Morphea Treatment & Management
- Author: Jennifer V Nguyen, MD; Chief Editor: Dirk M Elston, MD more...
Although several regimens have shown benefit in case series, few controlled trials have been performed and data suggest wide variation in the approach to treatment. In general, therapy aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions.
Superficial circumscribed morphea
Lesions of superficial circumscribed morphea often undergo gradual spontaneous resolution over a 3- to 5-year period. Limited disease can often be managed with topical therapy or lesion-limited phototherapy.
Treatment of active lesions with superpotent topical or intralesional corticosteroids may help reduce inflammation and prevent progression, although there is a lack of data supporting their efficacy.
Tacrolimus 0.1% ointment applied twice daily for 12 weeks may be a useful first-line agent for active, limited plaque morphea, as supported by a small double-blind, placebo-controlled study.
Topical calcipotriene may also be beneficial, especially when nightly occlusion (eg, with plastic wrap) is used to increase penetration of the medication. The combination of topical calcipotriol with betamethasone dipropionate has also been reported effective.
Imiquimod 5% cream 3-5 times per week has been shown to decrease lesional erythema and induration in small series.[53, 54, 55]
Generalized, linear, or deep morphea
Patients with potentially disabling generalized, linear, or deep morphea typically require more aggressive therapy.
Successful treatment of severe and/or rapidly progressive morphea with systemic corticosteroids (eg, high-dose intravenous methylprednisolone in monthly pulses or oral prednisone at various intervals) in combination with weekly low-dose methotrexate (MTX) has been reported in several case series. A randomized, double-blind, placebo-controlled trial demonstrated the efficacy of combination therapy with oral prednisone and methotrexate in children with active morphea. MTX alone can also be effective and has been used successfully as long-term therapy in both adults and children.[58, 59, 60, 61, 62]
Systemic corticosteroids can be helpful in the inflammatory phases of morphea, but they have little benefit for established sclerosis and are not recommended for long-term monotherapy given their risk of adverse effects and tendency for relapse with discontinuation.
Mycophenolate mofetil is a second-line agent that has been shown to be effective in patients with MTX-resistant disease. It is believed to function through antifibrotic mechanisms.[45, 63]
A few reports describe responses of severe morphea to cyclosporine and everolimus.[64, 65]
The use of hydroxychloroquine to treat morphea has been advocated, but little documentation of success is present in the medical literature.
Despite promising results in case series involving both adults and children, oral calcitriol did not lead to significant improvement in a double-blinded placebo-controlled trial.[66, 67]
Phototherapy may be beneficial as a second-line therapy for refractory or severe disease, or as a first-line therapy for patients with generalized morphea given its low side effect profile compared to immunosuppressive agents. Phototherapy may be beneficial as a second-line therapy for refractory or severe disease or as a first-line therapy for patients with generalized morphea, given its low adverse effect profile compared with immunosuppressive agents. Broadband UVA (320-400 nm, low-dose), long-wavelength UVA (UVA1; 340-400 nm, low- or medium-dose), and psoralen plus UVA (oral or bath) photochemotherapy has produced marked clinical improvement of morphea lesions in multiple case series and a randomized controlled trial. Because UVA1 wavelengths penetrate deeper into the dermis, this modality is particularly effective in the treatment of morphea. Low-, medium-, and high-dose UVA are all effective. While some data suggest that medium-dose UVA1 provides for better long-term results than low-dose UVA1 in morphea as shown by ultrasound assessment, other data suggest similar outcomes.[43, 70] Unfortunately, the availability of UVA1 is currently limited. Narrowband UVB therapy, although less potent owing to its limited dermal penetration, can also be beneficial. Regimens combining UV therapy with topical corticosteroids or calcipotriene may be superior to either method alone.[72, 71]
A combination of acitretin and PUVA has also shown efficacy.
Few cases have shown benefit using extracorporeal photopheresis, particularly for generalized deep morphea.[74, 75]
In one case report, treatment of plaque-type morphea with the 585-nm pulsed dye laser led to substantial improvement.
