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Systemic manifestations in localized scleroderma

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Abstract

In this paper, the various systemic manifestations reported in localized scleroderma, their incidence, their relationship with systemic sclerosis, and their relationship with other autoimmune or connective tissue diseases will be analyzed.

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References and Recommended Reading

  1. Cassidy JT, Petty RE: The systemic sclerodermas and related disorders. In Textbook of Pediatric Rheumatology. Edited by Cassidy JT, Petty RE. Philadelphia: W.B. Saunders Co.; 2001:505–534.

    Google Scholar 

  2. Martini G, Foeldvari I, Russo R, et al.: Systemic scleroderma syndromes (SSS) in children: clinical and immunological characteristics of 181 patients (abstract). Arthritis Rheum 2003, 48:S512.

    Google Scholar 

  3. Nelson A: Localized sclerodermas. In Textbook of Pediatric Rheumatology. Edited by Cassidy JT, Petty RE. Philadelphia: W.B. Saunders Co.; 2001:535–544.

    Google Scholar 

  4. Zulian F, De Oliveira SKF, Lehman TH, et al.: Juvenile localized scleroderma: clinical epidemiological features of 688 patients (abstract). Arthritis Rheum 2003, 48:S512.

    Google Scholar 

  5. Lunderschmidt C, König G, Leisner B et al.: Zirkumskripte sklerodermie: interne manifestationen und signifikante correlation zu HLA-DR1 und -DR5. Der Hautarzt 1985, 36:516–521.

    Google Scholar 

  6. Dehen L, Roujeau JC, Cosnes A, et al.: Internal involvement in localized scleroderma. Medicine 1994, 73:241–245. The first interesting study that evaluated systematically the prevalence of internal organ involvement in LS.

    Article  PubMed  CAS  Google Scholar 

  7. Zulian F, Russo R, Laxer R, et al.: Is juvenile localized scleroderma really "localized"? (abstract). Arthritis Rheum 2003, 48:S512. Despite this being in the form of an abstract, this preliminary study describes the extracutaneous manifestations of children with LS as a result of a multi-center multinational survey.

    Google Scholar 

  8. Heron E, Hernigou A, Fornes P, et al.: Central nervous system involvement in scleroderma. Ann Med Interne (Paris) 2002, 153:179–182.

    Google Scholar 

  9. Higashi Y, Kanekura T, Fukumaru K, Kanzaki T: Scleroderma en coup de sabre with central nervous system involvement. J Dermatol 2000, 27:486–488.

    PubMed  CAS  Google Scholar 

  10. Kanzato N, Matsuzaki T, Komine Y, et al.: Localized scleroderma associated with progressing ischemic stroke. J Neurol Sci 1999, 163:86–89.

    Article  PubMed  CAS  Google Scholar 

  11. Suttorp-Schulten MS, Koornneef L: Linear scleroderma associated with ptosis and motility disorders. Br J Ophthalmol 1990, 74:694–695.

    PubMed  CAS  Google Scholar 

  12. Littman BH: Linear scleroderma: a response to neurologic injury? Report and literature review. J Rheumatol 1989, 16:1135–1140.

    PubMed  CAS  Google Scholar 

  13. Fry JA, Alvarellos A, Fink CW, et al.: Intracranial findings in progressive facial hemiatrophy. J Rheumatol 1992, 19:856–858.

    Google Scholar 

  14. David J, Wilson J, Woo P: Scleroderma "en coup de sabre." Ann Rheum Dis 1991, 50:260–262.

    Article  PubMed  CAS  Google Scholar 

  15. Blaszczyk M, Krolicki L, Krasu M, et al.: Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol 2003, 30:1997–2004. A clear-cut demonstration that CNS involvement is present in all cases of PFH preceeded or not by sclerotic lesions, and in scleroderma ECDS with no signs of PFH, thus confirming a close relationship between these conditions.

