Juvenile Localized Scleroderma from the Perspective of Pediatric Rheumatologist. Aspects of Diagnostics
https://doi.org/10.15690/vsp.v19i2.2109
Abstract
The localized scleroderma (morphea) is the clinical option of the juvenile scleroderma, the third in prevalence rheumatic condition in pediatrics. The article summarizes all the data on the classification, diagnostics, and differential diagnosis of juvenile localized scleroderma. The recent international guidelines on the localized scleroderma in pediatrics (the European consensus of pediatric rheumatologists, the German and Japanese national guidelines) are presented in the article.
Keywords
About the Authors
Rinat K. RaupovRussian Federation
Saint Petersburg.
Disclosure of interest:
M.M. Kostik — receives fees for lecturing from Pfizer, Novartis, Sanofi companies. The other contributors confirmed the absence of a reportable conflict of interests.
Artur I. Imelbaev
Russian Federation
Saint Petersburg.
Disclosure of interest:
M.M. Kostik — receives fees for lecturing from Pfizer, Novartis, Sanofi companies. The other contributors confirmed the absence of a reportable conflict of interests.
Mikhail M. Kostik
Russian Federation
Saint Petersburg.
Disclosure of interest:
M.M. Kostik — receives fees for lecturing from Pfizer, Novartis, Sanofi companies. The other contributors confirmed the absence of a reportable conflict of interests.
References
1. Zulian F. Scleroderma in children. Best Pract Res Clin Rheumatol. 2017;31(4):576-595. doi: 10.1016/j.berh.2018.02.004.
2. Herrick AL, Ennis H, Bhushan M, et al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res. 2010;62(2):213-218. doi: 10.1002/acr.20070.
3. Zulian F, Athreya BH, Laxer RM, et al. Juvenile Localized Scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45(5): 614-620. doi: 10.1093/rheumatology/kei251.
4. Kreuter A, Krieg T, Worm M, et al. German guidelines for the diagnosis and therapy of localized scleroderma. J Dtsch Dermatol Ges. 2016;14(2):199-216. doi: 10.1111/ddg.12724.
5. Weibel L, Laguda B, Atherton D, Harper JI. Misdiagnosis and delay in referral of children with localized scleroderma. Br J Dermatol. 2011;(6):1308-1313. doi: 10.1111/j.1365-2133.2011.10600.x.
6. Hawley DP, Baildam EM, Amin TS, et al. Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK. Rheumatology (Oxford). 2012;51(7): 1235-1239. doi: 10.1093/rheumatology/ker521.
7. Li SC. Scleroderma in children and adolescents: localized scleroderma and systemic sclerosis. Pediatr Clin North Am. 2018; 65(4):757-781. doi: 10.1016/j.pcl.2018.04.002.
8. Hawley DP, Pain CE, Baildam EM, et al. United Kingdom survey of current management of juvenile localized scleroderma. Rheumatology (Oxford). 2014;53(10):1849-1854. doi: 10.1093/rheumatology/keu212.
9. Lokalizovannaya sklerodermiya. Klinicheskiye rekomendatsii. Moscow: Rossiyskoye obshchestvo dermatovenerologov i kosmetologov; 2016. (In Russ). Доступно по: http://kokb45.ru/wp-content/uploads/2018/06/Lokalizovannaya-sklerodermiya. pdf. Ссылка активна на 12.02.2020.
10. Strickland, N, Patel, G, Strickland, A, Jacobe, H. Attitudes and trends in the treatment of morphea: a national survey. J Ame Acad Derm. 2015;72(4):727-728. doi: 10.1016/j.jaad.2014.11.019.
11. Asano Y, Fujimoto M, Ishikawa O, et al. Diagnostic criteria, severity classification and guidelines of localized scleroderma. J Dermatol. 2018;45(7):755-780. doi: 10.1111/1346-8138.14161.
12. Zulian F, Culpo R, Sperotto F, et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis. 2019;78(8):1019-1024. doi: 10.1136/annrheum-dis-2018-214697.
13. Peterson LS, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997;24(1):73-80.
14. Kubanova AA, Kubanov AA, Melekhina LE, Bogdanova EV. The assessment of the incidence of skin disorders in Russian Federation in 2003-2016. Vestnik dermatologii i venerolo-gii. 2017;(6):22-33. (In Russ). doi: 10.25208/0042-4609-201793-6-22-33.
15. Khamaganova I. Localized scleroderma: predisposing and triggering factors. Open Derm J. 2017;11(1):1-11. doi: 10.2174/1874372201711010001.
