2019, Number 5
<< Back Next >>
Gac Med Mex 2019; 155 (5)
Morfea: revisión práctica de su diagnóstico, clasificación y tratamiento
Rodríguez-Salgado P, García-Romero MT
Language: Spanish
References: 86
Page: 522-531
PDF size: 430.95 Kb.
ABSTRACT
Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of
the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that
there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due
to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started
early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the
classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis
on existing clinical evidence regarding their efficacy and safety.
REFERENCES
Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90:62-73.
García de la Peña-Lefebvre P. Esclerodermia localizada. Protoc Diagn Ter Pediatr. 2014;1:1-6.
García-Romero M, Laxer RM, Pope E. Localized scleroderma: symptoms, diagnosis and treatment. En: De Winter R, editor. Scleroderma symptoms, diagnosis and treatment. EE. UU.: Nova Science Publishers; 2013.
Peterson L, Nelson A, Su W, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997; 24:73-80.
Zulian F, Athreya BH, Laxer RM, Nelson AM, Feitosa-De Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614-620.
Christen-Zaech S, Hakin MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008;59:385-396.
Alfaro-Cordero K. Morfea: el desequilibrio entre la producción y la destrucción del colágeno. Rev Med Cos. 2016;73:351-354.
Paller AS. Piecing together the puzzle of cutaneous mosaicism. J Clin Invest. 2004;114:1407-1409.
Yamane K, Ihn H, Kubo M, Yazawa N, Kikuchi K, Soma Y, et al. Increased serum levels of soluble vascular cell adhesion molecule 1 and E-selectin in patients with localized scleroderma. J Am Acad Dermatol. 2000;42:64-69.
Serpier H, Gillery P, Salmon-Ehr V, Garnotel R, Georges N, Kalis B, et al. Antagonistic effects of interferon-gamma and interleukin-4 on fibroblast cultures. J Invest Dermatol. 1997;109:158-162.
Bielsa Marsol I. Update on the classification and treatment of localized scleroderma. Act Dermosifiliogr. 2013;104:654-666.
Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA, Lehman TJ, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League Against Rheumatism Provisional Classification Criteria for Juvenile Systemic Sclerosis. Arthritis Rheum. 2007; 57:203-212.
Noguera M, Sauro-De Carvalho M, Lombardi V. Morfea. Arch Argent Dermatol. 2012;62:77-84.
Zulian F, Cuffaro G, Sperotto F. Scleroderma in children: an update. Curr Opin Rheumatol. 2013;25 643-650.
Pequet M, Holland KE, Zhao S, Drolet BA, Galbraith SS, Siegel DH, et al. Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients. Br J Dermatol. 2014;170:895-900.
Chiu YE, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol. 2012;29:738-748.
Kim A, Marinkovich N, Vásquez R, Jacobe HT. Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort. J Rheumatol. 2014;41:106-112.
Kreuter A, Wischnewski J, Terras S, Altmeyer P, Stücker M, Gambichler T. Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center. J Am Acad Dermatol. 2012;67:1157-1162.
Pope E, Laxer RM. Diagnosis and management of morphea and lichen sclerosus and atrophicus in children. Pediatr Clin North Am. 2014;61:309-319.
Fett NM. Morphea (localized scleroderma). JAMA Dermatol. 2013; 149:1124.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, 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;31:1401-1424.
Kreuter A, Krieg T, Worm M, Wenzel J, Moinzadeh P, Kuhn A, et al. German guidelines for the diagnosis and therapy of localized scleroderma. J German Soc Dermatol. 2016;14:199-216.
Chiu YE. Practice gaps: evaluating the clinical utility of autoantibodies in morphea. JAMA Dermatol. 2013;149:1166.
Walker D, Susa JS, Currimbhoy S, Jacobe H. Histopathological changes in morphea and their clinical correlates: results from the morphea in adults and children cohort V. J Am Acad Dermatol. 201/;76:1124-1130.