Photodynamic therapy using topical 5-aminolevulinic acid was also effective in a small series, but a prospective, lesion-controlled study showed no significant difference with photodynamic therapy between treated and untreated lesions.
Bosentan has shown benefit for refractory cutaneous ulcerations in pansclerotic morphea. It is an endothelin receptor antagonist with vasodilatative and antifibrotic properties.
Other approaches aim to alter the cytokine milieu but await further study. These include topical halofuginone (transforming growth factor-beta synthesis inhibitor), TNF-alpha inhibitors, imatinib, and thalidomide (interleukin 12 and tumor necrosis factor-alpha inducer).[79, 80] A randomized, double-blinded, placebo-controlled trial demonstrated no benefit with intralesional interferon gamma.
Orthopedic surgery may be indicated if patients develop deformities of the joints and bones as sequelae of linear or deep morphea. Such surgical interventions include release of joint contractures and limb-lengthening procedures.
Plastic surgery can help to correct deformities due to atrophy of subcutaneous tissues. Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials (eg, polyethylene).
Referral to a dermatologist can help establish the diagnosis and initiate appropriate treatment of morphea.
Consultation with a physical and occupational therapist and a program of physical therapy are of utmost importance in maintaining range of motion and function of the extremities in patients with linear or deep morphea that crosses joint lines. Programs typically include passive stretching, muscle strengthening, and resting splints.
Ophthalmologic screening is indicated for children with head and neck lesions and/or concomitant central nervous system involvement.
Consultation with a neurologist is helpful for patients with craniofacial morphea who present with neurologic symptoms or have abnormalities detected via MRI of the brain.
Orthopedics and oral and maxillofacial surgery should be consulted as needed for bony and structural abnormalities.
Consultation with a dentist is required when craniofacial morphea leads to altered dentition or malocclusion.
Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006 Nov. 18(6):606-13. [Medline].
Zulian F. Systemic manifestations in localized scleroderma. Curr Rheumatol Rep. 2004 Dec. 6(6):417-24. [Medline].
Chung L, Lin J, Furst DE, Fiorentino D. Systemic and localized scleroderma. Clin Dermatol. 2006 Sep-Oct. 24(5):374-92. [Medline].
Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe HT. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol. 2009 May. 145(5):545-50. [Medline].
Yamamoto T. Chemokines and chemokine receptors in scleroderma. Int Arch Allergy Immunol. 2006. 140(4):345-56. [Medline].
Igarashi A, Nashiro K, Kikuchi K, et al. Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid, and other fibrotic skin disorders. J Invest Dermatol. 1996 Apr. 106(4):729-33. [Medline].
Kikuchi K, Kadono T, Ihn H, et al. Growth regulation in scleroderma fibroblasts: increased response to transforming growth factor-beta 1. J Invest Dermatol. 1995 Jul. 105(1):128-32. [Medline].
Leask A, Denton CP, Abraham DJ. Insights into the molecular mechanism of chronic fibrosis: the role of connective tissue growth factor in scleroderma. J Invest Dermatol. 2004 Jan. 122(1):1-6. [Medline].
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 Jan. 42(1 Pt 1):64-9. [Medline].
Fawzi MM, Tawfik SO, Eissa AM, El-Komy MH, Abdel-Halim MR, Shaker OG. Expression of insulin-like growth factor-I in lesional and non-lesional skin of patients with morphoea. Br J Dermatol. 2008 Jul. 159(1):86-90. [Medline].
Tomimura S, Ogawa F, Iwata Y, Komura K, Hara T, Muroi E. Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. J Dermatol Sci. 2008 Oct. 52(1):47-54. [Medline].
Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011 Feb. 64(2):217-28; quiz 229-30. [Medline].
Alimova E, Farhi D, Plantier F, Carlotti A, Gorin I, Mouthon L. Morphoea (localized scleroderma): baseline body surface involvement and antinuclear antibody may have a prognostic value. Clin Exp Dermatol. 2009 Oct. 34(7):e491-2. [Medline].
