Publicado

2018-04-01

Systemic scleroderma: An approach from plastic surgery

Esclerodermia sistémica. Abordaje desde la cirugía plástica

Palabras clave:

Scleroderma Systemic, Surgery Plastic, Hand, Leg Ulcer (en)
Esclerodermia sistémica, Cirugía plástica, Mano, Úlcera de la pierna (es)

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Autores/as

  • Karen Rodríguez-Franco Hospital Universitario de Santander Empresa Social del Estado - Plastic Surgery Service - Bucaramanga - Colombia. Universidad Industrial de Santander - Faculty of Health - Surgery and Specialties Research Group GRICES-UIS - Bucaramanga - Colombia.
  • Audrey José Miranda-Díaz Hospital Universitario de Santander Empresa Social del Estado - Plastic Surgery Service - Bucaramanga - Colombia. Universidad Industrial de Santander - Faculty of Health - Surgery and Specialties Research Group GRICES-UIS - Bucaramanga - Colombia.
  • Juan David Hoyos-Restrepo Hospital Universitario de Santander Empresa Social del Estado - Plastic Surgery Service - Bucaramanga - Colombia. Universidad Industrial de Santander - Faculty of Health - Surgery and Specialties Research Group GRICES-UIS - Bucaramanga - Colombia.
  • Genny Liliana Meléndez Hospital Universitario de Santander Empresa Social del Estado - Plastic Surgery Service - Bucaramanga - Colombia. Universidad Industrial de Santander - Faculty of Health - Surgery and Specialties Research Group GRICES-UIS - Bucaramanga - Colombia.

Introduction: Systemic sclerosis (SSc) is an autoimmune disease of the connective tissue that is clinically characterized by dermal thickening, which occurs due to the accumulation of connective tissue, and may involve other organs and the limbs. The multifactorial etiology of this disease is related to alterations in remodeling interactions of the extracellular matrix (ECM), immune function and presence of proliferative vasculopathy under genetic and environmental influence.

Objective: To conduct a literature review on medical and surgical management from the point of view skin, hand and finger lesions surgery, as well as facial alterations in patients with systemic scleroderma.

Materials and methods: A bibliographic search was performed in the Medline, LILACS, PubMed, EMBASE and Current Contents databases, using the keywords “systemic scleroderma”, “plastic surgery”, “hand” and “ulcers”.

Results: SSc requires a multidisciplinary management, since pharmacological therapy is indicated for some cases and complementary surgical management is better for others.

Conclusion: This pathology significantly affects the skin by causing lesions ranging from digital ulcers to facial atrophy, which are susceptible to management by plastic surgery.

Introducción. La esclerosis sistémica (ES) es una enfermedad autoinmune del tejido conectivo que se caracteriza clínicamente por un engrosamiento cutáneo, el cual se da debido a la acumulación de tejido conectivo y puede afectar a otros órganos y a las extremidades. La etiología multifactorial de esta enfermedad corresponde a la interacción de alteraciones en el remodelamiento de la matriz extracelular, función inmunitaria y presencia de vasculopatía proliferativa bajo influencia genética y medioambiental.

Objetivo. Realizar una revisión sobre el manejo médico y quirúrgico desde el punto de vista de la cirugía plástica de las lesiones en piel y manos y las alteraciones faciales de los pacientes con ES.

Materiales y métodos. Se realizó una búsqueda bibliográfica en las bases de datos Medline, LILACS, PubMed, EMBASE y Current contents con las palabras claves esclerodermia sistémica, cirugía plástica, mano y ulceras.

Resultados. La ES requiere manejo multidisciplinario; se presentan casos en los que se indica terapia farmacológica y otros en los que el manejo es quirúrgico complementario.

Conclusión. Esta patología afecta de manera importante la piel al provocar lesiones que van desde ulceras digitales hasta atrofia facial, las cuales son susceptibles de manejo por el área de cirugía plástica.

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Chifflot H, Fautrel B, Sordet C, Chateelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008;37(4):223-35. http://doi.org/bhtnbt.

Zimmermann AF, Pizzichini MM. Atualização na etiopatogênese da esclerose sistêmica. Rev Bras Reumatol. 2013;53(6):516-24. http://doi.org/f2q79x.

