Abstract
Morphea is a fibrosing disorder of the skin without systemic involvement. It can be clinically classified in different forms: circumscribed, linear, generalized, pansclerotic and mixed forms. The pathogenesis is not completely understood. An underlying predisposition and environmental factors are required to develop the disease. No causal treatment for morphea exists. Topical systemic and phototherapy are used. Topical treatments are adequate for limited forms, while phototherapy and systemic treatment are used for the more severe forms of the disease. Corticosteroids are the mainstay of topical treatments. Topical calcipotriol and tacrolimus have been evaluated in open studies and they seem to improve significantly morphea. UVA1 which penetrate deeper in the dermis than narrow-band UVB are usually considered the best option in phototherapy. Methotrexate is the systemic drug which has the best evidences; it can be used in addition to corticosteroids, in particular in severe morphea of young people.
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Andreas C, Kollmar A, Mempel M, et al. Successful ultraviolet A1 phototherapy in the treatment of localized scleroderma: a retrospective and prospective study. Br J Dermatol. 2010;20:93–100.
Beltramelli M, Vercellesi P, Frasin A, et al. Localized severe scleroderma a retrospective study of 26 pediatric patients. Pediatr Dermatol. 2010;27:476–80.
Bibi Y, Gottlieb AB. A potential role for imatinib and other small molecule tyrosine kinase inhibitors in the treatment of systemic and localized sclerosis. J Am Acad Dermatol. 2008;59:654–8.
Browning JC. Pediatric morphea. Dermatol Clin. 2013;31:229–37.
Colomè-Grimmer MI, Payne DA, Tyring SK, et al. Borrelia burgdorferi DNA and Borrelia hermsii DNA are not associated with morphea or lichen sclerosus et atrophicus in the southwestern United States. Arch Dermatol. 1997;133:1174.
Cunningham BB, Landells ID, Langman C, et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. 1998;92:211–21.
Eisendle K, Grabner T, Zelger B. Morphea: a manifestation of infection with Borrelia species? Br J Dermatol. 2007;157:1189–98.
Fett N, Werth WP. Update on morphea. Epidemiology, clinical presentation and pathogenesis. J Am Acad Dermatol. 2011a;64:217–28.
Fett N, Werth VP. Update on morphea. Part II. Outcome measures and treatment. J Am Acad Dermatol. 2011b;64:231–42.
Haykawa I, Hasegawa M, Takehara K, Sato S. Anti-DNA topoisomerase II alpha autoantibodies in localized scleroderma. Arthritis Rheum. 2004;50:227–32.
Kerscher M, Volkenandt M, Gruss C, et al. Low-dose phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. 1998;38:21–6.
Kreuter A, Hyun J, Stucker M, et al. A randomized controlled study of low-dose A1, medium dose UVA1 and narrow band UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440–7.
Kroft EB. Efficacy of topical tacrolimus 0.1% in active plaque morphea: randomized double-blind, emollient-controlled pilot study. Am J Clin Dermatol. 2009;10:181–7.
Laxer RM, Zulin F. Localized scleroderma. Curr Opin Rheumatol. 2006;18:606–13.
Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol. 2009;145:545–50.
Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 patients. Eur J Dermatol. 2003;13:171–6.
Peroni A, Zini A, Braga V, et al. Drug-induced morphea: report of a case induced by Bilicatib and review of the literature. J Am Acad Dermatol. 2008;59:125–9.
Peterson L, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70:1068–76.
Peterson L, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted county 1960–1993. J Rheumatol. 1997;24:73–80.
Rak JM, Pagni PP, Tiev K, et al. Male microchimerism and HLA compatibility in French women with scleroderma: a different profile in limited and diffuse subset. Rheumatology. 2009;48:363–6.
Sc L, Torok KS, Pope E, et al. Development of consensus treatment plans for Juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res. 2012;64:1175–85.
Tomimura S, Ogawa F, Iwata Y, et al. Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. J Dermatol Sci. 2008;52:47–54.
Uziel Y, Feldman BM, Krafchik BR, et al. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr. 2000;136:91–5.
Yamamoto T. Chemokines and chemokine receptors in scleroderma. Int Arch Allergy Immunol. 2006;140:345–56.
Yamane K, Ihn H, Kubo M, 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;42:64–9.
Zulian F, Martini G, Vallongo C, et al. Methotrexate in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2011;63:1998–2006.
Zulian F, Vallongo C, Patrizi A, et al. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol. 2012;67:1151–6.
Zwischenberger BA, Jacobe HT. A systematic review of morphea treatments and therapeutic algorithm. J Am Acad Dermatol. 2011;65:925–41.
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Parodi, A. (2015). Morphea (Localized Scleroderma). In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D’Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45139-7_64
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DOI: https://doi.org/10.1007/978-3-662-45139-7_64
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