Complications
- In the linear and deep morphea subtypes, joint contractures, subcutaneous atrophy, and growth failure can be deforming and disabling.
- Rarely, pansclerotic morphea can give rise to chronic ulcers as well as squamous cell carcinoma.[10]
Prognosis
- Plaque-type morphea is a self-limited condition that tends to slowly involute with time; the duration of disease activity of each individual lesion averages 3-5 years; however, patients tend to develop new lesions for as long as 25 years.
- Linear lesions tend to persist for longer than plaque-type lesions, but they often improve over the years. However, linear morphea, especially the en coup de sabre subtype, may remit and reactivate, remain unchanged, or become more extensive with time. In addition, patients with linear lesions may develop limb atrophy and contractures that result in limited movement and permanent disability. Neurologic and ocular sequelae represent other potential complications of craniofacial linear morphea. Long-term follow-up and serial imaging may be indicated.
- Disabling pansclerotic morphea of children is a rare, aggressive, and mutilating variant of deep morphea that begins before age 14 years and has a disease course of relentless progression and severe disability.
- Rare cases of morphea progressing to systemic scleroderma are described.[49]
Zulian F. Systemic manifestations in localized scleroderma. Curr Rheumatol Rep. Dec 2004;6(6):417-24. [Medline].
Chung L, Lin J, Furst DE, Fiorentino D. Systemic and localized scleroderma. Clin Dermatol. Sep-Oct 2006;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. May 2009;145(5):545-50. [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. Apr 1996;106(4):729-33. [Medline].
Ihn H, Sato S, Fujimoto M, Kikuchi K, Takehara K. Clinical significance of serum levels of soluble interleukin-2 receptor in patients with localized scleroderma. Br J Dermatol. May 1996;134(5):843-7. [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. Jul 1995;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. Jan 2004;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. Jan 2000;42(1 Pt 1):64-9. [Medline].
Kroft EB, de Jong EM, Evers AW. Psychological distress in patients with morphea and eosinophilic fasciitis. Arch Dermatol. Sep 2009;145(9):1017-22. [Medline].
Zulian F. New developments in localized scleroderma. Curr Opin Rheumatol. Sep 2008;20(5):601-7. [Medline].
Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko's lines. J Am Acad Dermatol. Feb 1998;38(2 Pt 2):366-8. [Medline].
Weibel L, Harper JI. Linear morphoea follows Blaschko's lines. Br J Dermatol. Jul 2008;159(1):175-81. [Medline].
Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. Jan 2006;117(1):e132-6. [Medline].
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. Feb 2009;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. Oct 2009;34(7):e383-4. [Medline].
Eisendle K, Grabner T, Zelger B. Morphoea: a manifestation of infection with Borrelia species?. Br J Dermatol. Dec 2007;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. Jan 1997;133(1):41-4. [Medline].
Weide B, Walz T, Garbe C. Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol. Apr 2000;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. Oct 2000;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. Jan 1995;104(1):23-6. [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. Jul 2008;59(1):125-9. [Medline].
Jablonska S, Blaszczyk M. Is superficial morphea synonymous with atrophoderma Pasini-Pierini?. J Am Acad Dermatol. Jun 2004;50(6):979-80; author reply 980. [Medline].
Shono S, Imura M, Ota M, Osaku A, Shinomiya S, Toda K. Lichen sclerosus et atrophicus, morphea, and coexistence of both diseases. Histological studies using lectins. Arch Dermatol. Sep 1991;127(9):1352-6. [Medline].
Tomimura S, Ogawa F, Iwata Y, et al. Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. J Dermatol Sci. Oct 2008;52(1):47-54. [Medline].
Li SC, Liebling MS. The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep. Jul 2009;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. May 2009;60(5):786-91. [Medline].
Cunningham BB, Landells ID, Langman C, Sailer DE, Paller AS. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):211-5. [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. Nov 2008;35(11):712-8. [Medline].
Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. Jan 2005;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. Oct 2005;153(4):815-20. [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. May 2009;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. Nov 2006;155(5):1013-20. [Medline].