Yamazaki-Nakashimada MA, Saez-De Ocariz M, Maldonado-Colín G, García-Romero MT. Subcutaneous immunoglobulin for the treatment of deep morphoea in a child. Clin Exp Dermatol. 2017;43:303-305.
Zwischenberger B, Jacobe H. A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol. 2011;65:925-941.
García-Romero MT, Laxer RM, Pope E. Correlation of clinical tools to determine activity of localized scleroderma in paediatric patients. Br J Dermatol. 2015;174:408-410.
Arkachaisri T, Vilaiyuk S, Li S, O’Neil KM, Pope E, Higgins GC, et al. 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. 2009;36:2819-2829.
Arkachaisri T, Vilaiyuk S, Torok KS, Medsger TA. 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). 2010;49:373-381.
García-Romero MT, Randhawa HK, Laxer RM, Pope E. The role of local temperature and other clinical characteristics of localized scleroderma as markers of disease activity. Int J Dermatol. 2016;56:63-67.
Khan MA, Shaw L, Eleftheriou D, Prabhakar P, Chong WK, Glover M, et al. Radiologic improvement after early medical intervention in localised facial morphea. Pediatr Dermatol. 2016;33 e95-e98.
Brady SM, Shapiro L, Mousa SA. Current and future direction in the management of scleroderma. Arch Dermatol Res. 2016;308:461-471.
Hawley DP, Pain CE, Baildam EM, Murphy R, Taylor AE, Foster HE. United Kingdom survey of current management of juvenile localized scleroderma. Rheumatology (Oxford). 2014;53:1849-1854.
Warburton KL, McPhee MJ, Savage LJ, Honan AE, Montgomery R, Ghazavi M, et al. Management of morphoea: results of a national survey of U.K. clinicians. Br J Dermatol. 2014;171:1243-1245.
Fett NM, Werth VP. Update on morphea: part II. Outcome measures and treatment. J Am Acad Dermatol. 2011;64:231-242.
Strickland N, Patel G, Strickland A, Jacobe H. Attitudes and trends in the treatment of morphea: a national survey. J Am Acad Dermatol. 2015; 72:727-728.
Kreuter A, Altmeyer P, Gambichler T. Treatment of localized scleroderma depends on the clinical subtype. Br J Dermatol. 2007;156:1363-1364.
Piram M, McCuaig CC, Saint-Cyr C, Marcoux D, Hatami A, Haddad E, et al. Short- and long-term outcome of linear morphoea in children. Br J Dermatol. 2013;169:1265-1271.
Kroft EB, Groeneveld TJ, Seyger MM, De Jong EM. Efficacy of topical tacrolimus 0.1 % in active plaque morphea: randomized, double-blind controlled pilot study. Am J Clin Dermatol. 2009;10:181-187.
Dytoc M, Wat H, Cheung-Lee M, Sawyer D, Ackerman T, Fiorillo L. Evaluation of the efficacy and safety of topical imiquimod 5 % for plaque- type morphea: a multicenter, prospective, vehicle-controlled trial. J Cut Med Surg. 2015;19:132-139.
Dytoc MT, Kossintseva I, Ting PT. First case series on the use of calcipotriol- betaethasone dipropionate for morphea. Br J Dermatol. 2007; 157:615-618.
Tay YK. Topical calcipotriol ointment in the treatment of morphea. J Dermatolog Treat. 2003;14:219-221.
Rodríguez-Castellanos M, Tlacuilo-Parra A, Sánchez-Enriquez S, Vélez- Gómez E, Guevara-Gutiérrez E, et al. Pirfenidone gel in patients with localized scleroderma: a phase II study. Arthr Res Ther. 2015;16:510.
Hunzelmann N, Anders S, Fierlbeck G, Hein R, Herrmann K, Albrecht M. Double-blind, placebo-controlled study of intralesional interferongamma fot the treatment of localized scleroderma. J Am Acad Dermatol. 1997;36:433-435.
Li S, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, et al. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arth Care Res (Hoboken). 2012;64:1175-1185.
