The skin: A silent witness in systemic sclerosis
DOI:
https://doi.org/10.29176/2590843X.291Keywords:
Sclerosis, connective tissue, systemic scleroderma, limited scleroderma, diffuse sclerodermaAbstract
There are various cutaneous findings that suggest the diagnosis of systemic sclerosis to the dermatologist; among them are described the limitation of mouth opening, reduction of facial wrinkles, Raynaud´s phenomenon, sclerodactyly, nailfold capillaroscopy changes, calcifications and changes in the skin pigmentation and cutaneous sclerosis. It is important to know many of these dermatological findings since they allow us to guide the diagnosis, staging the disease, indicating early treatment and assess the prognosis. In this article we review the dermatological findings in patients with systemic sclerosis.
Author Biographies
Claudia Marcela Arenas, Centro Dermatológico Federico Lleras
Médica dermatóloga, especialista en Docencia Universitaria, Centro Dermatológico Federico Lleras Acosta, Bogotá, D.C., Colombia
Paola Andrea Olaya, Fundación Universitaria Sanitas
Médica, residente de tercer año de Dermatología, Fundación Universitaria Sanitas-Centro Dermatológico Federico Lleras Acosta, Bogotá, D.C., Colombia.
References
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2. Unanue L, González MR, Gardeazábal J. Esclerodermia (sclerosis sistémica). Piel. 2010;25:252-66.
3. Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011;6:509-37.
4. Szekanecz Z, Koch AE. Endothelial cells in inflammation and angiogenesis. Curr Drug Targets. 2005;4:319-23.
5. Sticherling M. Systemic sclerosis - dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges. 2012;10:705-16.
6. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003;29:239-54.
7. Chifflot H, Fautzi B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: A systematic literature review. Semin Arthritis Reum. 2008;37:223-35.
8. Laing TJ, Gillespie BW, Toth MB, Mayes MD, Gallavan Jr RH, Burns CJ, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 1997;40:734-42.
9. Vancheeswaran R, Black CM, David J, Hasson N, Harper J, Atherton D, et al. Childhood-onset scleroderma: Is it different from adult-onset disease. Arthritis Rheum. 1996;39:1041-9.
10. Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino Jr AV, et al. Childhood onset systemic sclerosis: Classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33:1004-13.
11. Janet E. Pope, Sindhu R. Johnson. New Classification Criteria for Systemic Sclerosis (Scleroderma). Rheum Dis Clin N Am 41. 2015; 383–98.
12. LeRoy EC, Medsger Jr TA. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28:1573.
13. Poormoghim H, Lucas M, Fertig N, Medsger Jr TA. Systemic sclerosis sine scleroderma: Demographic, clinical and serologic features and survival in forty-eight patients. Arthritis Rheum. 2000;43:444-51.
14. Arnson Y, Amital H, Guiducci S, Matucci-Cerinic M, Valentini G, Barzilai O, et al. The role of infections in the immunopathogenesis of systemic sclerosis evidence from serological studies. Ann N Y Acad Sci. 2009;1173:627-32.
15. Mora GF. Systemic sclerosis: Environmental factors. J Rheumatol. 2009;36:2383-96.
16. Viswanath V, Phiske M, Gopalani V. Systemic sclerosis: Current concepts in pathogenesis and therapeutic aspects of dermatology manifestations. Indian J Dermatol. 2013;58:255-69.
17. Mahoney WM Jr, Fleming JN, Schwartz SM. A unifying hypothesis for scleroderma: identifying a target cell for scleroderma. Curr Rheumatol Rep. 2011;13:28-36.
18. Wigley FM. Clinical practice. Raynaud’s phenomenon. N Engl J Med. 2002;347:1001-8.
19. Tolosa C, Simeon C, Gabarro L. El fenómeno de Raynaud. Med Clin. 2009;132:712-8.
20. Nietsche A. Raynaud, úlceras digitales y calcinosis en esclerodermia. Reumatol Clinic. 2012;8:270-7.
21. Strange G, Nash P. The manifestation of vasculophaty in systemic sclerosis and its evidence based therapy. Int J Rheum Dis. 2009;12:192-206.
22. Jones S. Skin manifestations of systemic disease. Medicine. 2004;32:40-3.
23. Chung L, Lin J, Furst D, Fiorentino D. Systemic and localizated scleroderma. Clin Dermatol. 2006;24:374-92.
24. Krieg T, Takehara K. Skin disease: A cardinal feature of systemic sclerosis. Rheumatology. 2009;48:14-8.
25. Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: Overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009;4(Suppl.3):19-24.