    PubMed  Google Scholar 

  16. Liu P, Uziel Y, Chuang S, et al.: Localized scleroderma: imaging features. Pediatr Radiol 1994, 24:207–209.

    Article  PubMed  CAS  Google Scholar 

  17. Chung MH, Sum J, Morrell MJ, Horoupian DS: Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. Ann Neurol 1995, 37:679–681. An interesting case report of an epileptic patient with scleroderma ECDS with densely sclerotic cerebral lesion with thickened blood vessel walls surrounded by gliotic parenchyma and calcifications. The surgical resection of the involved brain area resulted in epilepsy regression.

    Article  PubMed  CAS  Google Scholar 

  18. Luer W, Jockel D, Henze T, Schipper HI: Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head. J Neurol 1990, 237:379–381.

    Article  PubMed  CAS  Google Scholar 

  19. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M, Espinoza LR: Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship? J Rheumatol 2003, 30:193–195. An interesting case report that raises some questions on the origin and relationship of LS and brain calcification. Whether they are secondary to the cerebrovascular damage of scleroderma or represent a separate mucocutaneous syndrome of vascular dysplasis awaits further elucidation.

    PubMed  Google Scholar 

  20. Grosso S, Fioravanti A, Biasi G, et al.: Linear scleroderma associated with progressive brain atrophy. Brain Dev 2003, 25:57–61.

    Article  PubMed  Google Scholar 

  21. Goldberg-Stern H, deGrauw T, Passo M, Ball WS Jr.: Parry-Romberg syndrome: follow-up imaging during suppressive therapy. Neuroradiology 1997, 39:873–876.

    Article  PubMed  CAS  Google Scholar 

  22. Woolfenden AR, Tong DC, Norbash AM, Albers GW: Progressive facial hemiatrophy: abnormality of intracranial vasculature. Neurology 1998, 50:1915–1917.

    PubMed  CAS  Google Scholar 

  23. Dupont S, Catala M, Hasboun D, et al.: Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism. Neurology 1997, 48:1013–1018.

    PubMed  CAS  Google Scholar 

  24. DeFelipe J, Segura T, Arellano JI, et al.: Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. Epilepsia 2001, 42:1198–1203.

    Article  PubMed  CAS  Google Scholar 

  25. Gambichler T, Kreuter A, Hoffmann K, et al.: Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications. BMC Dermatol 2001, 1:9.

    Article  PubMed  CAS  Google Scholar 

  26. Menni S, Marzano AV, Passoni E: Neurologic abnormalities in two patients with facial hemiatrophy and sclerosis coexisting with morphea. Pediatr Dermatol 1997, 14:113–116.

    PubMed  CAS  Google Scholar 

  27. Schievink WI, Mellinger JF, Atkinson JL: Progressive intracranial aneurysmal disease in child with progressive hemifacial atrophy: case report. Neurosurgery 1996, 38:1237–1241.

    Article  PubMed  CAS  Google Scholar 

  28. Stone J, Franks AJ, Guthrie JA, Johnson MH: Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry 2001, 70:382–385.

    Article  PubMed  CAS  Google Scholar 

  29. Miedziak AI, Stefanyszyn M, Flamagan J, Eagle RC, Jr.:Parry-Romberg syndrome associated with intracranial vascular malformations. Arch Ophthalmol 1998, 116:1235–1237.

    PubMed  CAS  Google Scholar 

  30. Rees TD: Facial atrophy. Clin Plast Surg 1976, 3:637.

    PubMed  CAS  Google Scholar 

  31. Muchnick RS, Sherrell JA, Rees DT: Ocular manifestations and treatment of hemifacial atrophy. Am J Ophthalmol 1979, 88:889–897.

    PubMed  CAS  Google Scholar 

  32. West RH, Barnett AJ: Ocular involvement in scleroderma. Br J Ophtalmol 1979, 63:845–847.

    CAS  Google Scholar 

  33. Lecky BE, Hughes RA, Murray NM: Trigeminal sensory neuropathy. A study of 22 cases. Brain 1987, 110:1463–1485.

    Article  PubMed  Google Scholar 

  34. Nancarrow JD, Jawad SM: A rare case of severe bilateral ectropion from scleroderma. Plast Reconstr Surg 1981, 67:352–354.