Lee YJ, Chung KY, Kang HC, Kim HD, Lee JS. Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia. Korean J Pediatr. 2015 Sep. 58 (9):354-7. [Medline].
Kroft EB, de Jong EM, Evers AW. Psychological distress in patients with morphea and eosinophilic fasciitis. Arch Dermatol. 2009 Sep. 145(9):1017-22. [Medline].
Zulian F. New developments in localized scleroderma. Curr Opin Rheumatol. 2008 Sep. 20(5):601-7. [Medline].
Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008 Sep. 59(3):385-96. [Medline].
Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006 May. 45(5):614-20. [Medline].
Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko's lines. J Am Acad Dermatol. 1998 Feb. 38(2 Pt 2):366-8. [Medline].
Weibel L, Harper JI. Linear morphoea follows Blaschko's lines. Br J Dermatol. 2008 Jul. 159(1):175-81. [Medline].
Mazori DR, Wright NA, Patel M, Liu SW, Ramachandran SM, Franks AG Jr, et al. Characteristics and treatment of adult-onset linear morphea: A retrospective cohort study of 61 patients at 3 tertiary care centers. J Am Acad Dermatol. 2016 Mar. 74 (3):577-9. [Medline].
Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007 Feb. 56(2):257-63. [Medline].
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 Apr 23. [Medline].
Lutz V, Francès C, Bessis D, Cosnes A, Kluger N, Godet J. High frequency of genital lichen sclerosus in a prospective series of 76 patients with morphea: toward a better understanding of the spectrum of morphea. Arch Dermatol. 2012 Jan. 148(1):24-8. [Medline].
Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. 2006 Jan. 117(1):e132-6. [Medline].
Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007 Oct. 91(10):1311-4. [Medline]. [Full Text].
Prinz JC, Kutasi Z, Weisenseel P, Poto L, Battyani Z, Ruzicka T. "Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases. J Am Acad Dermatol. 2009 Feb. 60(2):248-55. [Medline].
Kreft B, Wohlrab J, Radant K, Danz B, Marsch WC, Fiedler E. Unrecognized radiation-induced localized scleroderma: a cause of postoperative wound-healing disorder. Clin Exp Dermatol. 2009 Oct. 34(7):e383-4. [Medline].
Laetsch B, Hofer T, Lombriser N, Lautenschlager S. Irradiation-induced morphea: x-rays as triggers of autoimmunity. Dermatology. 2011. 223(1):9-12. [Medline].
Eisendle K, Grabner T, Zelger B. Morphoea: a manifestation of infection with Borrelia species?. Br J Dermatol. 2007 Dec. 157(6):1189-98. [Medline].
Fujiwara H, Fujiwara K, Hashimoto K, et al. Detection of Borrelia burgdorferi DNA (B garinii or B afzelii) in morphea and lichen sclerosus et atrophicus tissues of German and Japanese but not of US patients. Arch Dermatol. 1997 Jan. 133(1):41-4. [Medline].
Weide B, Walz T, Garbe C. Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol. 2000 Apr. 142(4):636-44. [Medline].
Weide B, Schittek B, Klyscz T, et al. Morphoea is neither associated with features of Borrelia burgdorferi infection, nor is this agent detectable in lesional skin by polymerase chain reaction. Br J Dermatol. 2000 Oct. 143(4):780-5. [Medline].
Wienecke R, Schlüpen EM, Zöchling N, Neubert U, Meurer M, Volkenandt M. No evidence for Borrelia burgdorferi-specific DNA in lesions of localized scleroderma. J Invest Dermatol. 1995 Jan. 104(1):23-6. [Medline].
Zollinger T, Mertz KD, Schmid M, Schmitt A, Pfaltz M, Kempf W. Borrelia in granuloma annulare, morphea and lichen sclerosus: a PCR-based study and review of the literature. J Cutan Pathol. 2010 May. 37(5):571-7. [Medline].
Peroni A, Zini A, Braga V, Colato C, Adami S, Girolomoni G. Drug-induced morphea: report of a case induced by balicatib and review of the literature. J Am Acad Dermatol. 2008 Jul. 59(1):125-9. [Medline].