Fitch PG, Rettig P, Burnham JM, et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol. Mar 2006;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. Jul 2005;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. Jan 2000;136(1):91-5. [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. Jan-Feb 1999;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.
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. Jan-Feb 2009;26(1):118-20. [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). Nov 2009;48(11):1410-3. [Medline].
Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. May 1988;17(4):221-31. [Medline].
Falanga V, Medsger TA Jr. D-penicillamine in the treatment of localized scleroderma. Arch Dermatol. May 1990;126(5):609-12. [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. May-Jun 2001;18(3):241-5. [Medline].
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. Mar 2006;54(3):440-7. [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. Apr 2008;22(4):519-21. [Medline].
Sapadin AN, Fleischmajer R. Treatment of scleroderma. Arch Dermatol. Jan 2002;138(1):99-105. [Medline].
Neustadter JH, Samarin F, Carlson KR, Girardi M. Extracorporeal photochemotherapy for generalized deep morphea. Arch Dermatol. Feb 2009;145(2):127-30. [Medline].
Eisen D, Alster TS. Use of a 585 nm pulsed dye laser for the treatment of morphea. Dermatol Surg. Jul 2002;28(7):615-6. [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). Mar 2009;48(3):213-21. [Medline].
Toledano C, Rabhi S, Kettaneh A, et al. Localized scleroderma: a series of 52 patients. Eur J Intern Med. May 2009;20(3):331-6. [Medline].
Dutz J. Treatment options for localized scleroderma. Skin Therapy Lett. 2000;5(2):3-5. [Medline].
el-Azhary RA, Aponte CC, Nelson AM. Do antihistone autoantibodies reflect disease activity in linear scleroderma?. Arch Dermatol. Jun 2004;140(6):759-60. [Medline].
Hayakawa I, Hasegawa M, Takehara K, Sato S. Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma. Arthritis Rheum. Jan 2004;50(1):227-32. [Medline].
Karrer S, Abels C, Landthaler M, Szeimies RM. Topical photodynamic therapy for localized scleroderma. Acta Derm Venereol. Jan-Feb 2000;80(1):26-7. [Medline].
Kerscher M, Meurer M, Sander C, et al. PUVA bath photochemotherapy for localized scleroderma. Evaluation of 17 consecutive patients. Arch Dermatol. Nov 1996;132(11):1280-2. [Medline].
Kerscher M, Volkenandt M, Gruss C, et al. Low-dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. Jan 1998;38(1):21-6. [Medline].
Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. Mar-Apr 2003;13(2):171-6. [Medline].
Morison WL. Psoralen UVA therapy for linear and generalized morphea. J Am Acad Dermatol. Oct 1997;37(4):657-9. [Medline].
Nagai M, Hasegawa M, Takehara K, Sato S. Novel autoantibody to Cu/Zn superoxide dismutase in patients with localized scleroderma. J Invest Dermatol. Mar 2004;122(3):594-601. [Medline].
Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. Nov 1995;70(11):1068-76. [Medline].
Rosenberg AM, Uziel Y, Krafchik BR, et al. Antinuclear antibodies in children with localized scleroderma. J Rheumatol. Dec 1995;22(12):2337-43. [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. Aug 1991;127(8):1180-3. [Medline].
Schaffer JV, Carroll C, Dvoretsky I, Huether MJ, Girardi M. Postirradiation morphea of the breast presentation of two cases and review of the literature. Dermatology. 2000;200(1):67-71. [Medline].
Sommer A, Gambichler T, Bacharach-Buhles M, von Rothenburg T, Altmeyer P, Kreuter A. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. Feb 2006;54(2):227-33. [Medline].
Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford). Mar 2005;44(3):274-9. [Medline].
Vierra E, Cunningham BB. Morphea and localized scleroderma in children. Semin Cutan Med Surg. Sep 1999;18(3):210-25. [Medline].
Wollina U, Looks A, Uhlemann C, Wollina K. Pansclerotic morphea of childhood-follow-up over 6 years. Pediatr Dermatol. May-Jun 1999;16(3):245-7. [Medline].
Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). May 2006;45(5):614-20. [Medline].
Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. Sep 2005;52(9):2873-81. [Medline].