Dumoitier N, Lofek S, Mouthon L. Pathophysiology of systemic sclerosis: state of the art in 2014. Presse Med. 2014;43(10 Pt 2):e267-78. http://doi.org/f6thfz.

Wynn TA. Cellular and molecular mechanisms of fibrosis. J Pathol. 2008;214(2):199-210. http://doi.org/cf22mt.

Cox TR, Erler JT. Remodeling and homeostasis of the extracellular matrix: implications for fibrotic diseases and cancer. Dis Model Mech. 2011;4(2):165-78. http://doi.org/fwqcvh.

Hudson M, Fritzler MJ. Diagnostic criteria of systemic sclerosis. J Autoimmun. 2014;48-49:38-41. http://doi.org/cf9s.

Radic M, Martinovic-Kaliterna D, Radic J. Infectious disease as aetiological factor in the pathogenesis of systemic sclerosis. Neth J Med. 2010;68(11):348-53.

Zakrzewska K, Corcioli F, Carlsen KM, Giugguioli D, Fanci R, Rinieri A, et al. Human parvovirus B19 (B19V) infection in systemic sclerosis patients. Intervirology. 2009;52(5):279-82. http://doi.org/dn5gfq.

Levy Y, Rotman-Pikieelny P, Ehrenfeld M, Shoenfeld Y. Silicone breast implantation-induced scleroderma: description of four patients and a critical review of the literature. Lupus. 2009;18(13):1226-32. http://doi.org/bzncqg.

Johnson SR. New ACR EULAR guidelines for systemic sclerosis classification. Curr Rheumatol Rep. 2015;17(5):32. doi: http://doi.org/cf9w.

Tyndall A, Fistarol S. The differential diagnosis of systemic sclerosis. Curr Opin Rheumatol. 2013;25(6):692-9. http://doi.org/f522ng.

Medina YF, Ortiz M, Barrera N, Chalem P, Motta A, Zamora F, et al. Relación de los anticuerpos anti-péptido citrulinado con manifestaciones osteo-articulares en una cohorte de pacientes con esclerodermia. Rev Colomb Reumatol. 2011;18(3):155-62.

Wigley FM. Clinical practice. Raynaud’s Phenomenon. N Engl J Med. 2002;347(13):1001-8. http://doi.org/dmqfqr.

Herrick AL. Contemporary management of Raynaud’s phenomenon and digital ischaemic complications. Curr Opin Rheumatol. 2011;23(6):555-61. http://doi.org/b8472f.

Block JA, Sequeira W. Raynaud’s phenomenon. Lancet. 2001;357(9273):2042-8. http://doi.org/c6h97g.

Botzoris V, Drosos AA. Management of Raynaud’s phenomenon and digital ulcers in systemic sclerosis. Joint Bone Spine. 2011;78(4):341-6. http://doi.org/fft7ht.

Ennis H, Vail A, Wragg E, Taylor A, Moore T, Murray A, et al. A prospective study of systemic sclerosis-related digital ulcers: prevalence, location, and functional impact. Scand J Rheumatol. 2013;42(6):483-6. http://doi.org/cknt.

Nitsche A. Raynaud, úlceras digitales y calcinosis en esclerodermia. Reumatol Clin. 2012;8(5):270-7. http://doi.org/cf9z.

Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009;48(Suppl 3):iii19-24. http://doi.org/d3gpt5.

Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Artritis Rheum. 2005;34(6):805-12. http://doi.org/cwtrqf.

Anandacoomarasamy A, Englert H, Manolios N, Kirkham S. Reconstructive hand surgery for scleroderma joint contractures. J Hand Surg Am. 2007;32(7):1107-12. http://doi.org/cfsf84.

Fox P, Chung L, Chang J. Management of the Hand in Systemic Sclerosis. J Hand Surg Am. 2013;38(5):1012-6. http://doi.org/cf93.

Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium-channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum. 2001;44(8):1841-7. http://doi.org/dznz4z.

Wigley FM, Wise RA, Seibold JR, MacCloskey DA, Kujala G, Medsger TA Jr, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo controlled, double-blind study. Ann Intern Med. 1994;120(3):199-206. http://doi.org/wjq.

