro S, Ramos-e-Silva M Treatment of morphea or localized scleroderma: review of the literature. J Drugs Dermatol: JDD 9:1213–1219

  102. Zulian F, Martini G, Vallongo C, Vittadello F, Falcini F, Patrizi A, Alessio M, La Torre F, Podda RA, Gerloni V, Cutrone M, Belloni-Fortina A, Paradisi M, Martino S, Perilongo G (2011) Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 63:1998–2006

    Article  PubMed  CAS  Google Scholar 

  103. Weibel L, Sampaio MC, Visentin MT, Howell KJ, Woo P, Harper JI (2006) Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol 155:1013–1020

    Article  PubMed  CAS  Google Scholar 

  104. Saxton-Daniels S, Jacobe HT An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol 146:1044–1045

  105. Martini G, Ramanan AV, Falcini F, Girschick H, Goldsmith DP, Zulian F (2009) Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford, England) 48:1410–1413

    Article  Google Scholar 

  106. Stausbol-Gron B, Olesen AB, Deleuran B, Deleuran MS (2011) Abatacept is a promising treatment for patients with disseminated morphea profunda: presentation of two cases. Acta Derm Venereol 91:686–688

    Article  PubMed  Google Scholar 

  107. Perez Crespo M, Betlloch Mas I, Mataix Diaz J, Lucas Costa A, Ballester Nortes I (2009) Rapid response to cyclosporine and maintenance with methotrexate in linear scleroderma in a young girl. Pediatr Dermatol 26:118–120

    Article  PubMed  Google Scholar 

  108. Ferguson ID, Weiser P, Torok KS (2015) A case report of successful treatment of recalcitrant childhood localized scleroderma with infliximab and leflunomide. Open Rheumatol J 9:30–35

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  109. Heiligenhaus A, Michels H, Schumacher C, Kopp I, Neudorf U, Niehues T, Baus H, Becker M, Bertram B, Dannecker G, Deuter C, Foeldvari I, Frosch M, Ganser G, Gaubitz M, Gerdes G, Horneff G, Illhardt A, Mackensen F, Minden K, Pleyer U, Schneider M, Wagner N, Zierhut M (2012) Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis. Rheumatol Int 32:1121–1133

    Article  PubMed  CAS  Google Scholar 

  110. Ivan Foeldvari JA, Friswell M, Bica B, de Inocencio J, Aquilani A, Helmus N (2017) Tocilizumab is a promising treatment option for therapy resistant juvenile localized scleroderma patients. J Scleroderma Relat Disord 2:203–207

    Article  Google Scholar 

  111. Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C, Karampetsou M, Yiannopoulos G, Andonopoulos AP (2010) Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford, England) 49:271–280

    Article  CAS  Google Scholar 

  112. Torok KS (2012) Pediatric scleroderma: systemic or localized forms. Pediatr Clin N Am 59:381–405

    Article  Google Scholar 

  113. Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E (2010) En coup de sabre scleroderma and Parry–Romberg syndrome in adolescents: surgical options and patient-related outcomes. J Rheumatol 37:2174–2179

    Article  PubMed  Google Scholar 

  114. Sautereau N, Daumas A, Truillet R, Jouve E, Magalon J, Veran J, Casanova D, Frances Y, Magalon G, Granel B (2016) Efficacy of autologous microfat graft on facial handicap in systemic sclerosis patients. Plastic Reconstr Surg Global Open 4:e660

    Article  Google Scholar 

  115. Magalon J, Daumas A, Veran J, Magalon G, Rossi P, Granel B, Sabatier F (2015) Autologous adipose tissue-derived cells: are we talking about adipose derived stem cells, stromal vascular fraction, or Coleman fat grafting? Cell Transplant 24:2667–2668

    Article  PubMed  CAS  Google Scholar 

  116. Dueckers G, Guellac N, Arbogast M, Dannecker G, Foeldvari I, Frosch M, Ganser G, Heiligenhaus A, Horneff G, Illhardt A, Kopp I, Krauspe R, Markus B, Michels H, Schneider M, Singendonk W, Sitter H, Spamer M, Wagner N, Niehues T (2012) Evidence and consensus based GKJR guidelines for the treatment of juvenile idiopathic arthritis. Clin Immunol 142:176–193

    Article  PubMed  CAS  Google Scholar 

  117. Zulian F, Vallongo C, Patrizi A, Belloni-Fortina A, Cutrone M, Alessio M, Martino S, Gerloni V, Vittadello F, Martini G (2012) A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol 67:1151–1156

    Article  PubMed  CAS  Google Scholar 

  118. Martini G, Murray KJ, Howell KJ, Harper J, Atherton D, Woo P, Zulian F, Black CM (2002) Juvenile-onset localized scleroderma activity detection by infrared thermography. Rheumatology (Oxford, England) 41:1178–1182

    Article  CAS  Google Scholar 

  119. Adeeb F, Anjum S, Hodnett P, Kashif A, Brady M, Morrissey S, Devlin J, Fraser AD (2017) Early- and late-stage morphea subtypes with deep tissue involvement is treatable with abatacept (Orencia). Semin Arthritis Rheum 46:775–781

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Authors and Affiliations

  1. Semmelweis University, Budapest, Hungary

    Tamás Constantin, Annamária Pálinkás & Melinda Laczkovszki

  2. Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany

    Ivan Foeldvari

  3. Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

    Clare E. Pain

  4. Department of Paediatric Dermatology, Catholic Children’s Hospital Wilhelmstift, Hamburg, Germany

    Peter Höger

  5. University Childrens Hospital, Tuebingen, Germany

    Monika Moll

  6. Charles University, Prague, Czech Republic

    Dana Nemkova

  7. Department of Dermatology, University Children’s Hospital Zurich, Zurich, Switzerland

    Lisa Weibel

  8. David Geffen School of Medicine at UCLA, Division of Rheumatology, Los Angeles, CA, USA

    Philip Clements

  9. University of Pittsburgh, Pittsburgh, PA, USA

    Kathryn S. Torok

Authors
  1. Tamás Constantin
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  2. Ivan Foeldvari
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  3. Clare E. Pain
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  4. Peter Höger
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  5. Monika Moll
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  6. Dana Nemkova
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  7. Lisa Weibel
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  8. Melinda Laczkovszki
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  9. Philip Clements
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  10. Kathryn S. Torok
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Contributions

Kathryn S. Torok is senior author. Ivan Foeldvari designed the initiative. Annamária Pálinkás, Melinda Laczkovszki performed the systematic literature review, supervised by Tamás Constantin. Validity assessment of selected papers was done by Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok. Recommendations were formulated by Tamás Constantin, Ivan Foeldvari and Clare E Pain. The expert committee consisted of Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok; they completed the online surveys and participated in consensus meeting. Philip Clements assisted in the preparation of the live consensus meeting and facilitated the consensus procedure using nominal group technique. Tamás Constantin, Clare E Pain and Kathryn S. Torok wrote the manuscript, with contribution and approval of all co-authors.

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Correspondence to Tamás Constantin.

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Constantin, T., Foeldvari, I., Pain, C.E. et al. Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr 177, 961–977 (2018). https://doi.org/10.1007/s00431-018-3144-8

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  • DOI: https://doi.org/10.1007/s00431-018-3144-8

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