    Article  PubMed  CAS  Google Scholar 

  35. Ramboer K, Demaerel P, Baert AL, et al.: Linear scleroderma with orbital involvement: follow-up and magnetic resonance imaging. Br J Ophthalmol 1997, 81:90–91.

    PubMed  CAS  Google Scholar 

  36. Suttorp-Schulten MS, Koorn L: Linear scleroderma associated with ptosis and motility disorders. Br J Ophthalmol 1990, 74:694–695.

    PubMed  CAS  Google Scholar 

  37. Haim S, Sherf K: Scleroderma "en plaque" associated with systemic manifestation. Harefuah 1965, 69:172–174.

    Google Scholar 

  38. Tuffanelli DL, Marmelzat WL, Dorsey CS: Linear scleroderma with hemiatrophy: report of three cases associated with collagen-vascular disease. Dermatologica 1966, 132:51–58.

    PubMed  CAS  Google Scholar 

  39. Zaninotto G, Peserico A, Costantini M, et al.: Oesophageal motilit`a and lower oesophageal sphincter competence in progressive systemic sclerosis and localized sclerodermia. Scand J Gastroenterol 1989, 24:95–102.

    PubMed  CAS  Google Scholar 

  40. Weber P, Ganser G, Frosh M, et al.: Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol 2000, 27:2692–2695.

    PubMed  CAS  Google Scholar 

  41. Bourgeois-Droin C, Touraine R: Sclérodermie en plaque: Perturbations immunologique et viscérales. Ann Med Interne (Paris) 1978, 129:107–112.

    CAS  Google Scholar 

  42. Aguayo SM, Richardson CL, Roman J: Severe extrapulmonary thoracic restriction caused by morphea, a form of localized scleroderma. Chest 1993, 104:1304–1305.

    PubMed  CAS  Google Scholar 

  43. Moore EC, Altri E: Focal scleroderma and severe cardiomyopathy: patient report and brief review. Am J Dis Child 1991, 145:229–231.

    PubMed  CAS  Google Scholar 

  44. Rokicki W, Dukalska M, Rubisz-Brzezinska J, Gasior Z: Circulatory system in children with localized scleroderma. Pediatr Cardiol 1997, 18:213–217.

    Article  PubMed  CAS  Google Scholar 

  45. Basu S, Ganguly S: Homolateral linear morphea and coarctation of aorta. Indian Pediatr 2003, 40:1108–1109.

    PubMed  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  47. Uziel Y, Krafchik BR, Silverman ED, et al.: Localized scleroderma in childhood: a report of 30 cases. Semin Arthritis Rheum 1994, 23:328–340.

    Article  PubMed  CAS  Google Scholar 

  48. Vancheeswaran R, Black CM, David J, et al.: Childhood onset scleroderma: is it different from adult onset disease. Arthritis Rheum 1996, 39:1041–1049.

    Article  PubMed  CAS  Google Scholar 

  49. Marzano AV, Menni S, Parodi A, et al.: Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 2003, 13:171–176.

    PubMed  Google Scholar 

  50. Falanga V, Medsger TA, Reichlin M, Rodnan GP: Linear scleroderma: clinical spectrum, prognosis and laboratory abnormalities. Ann Intern Med 1986, 104:849–857.

    PubMed  CAS  Google Scholar 

  51. Hanson V, Drexler E, Konreich H: Rheumatoid factor in children with focal scleroderma. Pediatrics 1974, 53:945–947. One of the first reports on the presence of RF in children with LS and its relationship with the course of arthritis observed in some patients.

    PubMed  CAS  Google Scholar 

  52. Christianson HB, Dorsey CS, O’Leary PA, Kierland RR: Localized scleroderma. A clinical study of two hundred thirty-five cases. Arch Dermatol 1956, 74:629–639.

    CAS  Google Scholar 

  53. Curtis AC, Jansen TG: The prognosis of localized scleroderma. Arch Dermatol 1958, 78:749–757.

    CAS  Google Scholar 

  54. Jablonska S, Chorzelski TP, Blaszczyk M, et al.: Clinical relevance of immunologic findings in scleroderma. Clin Dermatol 1993, 10:407–421.