26. Arenas C, Jiménez J, González MI. Úlcera digital como manifestación inicial de esclerodermia sistémica cutánea límitada. Rev Asoc Colomb Dermatol.2013;21:360-3.
27. Amanzi L, Braschi F, Fiori G, Galluccio F, Miniati I, Giudicci S, et al. Digital ulcers in scleroderma: Staging, characteristics and sub-setting through observation of 1614 lesions. Rheumatology. 2010;49:1374-82.
28. Alivernini S, De Santis M, Tolusso B, Mannocci A, Bosello SL, Peluso G, et al. Skin ulcers in systemic sclerosis: Determinants of presence and predictive factors of healing. J Am Acad Dermatol. 2009;60:426-35.
29. Tabata H, Hara N, Otsuka S, Yamakage A, Yamazaki S, Koibuchi N. Correlation between diffuse pigmentation and keratinocyte derived endothelin-1 in systemic sclerosis. Int J Dermatol. 2000;39:899-902.
30. Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Artritis Rheum. 2005;34:805-12.
31. Davies CA, Jeziorska M, Freemont AJ, Herrick AL. Expression of osteonectin and matrix Gla protein in scleroderma patients with and without calcinosis. Rheumatology.2006;45:1349-55.
32. Czirjak L, Foeldvari I, Muller-Ladner U. Skin Involvement in systemic sclerosis. Rheumatology. 2008;47:44-5.
33. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am.2003;29:391-408.
34. Clements PJ, Furst DE, Campion DS, Bohan A, Harris R, Levy J, Paulus HE. Muscle disease in progressive systemic sclerosis: Diagnostic and therapeutic considerations. Arthritis Rheum.1978;21:62-71.
35. Denton C, Black C. Scleroderma (Systemic sclerosis). In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick´s Dermatology in General Medicine. Seventh edition. New York: McGraw-Hill; 2008. p. 1553-62.
36. Chojnowski MM, Felis-Giemza A, Olesinska M. Capillaroscopy-a role in modern rheumatology. Rheum.2016;54:67-72.
37. Cutulo M, Sulli A, Pizzorni C, Accardo S. Nailfold videocapillaroscopy assesment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27:155-160.
38. Sebastiani M, Manfredi A, Colaci M, D’amico R, Malagoli V, Giuggioli D, Ferri C. Capillaroscopic skin ulcer risk index: A new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum.2009;61:688-94.
39. Sebastiani M, Manfredi A, Vukatana G, Moscatelli S, Riato L, Bocci M,et al. Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: A multicenter validation study. Ann Rheum Dis.2012;71:67-70.
40. Sticherling M. Systemic sclerosis - focus on dermatological aspects. Part 2: Diagnostics, therapy. J Dtsch Dermatol Ges. 2012;10:783-91.
41. Klein-Weigel P, Opitz C, Riemekasten G. Systemic sclerosis: A systematic overview. Part 1. Disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance. Vasa. 2011;40:6-19.
42. Grassegger A, Pohla-Gubo G, Frauscher M, Hintner H. Autoantibodies in systemic sclerosis (scleroderma): Clues for clinical evaluation, prognosis and pathogenesis. Wien Med Wochenschr. 2008;158:19-28.
43. Ho KT, Reveille JD. The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther. 2003;5:80-93
44. Czömpóly T, Simon D, Czirják L, Németh P. Anti-topoisomerasa I autoantibodies in systemic sclerosis. Autoimmun Rev. 2009;8:692-6.
45. Black CM. Measurement of skin involvement in scleroderma. J Rheumatol. 1995;22:1217-9.
46. Amjadi S, Maranian P, Furst DE, Clements PJ, Wong WK, Postlethwaite AE, et al. Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: Analysis of three large multicenter, double-blind, randomized con- trolled trials. Arthritis Rheum. 2009;60:2490-8.
47. Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66:754-63.
48. Vitiello M, Abuchar A, Santana N, Dehesa L, Kerdel FA. An update on the treatment of the cutaneous manifestation of systemic sclerosis: The dermatologist’s point of view. J Clin Aesthet Dermatol. 2012;5:33-43.