Hanami Y, Ohtsuka M, Yamamoto T. Paraneoplastic eosinophilic fasciitis with generalized morphea and vitiligo in a patient working with organic solvents. J Dermatol. 2015 Oct 28. [Medline].
Jablonska S, Blaszczyk M. Is superficial morphea synonymous with atrophoderma Pasini-Pierini?. J Am Acad Dermatol. 2004 Jun. 50(6):979-80; author reply 980. [Medline].
Arkachaisri T, Fertig N, Pino S, Medsger TA Jr. Serum autoantibodies and their clinical associations in patients with childhood- and adult-onset linear scleroderma. A single-center study. J Rheumatol. 2008 Dec. 35(12):2439-44. [Medline].
Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford). 2005 Mar. 44(3):274-9. [Medline].
Tomimura S, Ogawa F, Iwata Y, et al. Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. J Dermatol Sci. 2008 Oct. 52(1):47-54. [Medline].
Li SC, Liebling MS. The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep. 2009 Jul. 11(3):205-11. [Medline].
Sator PG, Radakovic S, Schulmeister K, Hönigsmann H, Tanew A. Medium-dose is more effective than low-dose ultraviolet A1 phototherapy for localized scleroderma as shown by 20-MHz ultrasound assessment. J Am Acad Dermatol. 2009 May. 60(5):786-91. [Medline].
Wortsman X, Wortsman J, Sazunic I, Carreño L. Activity assessment in morphea using color Doppler ultrasound. J Am Acad Dermatol. 2011 Nov. 65(5):942-8. [Medline].
Fett N, Werth VP. Update on morphea: part II. Outcome measures and treatment. J Am Acad Dermatol. 2011 Feb. 64(2):231-42; quiz 243-4. [Medline].
Arkachaisri T, Vilaiyuk S, Torok KS, Medsger TA Jr. 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 Feb. 49(2):373-81. [Medline].
Succaria F, Kurban M, Kibbi AG, Abbas O. Clinicopathological study of 81 cases of localized and systemic scleroderma. J Eur Acad Dermatol Venereol. 2012 May 23. [Medline].
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 May 21. [Medline].
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 Dtsch Dermatol Ges. 2016 Feb. 14 (2):199-216. [Medline].
Kroft EB, Groeneveld TJ, Seyger MM, de Jong EM. Efficacy of topical tacrolimus 0.1% in active plaque morphea: randomized, double-blind, emollient-controlled pilot study. Am J Clin Dermatol. 2009. 10(3):181-7. [Medline].
Cunningham BB, Landells ID, Langman C, Sailer DE, Paller AS. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. 1998 Aug. 39(2 Pt 1):211-5. [Medline].
Ruffatti A, Peserico A, Rondinone R, et al. Prevalence and characteristics of anti-single-stranded DNA antibodies in localized scleroderma. Comparison with systemic lupus erythematosus. Arch Dermatol. 1991 Aug. 127(8):1180-3. [Medline].
Stefanaki C, Stefanaki K, Kontochristopoulos G, et al. Topical tacrolimus 0.1% ointment in the treatment of localized scleroderma. An open label clinical and histological study. J Dermatol. 2008 Nov. 35(11):712-8. [Medline].
Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. 2005 Jan. 152(1):180-2. [Medline].
Dytoc M, Ting PT, Man J, Sawyer D, Fiorillo L. First case series on the use of imiquimod for morphoea. Br J Dermatol. 2005 Oct. 153(4):815-20. [Medline].
Torok KS, Arkachaisri T. Methotrexate and corticosteroids in the treatment of localized scleroderma: a standardized prospective longitudinal single-center study. J Rheumatol. 2012 Feb. 39(2):286-94. [Medline].
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 Jul. 63(7):1998-2006. [Medline].
Kroft EB, Creemers MC, van den Hoogen FH, Boezeman JB, de Jong EM. Effectiveness, side-effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study. Br J Dermatol. 2009 May. 160(5):1075-82. [Medline].