Tratenberg M, Gutwein F, Rao V, Sperber K, Wasserrman A, Ash J. Localized scleroderma: a clinical review. Curr Rheumatol Rev. 2017; 13:86-92.
Zulian F, Martini G, Vallongo C, Vittadello F, Falcini F, Patrizi A, et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2011; 63:1998-2006.
Zulian F, Vallongo C, Patrizi A, Belloni-Fortina A, Cutrone M, Alessio M, et al. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol. 2012;67:1151-1156.
Rattanakaemakorn P, Jorizzo J. The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype). J Dermatol Treat. 2017;29:197-199.
Uziel Y, Feldman B, Krafchik B, Yeung RS, Laxer RM. Methotrexate and corticosteroids therapy for pediatric localized scleroderma. J Pediatr. 2000;136:91-95.
Weibel L, Sampaio MC, Visentin MT, Howell KJ, Woo P, Harper JI. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol. 2006;155:1013-1020.
Breuckmann F, Gambichler T, Altmeyer P, Kreuter A. UVA/UVA1 phototherapy and PUVA photochemotherapy in connective tissue diseases and related disorders: a research based review. BMC Dermatol. 2004;4:11.
De Rie MA, Enomoto DN, De Vries H, Bos JD. Evaluation of medium-dose UVA-1 phototherapy in localized scleroderma with the cutometer and fast Fourier transform method. Dermatology. 2003;207:298-301.
El-Mofty M, Mostafa W, El-Darouty M, Bosseila M, Nada H, Yousef R, et al. Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis. Photodermatol Photoimmunol Photomed. 2004; 20:148-156.
El-Mofty M, Zaher H, Bosseila M, Yousef R, Saad B. Low-dose broadband UVA in morphea using a new method for evaluation. Photodermatol Photoimmunol Photomed. 2000;16:43-49.
Keyal U, Bhatta AK, Wang XL. UVA1 a promising approach for scleroderma. Am J Transl Res. 2017;9:4280-4287.
Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA-1, medium-dose UVA-1 and narrowband UVB phototherapy in the treatment of localized slceroderma. J Am Acad Dermatol. 2006;54:440-447.
Pavlotsky F, Sakka N, Lozinski A, Barzilai A. Bath psoralen-UVA photochemotherapy for localized scleroderma: experience from a single institute. Photodermatol Photoimmunol Photomed. 2013;29:247-252.
Vásquez R, Jabbar A, Khan F, Buethe D, Ahn C, Jacobe H. Recurrence of morphea after successful ultraviolet A1 phototherapy: a cohort study. J Am Acad Dermatol. 2014;70:481-488.
Elst EF, Van Suijlekom-Smit L, Oranje AP. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children. Pediatric Dermatol. 1999;16:53-58.
Hulshof MM, Bouwes-Bavinck JN, Bergman W, Masclee AA, Heickendorff L, Breedveld FC, et al. Double-blind, placebo controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. 2000;43:1017-1023.
Barin EZ, Cinal H, Cakmak MA, Tan O. Treatment of linear scleroderma (en coup de sabre) with dermal fat grafting. J Cutan Med Surg. 2016; 20:269-271.
Pimenta de Araujo-Franco J, Soares-Serra M, Barbosa-Lima R, Macedo- D’Acri A, Martins CJ. Scleroderma en coup de sabre treated with polymethylmethacrylate. Case report. An Bras Dermatol. 2016;91:209-211.
Ibler KS, Gramkow C, Siemssen PA. Autologous fat transplantation for the treatment of linear scleroderma en coup de sabre. Skinmed. 2015;13:74-76.
Rimoin L, Arbiser J. Improvement of en coup de sabre morphea and associated headaches with botulinum toxin injections. Dermatol Surg. 2016;42:1216-1219.
Íñigo F, Jiménez-Murat Y, Arroyo O, Fernández M, Ysunza A. Restoration of facial contour in Romberg’s disease and hemifacial microssomia: experience with 118 cases. Microsurgery. 2000;20:167-172.
Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E. En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes. J Rheumatol. 2010;37:2174-2179.
Schoch JJ, Schoch BS, Werthel JD, McIntosh AL, Davis DMR. Orthopedic complications of linear morphea: implications for early interdisciplinary care. Pediatr Dermatol. 2018;35:43-46.
Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006; 18:606-613.
Grundmann-Kollmann M, Ochsendorf F, Zollner TM, Spieth K, Sachsenberg- Studer E, Kaufmann R, et al. PUVA-cream photochemotherapy for the treatment of localized scleroderma. J Am Acad Dermatol. 2000; 43:675-678.
Shalaby SM, Bosseila M, Fawzy MM, Abdel Halim DM, Sayed SS, Allam RS, et al. Fractional carbon dioxide laser versus low-dose UVA-1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study. Laser Med Sci. 2016;31:1707-1715.
Usmani N, Murphy A, Veale D, Goulden V, Goodfield M, et al. Photochemotherapy for localized morphea: effect on clinical and molecular markers. Clin Exp Dermatol. 2008;33:698-704.
Adeeb F, Anjum S, Hodnett P, et al. Early- and late-stage morphea subtypes with deep tissue involvement is treatable with abatacept (Orencia). Semin Arthr Rheumatism. 2017;46:775-781.
Bali G, Fruhauf J, Wutte N, et al. Cyclosporine reduces sclerosis in morphea: a retrospective study in 12 patients and a literature review. Dermatology (Basel, Switzerland). 2016;232:503-510.
Martini G, Ramanan AV, Falcini F, Girschick H, Goldsmith DP, Zulian F. Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatol (Oxford). 2009; 48:1410-1413.
Mertens JS, Marsman D, Van de Kerkhof PC, Hoppenreijs EP, Knaapen HK, Radstake TR, et al. Use of mycophenolate mofetil in patients with severe localized scleroderma resistant or intolerant to methotrexate. Act Derm Venereol. 2016;96:510-513.
Martini G, Campus S, Raffeiner B, Boscarol G, Meneghel A, Zulian F. Tocilizumab in two children with pansclerotic morphoea: a hopeful therapy for refractory cases? Clin Exp Rheumatol. 2017;106:211-213.
Kaur S, Dhar S, Kanwar AJ. Treatment of childhood linear morphea with D-penicillamine. Pediatr Dermatol. 1993;10:201-202.
Alcántara-Reifs CM, Garnacho-Saucedo GM, Salido-Vallejo R, De la Corte-Sánchez S, García-Nieto AV. Imatinib treatment of therapy resistant generalized deep morphea. Dermatol Ther. 2015;28:271-273.
Coelho-Macías V, Mendes-Bastos P, Assis-Pacheco F, Cardoso J. Imatinib: a novel treatment approach for generalized morphea. Int J Dermatol. 2014;53:1299-1302.
Hanson AH, Fivenson DP, Schapiro B. Linear scleroderma in an adolescent woman treated with methotrexate and excimer laser. Dermatol Ther. 2014;27:203-205.
Ferguson ID, Weiser P, Torok KS. A case report of successful treatment of recalcitrant childhood localized scleroderma with infliximab and leflunomide. Open Rheumatol J. 2015;9:30-35.
Shima T, Yamamoto Y, Ikeda T, Furukawa F. A patient with localized scleroderma successfully treated with etretinate. Case Rep Dermatol. 2014;6:200-206.
Pileri A, Raone B, Raboni R, Giudice V, Patrizi A. Generalized morphea successfully treated with extracorporeal photochemotherapy (ECP). Dermatol Online J. 2014;20:21258.
Howick J, Chalmers I, Glasziou. Explanation of the 2011 Oxford Centre for Evidence-Based Medicine (OCEBM) levels of evidence. EE. UU.: Oxford Centre for Evidence-Based Medicine; 2011.
OCEBM Levels of Evidence Workin Group. The Oxford Levels of Evidence 2. EE. UU.: Oxford Centre for Evidence-Based Medicine; 2009.