49. Kowal-Bieleka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68:620-8.
50. Meyer O. Prognostic markers for systemic sclerosis. Joint Bone Spine. 2006;73:490-4.
51. Steen VD. The lung in systemic sclerosis. J Clin Rheumatol. 2005;11:40-6.
52. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger Jr TA, Lucas M, Michet CJ, et al. Mortality in systemic sclerosis: An international meta-analysis of individual patient data. Am J Med. 2005;118:2-10.
2. Unanue L, González MR, Gardeazábal J. Esclerodermia (sclerosis sistémica). Piel. 2010;25:252-66.
3. Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011;6:509-37.
4. Szekanecz Z, Koch AE. Endothelial cells in inflammation and angiogenesis. Curr Drug Targets. 2005;4:319-23.
5. Sticherling M. Systemic sclerosis - dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges. 2012;10:705-16.
6. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003;29:239-54.
7. Chifflot H, Fautzi B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: A systematic literature review. Semin Arthritis Reum. 2008;37:223-35.
8. Laing TJ, Gillespie BW, Toth MB, Mayes MD, Gallavan Jr RH, Burns CJ, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 1997;40:734-42.
9. Vancheeswaran R, Black CM, David J, Hasson N, Harper J, Atherton D, et al. Childhood-onset scleroderma: Is it different from adult-onset disease. Arthritis Rheum. 1996;39:1041-9.
10. Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino Jr AV, et al. Childhood onset systemic sclerosis: Classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33:1004-13.
11. Janet E. Pope, Sindhu R. Johnson. New Classification Criteria for Systemic Sclerosis (Scleroderma). Rheum Dis Clin N Am 41. 2015; 383–98.
12. LeRoy EC, Medsger Jr TA. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28:1573.
13. Poormoghim H, Lucas M, Fertig N, Medsger Jr TA. Systemic sclerosis sine scleroderma: Demographic, clinical and serologic features and survival in forty-eight patients. Arthritis Rheum. 2000;43:444-51.
14. Arnson Y, Amital H, Guiducci S, Matucci-Cerinic M, Valentini G, Barzilai O, et al. The role of infections in the immunopathogenesis of systemic sclerosis evidence from serological studies. Ann N Y Acad Sci. 2009;1173:627-32.
15. Mora GF. Systemic sclerosis: Environmental factors. J Rheumatol. 2009;36:2383-96.
16. Viswanath V, Phiske M, Gopalani V. Systemic sclerosis: Current concepts in pathogenesis and therapeutic aspects of dermatology manifestations. Indian J Dermatol. 2013;58:255-69.
17. Mahoney WM Jr, Fleming JN, Schwartz SM. A unifying hypothesis for scleroderma: identifying a target cell for scleroderma. Curr Rheumatol Rep. 2011;13:28-36.
18. Wigley FM. Clinical practice. Raynaud’s phenomenon. N Engl J Med. 2002;347:1001-8.
19. Tolosa C, Simeon C, Gabarro L. El fenómeno de Raynaud. Med Clin. 2009;132:712-8.
20. Nietsche A. Raynaud, úlceras digitales y calcinosis en esclerodermia. Reumatol Clinic. 2012;8:270-7.
21. Strange G, Nash P. The manifestation of vasculophaty in systemic sclerosis and its evidence based therapy. Int J Rheum Dis. 2009;12:192-206.
22. Jones S. Skin manifestations of systemic disease. Medicine. 2004;32:40-3.
23. Chung L, Lin J, Furst D, Fiorentino D. Systemic and localizated scleroderma. Clin Dermatol. 2006;24:374-92.
24. Krieg T, Takehara K. Skin disease: A cardinal feature of systemic sclerosis. Rheumatology. 2009;48:14-8.
25. Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: Overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009;4(Suppl.3):19-24.
26. Arenas C, Jiménez J, González MI. Úlcera digital como manifestación inicial de esclerodermia sistémica cutánea límitada. Rev Asoc Colomb Dermatol.2013;21:360-3.
27. Amanzi L, Braschi F, Fiori G, Galluccio F, Miniati I, Giudicci S, et al. Digital ulcers in scleroderma: Staging, characteristics and sub-setting through observation of 1614 lesions. Rheumatology. 2010;49:1374-82.
28. Alivernini S, De Santis M, Tolusso B, Mannocci A, Bosello SL, Peluso G, et al. Skin ulcers in systemic sclerosis: Determinants of presence and predictive factors of healing. J Am Acad Dermatol. 2009;60:426-35.