Rosenkranz S, Diet F, Karasch T, Weihrauch J, Wassermann K, Erdmann E. Sildenafil improved pulmonary hypertension and peripheral blood flow in a patient with scleroderma-associated lung fibrosis and the Raynaud phenomenon. Ann Intern Med. 2003;139(10):871-3. http://doi.org/cgbr.

Fries R, Shariat K, von Wilmowsky H, Böhm M. Sildenafil in the treatment of Raynaud’s phenomenon resistant to vasodilatory therapy. Circulation. 2005;112(19):2980-5.

Korn JH, Mayes M, Matucci-Cerinic M, Rainisio M, Pope J, Hachulla E, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50(12):3985-93. http://doi.org/dk2wnd.

Ambach A, Seo W, Bonnekoh B, Gollnick H. Low-dose combination therapy of severe digital ulcers in diffuse progressive systemic sclerosis with the endothelin-1 receptor antagonist bosentan and the phosphodiesterase V inhibitor sildenafil. J Dtsch Dermatol Ges. 2009;7(10):888-91. http://doi.org/dksj3g.

Dziadzio M, Denton CP, Smith R, Howell K, Blann A, Bowers E, et al. Losartan therapy for Raynaud’s phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial. Arthritis Rheum. 1999;42(12):2646-55. http://doi.org/fht8zr.

Schmidt J, Monnet P, Normand B, Fabry R. Microcirulatory and clinical effects of serial percutaneous application of carbon dioxide in primary and secondary Raynaud’s phenomenon. Vasa. 2005;34(2):93-100. http://doi.org/dspf4m.

Daigeler A, Meyer M, Joneidi-Jafari H, Möcklinghoff C, Stinanu Hu, Lehnhardt M. Handbeteiligungen im Rahmen der progressiven systemischen Sklerodermie-Möglichkeiten der Handchirurgie im interdisziplinären Konzept. Handchir Mikrochir Plast Chir. 2007;39(2):128-34. http://doi.org/bzjxsq.

Melone CP Jr, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Curr Opin Rheumatol. 1999;11(6):514-20.

Coveliers HM, Hoexum F, Nederhoeed JH, Wisselink W, Rauwerda JA. Thoracic sympathectomy for digital ischemia: a summary of evidence. J Vasc Surg. 2011;54(1):273-7. http://doi.org/dtnfx9.

Cutolo M, Smith V, Furst DE, Khanna D, Herrick AL. Points to consider-Raynaud’s phenomenon in systemic sclerosis. Rheumatology (Oxford). 2017;56(suppl 5):v45-8. http://doi.org/gb2zh3.

Fonollosa-Pla V, Simeón-Aznar CP, Vilardell-Tarrés M. Tratamiento del fenómeno de Raynaud. Rev Clin Esp. 2009;209(1):21-4. http://doi.org/fn4vpd.

Jones NF, Imbriglia JE, Steen VD, Medsgar TA. Surgery for scleroderma of the hand. J Hand Surg Am. 1987;12(3):391-400. http://doi.org/cgbt .

Ruch DS, Holden M, Smith BP, Smith TL, Koman LA. Periarterial sympathectomy in scleroderma patients: intermediate-term follow-up. J Hand Surg Am. 2002;27(2):258-64. http://doi.org/c78tbp.

Mendelson BC, Linscheid RL, Dobyns JH, Mullar SA. Surgical treatment of calcinosis cutis in the upper extremity. J Hand Surg Am. 1977;2(4):318-24. http://doi.org/cgbv.

Chamberlain AJ, Walker NP. Successful palliation and significant remission of cutaneous calcinosis in CRSScT syndrome with carbon dioxide laser. Dermatol Surg. 2003;29(9):968-70.

Bottomley WW, Goodfield MJ, Sheehan-Dare RA. Digital calcification in systemic sclerosis: effective treatment with good tissue preservation using the carbon dioxide laser. Br J Dermatol. 1996;135(2):302-4. http://doi.org/bmnnhh.