    Article  Google Scholar 

  55. Harrington I, Dunsmore IR: An investigation into the incidence of autoimmune disorders in patients with localized morphea. Br J Dermatol 1989, 120:645–648. An interesting "historical" study on the incidence of autoimmune disorders in a cohort of patients with LS and their relatives.

    Article  PubMed  CAS  Google Scholar 

  56. Scarola JA, Shulman LE: Serologic abnormalities and their significance in localized scleroderma. Arthritis Rheum 1975, 18:526–527.

    PubMed  CAS  Google Scholar 

  57. Blaszczyk M, Jarzabek-Chorleska M, Jablonska S: Relationship between cutaneous and systemic scleroderma. Are immunopathological studies helpful in evaluating transition of cutaneous to systemic scleroderma? Przeglad Dermatologiczny 2000, 2:119–125. An immunologic study of a large series of patients with LS describing the incidence of scleroderma-specific antibodies and their role in predicting the evolution to SSc during a long term follow-up.

    Google Scholar 

  58. Birdi N, Laxer RM, Thorner P, et al.: Localized scleroderma progressing to systemic disease. Case report and review of the literature. Arthritis Rheum 1993, 36:410–415. A well documented case report of a patient with morphea who developed progression to SSc 2 years after initial presentation. The role of antibodies directed against Ku antigens as predictors of possible transition to SSc is discussed.

    Article  PubMed  CAS  Google Scholar 

  59. Temine P, Privat Y, Vallette P: Sclerodermie en plaques et en gouttes avec hypertension arteriell pulmonaire. Bull Soc Fr Dermatol Syphiligr 1970, 77:216–217.

    Google Scholar 

  60. Ikai K, Tagami H, Imamura S, et al.: Morphea-like cutaneous changes in a patient with systemic scleroderma. Dermatologica 1979, 158:438–442.

    PubMed  CAS  Google Scholar 

  61. Ryckmanns F, Konz B: Disseminierte zirkumskripte sklerodermie und diffuse sklerodermie vom typ der akrosklerodermie. Hautarzt 1980, 31:86–90.

    PubMed  CAS  Google Scholar 

  62. Mizutani H, Tanaka H, Okada H, et al.: Palindromic morphea: multiple recurrence of morphea lesions in a case of systemic sclerosis. J Dermatol 1992, 19:298–301.

    PubMed  CAS  Google Scholar 

  63. Tufanelli DL, Marmalrat WL, Dorsey CS: Linear scleroderma with hemiatrophy: report of three cases associated with collagen-vascular disease. Dermatologica 1966, 132:51–58.

    Google Scholar 

  64. Lee HJ, Kim MY, Ha SJ, Kim JW: Two cases of morphea associated with Hashimoto’s thyroiditis. Acta Derm Venereol 2002, 82:58–59.

    Article  PubMed  Google Scholar 

  65. Tremaine R, Adam JE, Orizaga M: Morphea coexisting with lichen sclerosus et atrophicus. Int J Dermatol 1990, 29:486–489.

    Article  PubMed  CAS  Google Scholar 

  66. Dervis E, Acbay O, Barut G, et al.: Association of vitiligo, morphea and Hashimoto’s thyroiditis. Int J Dermatol 2004, 43:236–237.

    Article  PubMed  Google Scholar 

  67. Finkelstein E, Amichai B, Metzker A: Coexistence of vitiligo and morphea: a case report and review of the literature. J Dermatol 1995, 22:351–353.

    PubMed  CAS  Google Scholar 

  68. Lerner AB, Sansung J: Vitiligo and linear scleroderma. Arch Dermatol 1973, 108:286–287.

    Google Scholar 

  69. Thompson DM, Robinson TWE, Lennard-Jones J: Alopecia areata vitiligo, scleroderma and ulcerative colitis. Proc R Soc Med 1974, 67:1010–1012.

    PubMed  CAS  Google Scholar 

  70. Brenner W, Diem F, Gschnait F: Coincidence of vitiligo, alopecia areata, onychodystrophy, localized scleroderma and lichen planus. Dermatologica 1979, 159:356–360.