29. Tabata H, Hara N, Otsuka S, Yamakage A, Yamazaki S, Koibuchi N. Correlation between diffuse pigmentation and keratinocyte derived endothelin-1 in systemic sclerosis. Int J Dermatol. 2000;39:899-902.
30. Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Artritis Rheum. 2005;34:805-12.
31. Davies CA, Jeziorska M, Freemont AJ, Herrick AL. Expression of osteonectin and matrix Gla protein in scleroderma patients with and without calcinosis. Rheumatology.2006;45:1349-55.
32. Czirjak L, Foeldvari I, Muller-Ladner U. Skin Involvement in systemic sclerosis. Rheumatology. 2008;47:44-5.
33. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am.2003;29:391-408.
34. Clements PJ, Furst DE, Campion DS, Bohan A, Harris R, Levy J, Paulus HE. Muscle disease in progressive systemic sclerosis: Diagnostic and therapeutic considerations. Arthritis Rheum.1978;21:62-71.
35. Denton C, Black C. Scleroderma (Systemic sclerosis). In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick´s Dermatology in General Medicine. Seventh edition. New York: McGraw-Hill; 2008. p. 1553-62.
36. Chojnowski MM, Felis-Giemza A, Olesinska M. Capillaroscopy-a role in modern rheumatology. Rheum.2016;54:67-72.
37. Cutulo M, Sulli A, Pizzorni C, Accardo S. Nailfold videocapillaroscopy assesment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27:155-160.
38. Sebastiani M, Manfredi A, Colaci M, D’amico R, Malagoli V, Giuggioli D, Ferri C. Capillaroscopic skin ulcer risk index: A new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum.2009;61:688-94.
39. Sebastiani M, Manfredi A, Vukatana G, Moscatelli S, Riato L, Bocci M,et al. Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: A multicenter validation study. Ann Rheum Dis.2012;71:67-70.
40. Sticherling M. Systemic sclerosis - focus on dermatological aspects. Part 2: Diagnostics, therapy. J Dtsch Dermatol Ges. 2012;10:783-91.
41. Klein-Weigel P, Opitz C, Riemekasten G. Systemic sclerosis: A systematic overview. Part 1. Disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance. Vasa. 2011;40:6-19.
42. Grassegger A, Pohla-Gubo G, Frauscher M, Hintner H. Autoantibodies in systemic sclerosis (scleroderma): Clues for clinical evaluation, prognosis and pathogenesis. Wien Med Wochenschr. 2008;158:19-28.
43. Ho KT, Reveille JD. The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther. 2003;5:80-93
44. Czömpóly T, Simon D, Czirják L, Németh P. Anti-topoisomerasa I autoantibodies in systemic sclerosis. Autoimmun Rev. 2009;8:692-6.
45. Black CM. Measurement of skin involvement in scleroderma. J Rheumatol. 1995;22:1217-9.
46. Amjadi S, Maranian P, Furst DE, Clements PJ, Wong WK, Postlethwaite AE, et al. Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: Analysis of three large multicenter, double-blind, randomized con- trolled trials. Arthritis Rheum. 2009;60:2490-8.
47. Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66:754-63.
48. Vitiello M, Abuchar A, Santana N, Dehesa L, Kerdel FA. An update on the treatment of the cutaneous manifestation of systemic sclerosis: The dermatologist’s point of view. J Clin Aesthet Dermatol. 2012;5:33-43.
49. Kowal-Bieleka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68:620-8.
50. Meyer O. Prognostic markers for systemic sclerosis. Joint Bone Spine. 2006;73:490-4.
51. Steen VD. The lung in systemic sclerosis. J Clin Rheumatol. 2005;11:40-6.
52. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger Jr TA, Lucas M, Michet CJ, et al. Mortality in systemic sclerosis: An international meta-analysis of individual patient data. Am J Med. 2005;118:2-10.
How to Cite
1.
Arenas CM, Olaya PA. The skin: A silent witness in systemic sclerosis. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2016 Oct. 1 [cited 2024 Jul. 3];24(4):279-8. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/291
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2016-10-01
How to Cite
1.
Arenas CM, Olaya PA. The skin: A silent witness in systemic sclerosis. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2016 Oct. 1 [cited 2024 Jul. 3];24(4):279-8. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/291
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