Balin SJ, Wetter DA, Andersen LK, Davis MD. Calcinosis cutis occurring in association with autoimmune connective tissue disease. The Mayo Clinic Experience with 78 patients, 1996-2009. Arch Dermatol. 2012;184(4):455-62. http://doi.org/fx25v4.

Nalebuff EA. Surgery in patients with systemic sclerosis of the hand. Clin Orthop Relat Res. 1999;(366):91-7.

Bennett RM. Mixed connective tissue disease and other overlap syndromes. In: Kelley WN, Harris ED, Ruddy S, Sledge G, editors. Textbook of Rheumatology. Philadelphia: WB Saunders; 1985. p. 1115.

Feldon P, Terrono A, Millender L, Nalebuff E. Rheumatoid Arthritis and Other Connective Tissue Diseases. In: Wolfe SW,‎ Pederson WC,‎ Hotchkiss RN,‎ Kozin SH, Cohen MS. Green’s Operative Hand Surgery. 7th ed. Philadelphia: Elsevier; 2017. p. 1832-1903.

Meyer MF, Daigeler A, Lehnhardt M, Steinau HU, Kelin HH. Therapeutisches Management akraler Manifestationen der systemischen Sklerose. Med Klin (Munich). 2007:102(3):209-18. http://doi.org/fg6r3n.

Crincoli V, Fatone L, Fanelli M, Rotolo RP, Chialà A, Favia G, et al. Orofacial Manifestations and Temporomandibular Disorders of Systemic Scleroderma: An Observational Study. Int J Mol Sci. 2016;17(7). http://doi.org/cgbx .

Méndez-Baca SI, Enríquez-Merino J, Alcalá-Pérez D. Esclerodermia localizada: corrección mediante trasplante autólogo de grasa. Comunicación de seis casos. Dermatol Rev Mex. 2013;57(1):60-3.

Gutiérrez GC, Hayakawa V, Franco A, Reyes L. Lipoinyección para reconstrucción del contorno facial en S. Parry Romberg, esclerodermia y secuelas de trauma: una alternativa práctica utilizando cánula para bloqueo peridural. Cir Plast.2007;17(3):168-75.

Pastor-Oliver JF, Calcedo-Ascoz A, Paricio-Talayero JM, Santos-Serrano L, Fernández-Feijjóo A, Ferriol-Camacho M. Esclerodermia lineal: Lesión en golpe de sable. Revisión de la literatura y aportación de un caso. An Esp Pediatr.1998;49:499-502.

Karaaltin MV, Akpinar AC, Baghaki S, Akpinar F. Treatment of “en coup de sabre” deformity with adipose-derived regenerative cell-enriched fat graft. J Craniofac Surg. 2012;23(2):e103-5. http://doi.org/f3wx65.

Roh MR, Jung JY, Chung KY. Autologous Fat Transplantation for Depressed Linear Scleroderma-Induced Facial Atrophic Scars. Dermatol Surg. 2008;34(12):1659-65. http://doi.org/cwtshk.

Thareja SK, Sadhwani D, Alan-Fenske N. En coup de sabre morphea treated with hyaluronic acid filler. Report of a case and review of the literature. Int J Dermatol. 2015;54(7):823-6. http://doi.org/cgb2.

MacIntosh RB, Shivapuja PK, Naqvi R. Scleroderma and the temporomandibular joint: reconstruction in 2 variants. J Oral Maxillofac Surg. 2015;73(6):1199-210. http://doi.org/f7b6r2.

Dinsdale G, Murray A, Moore T, Ferquson J, Wilkinson J, Richards H, et al. A comparison of intense pulsed light and laser treatment of telangiectases in patients with systemic sclerosis: a within-subject randomized trial. Rheumatology (Oxford). 2014;53(8):1422-30. http://doi.org/f6c5cm.

Chung L, Fiorentino D. Digital ulcers in patients with systemic sclerosis. Autoimmun Rev. 2006;5(2):125-8.

Hummers LK, Wigley FM. Management of Raynaud’s phenomenon and digital ischemic lesions in scleroderma. Rheum Dis Clin North Am. 2003;29(2):293-313. http://doi.org/fh2gxh.