16. Wolf R, Wolf D, Ruocco V, Ruocco Е. The role of skin trauma (isotopic and isomorphic) in the distribution of morphea. J Am Acad Dermatol. 2015;72(3):560-561. doi: 10.1016/j.jaad.2014.10.046.
17. Weide B, Walz T, Garbe C. Is morphoea caused by Borrelia burgdorferi? a review. Br J Dermatol. 2000;142(4):636-644. doi: 10.1046/j.1365-2133.2000.03407.x.
18. Dillon WI, Saed GM, Fivenson DP. Borreliaburgdorferi DNA is undetectable by polymerase chain reaction in skin lesions of morphea, scleroderma, or lichen sclerosusetatrophicus of patients from North America. J Am Acad Dermatol. 1995;33(4):617-620. doi: 10.1016/0190-9622(95)91281-9.
19. Weber K. Is juvenile localized scleroderma related to lyme Borreliosis? J Am Acad Dermatol. 2009;61(5):901. doi: 10.1016/j.jaad.2009.05.022.
20. Jacobe H, Ahn C, Arnett FC, Reveille JD. Major histocompatibility complex class I and class II alleles may confer susceptibility to or protection against morphea: findings from the morphea in adults and children cohort. Arthritis Rheumatol. 2014;66(11):3170-3177. doi: 10.1002/art.38814.
21. Hemminki K, Li X, Sundquist J, Sundquist K. Familial associations of rheumatoid arthritis with autoimmune diseases and related conditions. Arthritis Rheum. 2009;60(3):661-668. doi: 10.1002/art.24328.
22. Torok KS, Li SC, Jacobe HM, et al. Immunopathogenesis of pediatric localized scleroderma. Front Immunol. 2019;10:908. doi: 10.3389/fimmu.2019.00908.
23. Knobler R, Moinzadeh P, Hunzelmann N, et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol. 2017;31(9):1401-1424. doi: 10.1111/jdv.14458.
24. Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64(2):217-228. doi: 10.1016/j.jaad.2010.05.045.
25. Magee KE, Kelsey CE, Kurzinski KL, et al. Interferon-gamma inducible protein-10 as a potential biomarker in localized scleroderma. Arthritis Res Ther. 2013;15(6):R188. doi: 10.1186/ar4378.
26. Torok KS, Mi Q, Mirizio E, et al. Chemokine ligand 9 (CXCL9) [monokine induced by gamma interferon (MIG)] as a predictor of active disease status in localized scleroderma. Arthritis Rheumatol. 2017;69(Suppl 10):2346.
27. Torok KS, Kurzinski K, Kelsey C, et al. Peripheral blood cytokine and chemokine profiles in juvenile localized scleroderma: T-helper cell-associated cytokine profiles. Semin Arthritis Rheum. 2015;45(3):284-293. doi: 10.1016/j.semarthrit.2015.06.006.
28. Aractingi S, Socie G, Devergie A, et al. Localized sclerodermalike lesions on the legs in bone marrow transplant recipients: association with polyneuropathy in the same distribution. Br J Dermatol. 1993;129(2):201-203. doi: 10.1111/j.1365-2133.1993.tb03530.x.
29. McNallan KT, Aponte C, el-Azhary R, et al. Immunophenotyping of chimeric cells in localized scleroderma. Rheumatology (Oxford). 2007;46(3):398-402. doi: 10.1093/rheumatology/kel297.
30. Yi Ye, van Zyl B, Hemlata V, et al. Maternal microchimerism in muscle biopsies from children with juvenile dermatomyositis. Rheumatology (Oxford). 2012;51(6):987-991. doi: 10.1093/rheumatology/ker430.
31. Stevens AM, Hermes HM, Rutledge JC, et al. Myocardial-tissuespecific phenotype of maternal microchimerism in neonatal lupus congenital heart block. Lancet. 2003;362(9396):1617-1623. doi: 10.1016/S0140-6736(03)14795-2.
32. Vancheeswaran R, Black CM, David J, et al. Childhood-onset scleroderma: is it different from adult-onset disease? Arthritis Rheum. 1996;39(6):1041-1049. doi: 10.1002/art.1780390624.
33. Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52(9): 2873-2881. doi: 10.1002/art.21264.
34. Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol. 2009;145(5):545-550. doi: 10.1001/archdermatol.2009.79.
35. Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18(6):606-613. doi: 10.1097/01.bor.0000245727.40630.c3.
36. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70(11):1068-1076 doi: 10.4065/70.11.1068.
37. Berianu F, Cohen MD, Abril A, Ginsburg WW. Eosinophilic fasciitis: clinical characteristics and response to methotrexate. Int J Rheum Dis. 2015;18(1):91-98. doi: 10.1111/1756-185X.12499.