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 Nov. 155(5):1013-20. [Medline].
Fitch PG, Rettig P, Burnham JM, et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol. 2006 Mar. 33(3):609-14. [Medline].
Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol. 2005 Jul. 141(7):847-52. [Medline].
Uziel Y, Feldman BM, Krafchik BR, Yeung RS, Laxer RM. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr. 2000 Jan. 136(1):91-5. [Medline].
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. Rheumatology (Oxford). 2009 Nov. 48(11):1410-3. [Medline].
Crespo MP, Mas IB, Diaz JM, Costa AL, Nortes IB. Rapid response to cyclosporine and maintenance with methotrexate in linear scleroderma in a young girl. Pediatr Dermatol. 2009 Jan-Feb. 26(1):118-20. [Medline].
Frumholtz L, Roux J, Bagot M, Rybojad M, Bouaziz JD. Treatment of Generalized Deep Morphea With Everolimus. JAMA Dermatol. 2016 Jul 20. [Medline].
Elst EF, Van Suijlekom-Smit LW, Oranje AP. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children. Pediatr Dermatol. 1999 Jan-Feb. 16(1):53-8. [Medline].
Hulshof MM, Bouwes Bavinck JN, Bergman W, et al. Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. 2000/12. 43(6):1017-23.
Fett NM. Morphea: evidence-based recommendations for treatment. Indian J Dermatol Venereol Leprol. 2012 Mar-Apr. 78(2):135-41. [Medline].
El-Mofty M, Mostafa W, El-Darouty M, Bosseila M, Nada H, Yousef R. Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis. Photodermatol Photoimmunol Photomed. 2004 Jun. 20(3):148-56. [Medline].
Vasquez 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 Mar. 70(3):481-8. [Medline]. [Full Text].
Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006 Mar. 54(3):440-7. [Medline].
Kreuter A, Gambichler T, Avermaete A, et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol. 2001 May-Jun. 18(3):241-5. [Medline].
Ozdemir M, Engin B, Toy H, Mevlitoglu I. Treatment of plaque-type localized scleroderma with retinoic acid and ultraviolet A plus the photosensitizer psoralen: a case series. J Eur Acad Dermatol Venereol. 2008 Apr. 22(4):519-21. [Medline].
Sapadin AN, Fleischmajer R. Treatment of scleroderma. Arch Dermatol. 2002 Jan. 138(1):99-105. [Medline].
Neustadter JH, Samarin F, Carlson KR, Girardi M. Extracorporeal photochemotherapy for generalized deep morphea. Arch Dermatol. 2009 Feb. 145(2):127-30. [Medline].
Eisen D, Alster TS. Use of a 585 nm pulsed dye laser for the treatment of morphea. Dermatol Surg. 2002 Jul. 28(7):615-6. [Medline].
Karrer S, Abels C, Landthaler M, Szeimies RM. Topical photodynamic therapy for localized scleroderma. Acta Derm Venereol. 2000 Jan-Feb. 80(1):26-7. [Medline].
Batchelor R, Lamb S, Goulden V, Stables G, Goodfield M, Merchant W. Photodynamic therapy for the treatment of morphoea. Clin Exp Dermatol. 2008 Aug. 33(5):661-3. [Medline].
Badea I, Taylor M, Rosenberg A, Foldvari M. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology (Oxford). 2009 Mar. 48(3):213-21. [Medline].
Coelho-Macias V, Mendes-Bastos P, Assis-Pacheco F, Cardoso J. Imatinib: a novel treatment approach for generalized morphea. Int J Dermatol. 2014 Apr 2. [Medline].
Hunzelmann N, Anders S, Fierlbeck G, Hein R, Herrmann K, Albrecht M, et al. Double-blind, placebo-controlled study of intralesional interferon gamma for the treatment of localized scleroderma. J Am Acad Dermatol. 1997 Mar. 36(3 Pt 1):433-5. [Medline].
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 Oct. 37(10):2174-9. [Medline].