Merkel PA, Herlyn K, Martin RW, Anderson JJ, Mayes MD, Bell P, et al. Measuring disease activity and functional status in patients with scleroderma and Raynaud’s phenomenon. Arthritis Rheum. 2002;46(9):2410-20. http://doi.org/fhd9zt.

Denton CP, Korn JH. Digital ulceration and critical digital ischemia in scleroderma. Scleroderma Care Res. 2003;1:12-6.

Sticherling M. Systemic sclerosis-dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges. 2012;10(10):705-18. http://doi.org/f4fbf6.

Hafner J, Schneider, Burg G, Cassina PC. Management of leg ulcers in patients with rheumatoid arthritis or systemic sclerosis: The importance of concomitant arterial and venous disease. J Vasc Surg. 2000;32(2):322-9. http://doi.org/dsg9n7.

McRorie E. Regarding “management of leg ulcers in patients with rheumatoid arthritis or systemic sclerosis: the importance of concomitant arterial and venous disease”. J Vasc Surg. 2001;33(3):664-5. http://doi.org/dzrr5w.

Giuggioli D, Sebastiani M, Cazzato M, Piaggesi A, Abatangelo G, Ferri C. Autologous skin grafting in the treatment of severe scleroderma cutaneous ulcers: a case report. Rheumatology (Oxford). 2003;42(5):694-6.

Arvedson T, O’Kelly J, Yang BB. Design Rationale and Development Approach for Pegfilgrastim as a Long-Acting Granulocyte Colony-Stimulating Factor. BioDrugs. 2015;29(3):185-98. http://doi.org/f7hj4v.

Kästenbauer T, Hörnlein B, Skol G, Irsigler K. Evaluation of granulocyte-colony stimulating factor (Filgrastim) in infected diabetic foot ulcers. Diabetologia. 2003;46(1):27-30. http://doi.org/btnrwz.

Yönem A, Cakir B, Güler S, Azal OO, Corakçi A. Effects of granulocyte-colony stimulating factor in the treatment of diabetic foot infection. Diabetes Obes Metab. 2001;3(5):332-7. http://doi.org/dk2sd5.

Martinez-Zapata MJ, Artí-Carvajal AJ, Solà I, Expósito JA, Bolíbar I, Rodríguez L, et al. Autologous platelet-rich plasma for treating chronic wounds. Cochrane Database Syst Rev. 2016;10:CD006899. http://doi.org/gbb4jn.

Salazar-Álvarez AE, Riera-del Moral LF, García-Arranz M, Álvarez-García J, Concepción-Rodriguez NA, Riera-de Cuba L. Uso de plasma rico en plaquetas para cicatrización de úlceras crónicas de miembros inferiores. Actas Dermosifiliogr. 2014;105(6):597-604. http://doi.org/f2rsrz.

Kirana S, Stratmann B, Prante C, Prohaska W, Koerperich H, Lammers D, et al. Autologous stem cell therapy in the treatment of limb ischaemia induced chronic tissue ulcers of diabetic foot patients. Int J Clin Pract. 2012;66(4):384-93. http://doi.org/fxtqrb.

Gaviria CM, Jiménez SB, Gutiérrez J. Morfea o esclerodermia localizada. Rev Asoc Colomb Dermatol. 2014;22(2):126-40.

Garza-Rodríguez V, Villarreal-Alarcón MA, Ocampo-Candiani J. Etiopatogenia y tratamiento de esclerodermia. Conceptos actuales. Rev Med Inst Mex Seguro Soc. 2013;51(5):50-7.

Denton CP. Overview and classification of scleroderma disorders. Waltham: UpToDate. [Cited 2016 Jun 12]. Available from: https://goo.gl/JBaC3R.

Bielsa-Marsol I. Actualización en la clasificación y el tratamiento de la esclerodermia localizada. Actas Dermosifiliogr. 2013;104(8):654-66. http://doi.org/f2fnhd.

Avouac J, Fransen J, Walker UA, Riccieri V, Smith V, Muller C, et al. Preliminary criteria for the very early diagnosis of systemic sclerosis; results of a Delphi Consensus Study from EULAR scleroderma trial and research group. Ann Rheum Dis. 2011;70(3):476-81. http://doi.org/fr8jr5.

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