38. Wu EY, Rabinovich EC, Torok KS, et al. Description of the localized scleroderma subgroup of the CARRAnet. Arthritis Rheumatol. 2011;63(Suppl 10):S787-88.
39. Weibel L, Harper JI. Linear morphoea follows Blaschko's lines. Br J Dermatol. 2008;159(1):175-181. doi: 10.1111/j.1365-2133.2008.08647.x.
40. Jablonska S, Blaszczyk M. Long-lasting follow-up favours a close relationship between progressive facial hemiatrophy and scleroderma en coup de sabre. J Eur Acad Dermatol Venereol. 2005; 19(4):403-404. doi: 10.1111/j.1468-3083.2005.00979.x.
41. Sommer A, Gambichler T, Bacharach-Buhles M, et al. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. 2006;54: 227-233. doi: 10.1016/j.jaad.2005.10.020.
42. 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(2):257-263. doi: 10.1016/j.jaad.2006.10.959.
43. Wollina U, Buslau M, Weyers W, et al. Squamous cell carcinoma in panscleroticmorphea of childhood. Pediatr Dermatol. 2002; 19(2):151-154. doi: 10.1046/j.1525-1470.2002.00033.x.
44. Parodi PC, Riberti C, Draganic Stinco D, et al. Squamous cell carcinoma arising in a patient with long standing pansclerotic morphea. Br J Dermatol. 2001;144(2):417-419. doi: 10.1046/j.1365-2133.2001.04041.x.
45. Constantin T, Foeldvari I, Pain CE, et al. Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr. 2018;177(7):961-977. doi: 10.1007/s00431-018-3144-8.
46. Li S, Torok K, Pope E, et al. Development of a clinical disease activity measure for juvenile localized scleroderma. Arthritis Rheum. 2011;63(Suppl 10):2453.
47. Weibel L, Sampaio MC, Visentin MT, et al. Evaluation of methotrexate and corticosteroidsfor the treatment of localized scleroderma (morphoea) in children. Br J Dermatol. 2006;155(5): 1013-1020. doi: 10.1111/j.1365-2133.2006.07497.x.
48. Kelsey CE, Torok KS. The localized scleroderma cutaneous assessment tool: responsiveness to change in a pediatric clinical population. J Am Acad Dermatol. 2013;69(2):214-220. doi: 10.1016/j.jaad.2013.02.007.
49. Li SC, Andrews T, Chen M, et al. Extracutaneous involvement is common in juvenile localized scleroderma and associated with a higher level of perceived disease impact. Arthritis Rheumatol. 2017;69(suppl 10). Avlable from: https://acrabstracts.org/abstract/extracutaneous-involvement-is-common-in-juvenile-localized-scleroderma-and-associated-with-a-higher-level-of-per-ceived-disease-impact/.
50. Foster TD. The effects of hemifacial atrophy on dental growth. Br Dent J. 1979;146(5):148-150. doi: 10.1038/sj.bdj.4804213.
51. You KH, Baik HS. Orthopedic and orthodontic treatment of Parry-Romberg syndrome. J Craniofac Surg. 2011;22(3):970-973. doi: 10.1097/SCS.0b013e31820fe339.
52. Grippaudo C, Deli R, Grippaudo FR, et al. Management of craniofacial development in the Parry-Romberg syndrome: report of two patients. Cleft Palate Craniofac J. 2004;41(1):95-104. doi: 10.1597/02-066.
53. Trainito S, Favero L, Martini G, et al. Odontostomatologic involvement in juvenile localised scleroderma of the face. J Paediatr Child Health. 2012;48(7):572-576. doi: 10.1111/j.1440-1754.2012.02435.x.
54. Zannin ME, Martini G, Athreya BH, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007;91(10):1311-1314. doi: 10.1136/bjo.2007.116038.
55. Nelson SP, Chen EH, Syniar GM, Christoffel KK. Prevalence of symptoms of gastroesophageal reflux during childhood: a pediatric practice-based survey. Pediatric practice Res Group. Arch Pediatr Adolesc Med. 2000;154(2):150-154 doi: 10.1001/arch-pedi.154.2.150.
56. Hayashi M, Ichiki Y, Kitajima Y. Coexistence of recurrent generalized morphea and systemic sclerosis. Acta Derm Venereol. 2009;l89(3):329-330. doi: 10.2340/00015555-0645.
57. Carneiro S, Ramos-e-Silva M, Russi DC, et al. Coexistence of generalized morphea and lichen sclerosus et atrophicus mimicking systemic disease. Skinmed. 2011;9(2):131-133.