    Article  PubMed  CAS  Google Scholar 

  71. Reed JR, De Luca N, McIntyre AS, Wilkinson JD: Localized morphea, xantomatosis and primary biliary cirrhosis. Br J Dermatol 2000, 143:652–653.

    Article  PubMed  CAS  Google Scholar 

  72. Kim HS, Chun YS, Hann SK, Park WH: A case of linear scleroderma and myasthenia gravis. J Dermatol 2000, 27:31–34.

    PubMed  CAS  Google Scholar 

  73. De P, Lloyd HJ, Rashid AM, Anstey AV: Morphea presenting shortly after the onset of Schmidt’s syndrome. Clin Exp Dermatol 2000, 25:168–169.

    Article  PubMed  CAS  Google Scholar 

  74. Shelkovitz-Shiloh I, Ehrenfeld M, Schewch-Millet M, et al.: Coexistence of family systemic lupus erythematosus and localized scleroderma. Int J Dermatol 1992, 31:819–820.

    Article  PubMed  CAS  Google Scholar 

  75. Mizutani H, Tanaka H, Okada H, et al.: Palindromic morphea: multiple recurrence of morphea lesions in a case of systemic sclerosis. J Dermatol 1992, 19:298–301.

    PubMed  CAS  Google Scholar 

  76. Mackel SE, Kozin F, Ryan LM, et al.: Concurrent linear scleroderma and systemic lupus erythematosus: a report of two cases. J Invest Dermatol 1979, 73:368–372.

    Article  PubMed  CAS  Google Scholar 

  77. Mork NJ: Clinical and histopathologic morphea with immunological evidence of lupus erythematosus: a case report. Acta Derm Venereol 1981; 61:367–368.

    PubMed  CAS  Google Scholar 

  78. Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S: Coexistent linear scleroderma and juvenile lupus erythematosus. Pediatr Dermatol 2000, 17:565–569.

    Article  Google Scholar 

  79. Wu KH, Dai YS, Tsai MJ, et al.: Lichen sclerosus et atrophicus, bullous morphea and systemic lupus erythematosus: a case report. J Microbiol Immunol Infect 2000, 33:53–56.

    PubMed  CAS  Google Scholar 

  80. Robertson LP, Davies MG, Hickling P: Generalized morphoea in a patient with Felty’s syndrome. J Eur Acad Dermatol Venereol 2000, 14:191–193.

    Article  PubMed  CAS  Google Scholar 

  81. Adebajo AO, Crisp AJ, Nicholls A, Hazleman BL: Localized Scleroderma and hemiatrophy in association with antibodies to double-stranded DNA. Postgrad Med J 1992, 68:216–218.

    Article  PubMed  CAS  Google Scholar 

  82. Hernandez-Beriain JA, San Roman A, Espinel ML, Segura-Garcia C: Morphea and rheumatoid arthritis. Clin Exp Rheumatol 1996, 14:446–447.

    PubMed  CAS  Google Scholar 

  83. Morita A, Tsuji T: Necrotizing vasculitis in a patient with generalized morphea. J Am Acad Dermatol 2001, 45:S215-S217.

    Article  PubMed  CAS  Google Scholar 

  84. Hardford R, Smith KJ, Skelton H: Development of histologic features of scleroderma in congenital lesions. J Cutan Pathol 2002, 29:249–254. Starting from a case report describing two patients, who, over congenital hypopigmented areas, developed LS in adulthood, the authors proposed that somatic mutations affecting vessels may predispose to increased endothelial cell apoptosis leading to autoimmune response.

    Article  Google Scholar 

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  1. Pediatric Rheumatology Unit, Department of Pediatrics, University of Padua, Via Giustiniani 3, 35128, Padova, Italy

    Francesco Zulian MD

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  1. Francesco Zulian MD
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Zulian, F. Systemic manifestations in localized scleroderma. Curr Rheumatol Rep 6, 417–424 (2004). https://doi.org/10.1007/s11926-004-0020-5

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