58. Wu EY, Li SC, Torok KS, et al. Baseline description of the juvenile localized scleroderma subgroup of the CARRA registry. ACR Open Rheumatol. 2019;1(2):119-124. doi: 10.1002/acr2.1019.
59. Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford). 2005;44(3):274-279. doi: 10.1093/rheumatology/keh487.
60. Wortsman X, Wortsman J, Sazunic I, Carreno L. Activity assessment in morphea using color doppler ultrasound. J Am Acad Dermatol. 2011;65(5):942-948. doi: 10.1016/j.jaad.2010.08.027.
61. 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(5):735-742. doi: 10.1002/acr.20407.
62. Serralta San MG, Lebrato J Canora. Clinical ultrasonography in systemic autoimmune diseases. Rev Clin Esp. 2019;S0014-2565(19)30223-30221. doi: 10.1016/j.rce.2019.07.014.
63. Horger M, Fierlbeck G, Kuemmerle-Deschner J, et al. MRI findings in deep and generalized morphea (Localized Scleroderma). Am J Roentgenol. 2008;190(1):32-39. doi: 10.2214/AJR.07.2163.
64. 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.11102136.
65. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M, Luis R Espinoza. Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship? J Rheumatol. 2003;30(1):193-195.
66. Garcia-Romero MT, Randhawa HK, Laxer R, Pope E. The role of local temperature and other clinical characteristics of localized scleroderma as markers of disease activity. Int J Dermatol. 2017;56(1):63-67. doi: 10.1111/ijd.13452.
67. Martini G, Foeldvari I, Russo R, et al. Systemic sclerosis in childhood: clinical and immunological features of 153 patients in an international database. Arthritis Rheum. 2006;54(12):3971-3978. doi: 10.1002/art.22207.
68. Zulian F, Meneghesso D, Grisan E, et al. A new computerized method for the assessment of skin lesions in localized scleroderma. Rheumatology (Oxford). 2007;46(5):856-860. doi: 10.1093/rheumatology/kel446.
69. McKee P, Calonje J, Granter S. Localized scleroderma. In: McKee P, Calonje E, Granter S. Pathology of the skin, with clinical correlations. 3rd ed. Philadelphia, PA: Mosby; 2005. Р. 815-821.
70. 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(2):364-373. doi: 10.1016/j.jaad.2010.02.065.
71. Ingegnoli F, Boracchi P, Gualtierotti R, et al. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adultonset systemic sclerosis: a EUSTAR exploratory study. Microvasc Res. 2015;102:19-24. doi: 10.1016/j.mvr.2015.07.007.
72. Dolezalova P, Young SP, Bacon PA, Southwood TR. Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis. 2003;62(5):444-449. doi: 10.1136/ard.62.5.444.
73. Scalapino K, Arkachaisri T, Lucas M, et al. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33(5):1004-1013.
74. Mertens JS, Seyger MM, Kievit W, et al. Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br J Dermatol. 2015;172(3): 722-728. doi:10.1111/bjd.13514.
75. Huyenlan D, Stephen MP, Chung C, Andrea LZ. Pediatric lichen sclerosus: a review of the literature and management recommendations. Clin Aesthet Dermatol. 2016;9(9):49-54.
76. Kirtschig G. Lichen sclerosus — presentation, diagnosis and management. Dtsch Arztebl Int. 2016;113(19):337-343. doi: 10.3238/arztebl.2016.0337.
77. Federal'nyye klinicheskiye rekomendatsii po vedeniyu bol'nykh vitiligo. Moscow: Rossiyskoye obshchestvo dermatovenerologov i kosmetologov; 2013. (In Russ). Доступно по: https://mz19.ru/upload/iblock/d1d/vit.pdf. Ссылка активна на 12.02.2020.
78. Moniuszko AM, Czupryna P, Dunaj J, et al. Acrodermatitis chronica atrophicans: various faces of a late form of Lyme borreliosis. Postepy Dermatol Alergol. 2018;35(5):490-494. doi: 10.5114/ada.2018.77240.
79. David P Arps, Rajiv M Patel. Lupus Profundus (panniculitis): a potential mimic of subcutaneous panniculitis-like T-Cell lymphoma. Arch Pathol Lab Med. 2013;137(9):1211-1215. doi: 10.5858/arpa.2013-0253-CR.
Review
For citations:
Raupov R.K., Imelbaev A.I., Kostik M.M. Juvenile Localized Scleroderma from the Perspective of Pediatric Rheumatologist. Aspects of Diagnostics. Current Pediatrics. 2020;19(2):150-161. (In Russ.) https://doi.org/10.15690/vsp.v19i2.2109