eMedicine Specialties > Dermatology > Connective Tissue Diseases

Morphea: Follow-up

Author: Kendra G Bergstrom, MD, Private Practice, Manhattan Dermatology
Contributor Information and Disclosures

Updated: Aug 3, 2009

Follow-up

Complications

  • In the linear and deep morphea subtypes, joint contractures, subcutaneous atrophy, and growth failure can be deforming and disabling.

Prognosis

  • Plaque-type morphea is a self-limited condition that tends to slowly involute with time; the duration of disease activity averages 3-5 years, although it may last 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.

Miscellaneous

Special Concerns

  • Extracutaneous manifestations of morphea are more common in the pediatric population, most often affecting the joints, central nervous system, and eyes.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Michael Girardi, MD, Kendra G. Bergstrom, MD, and Julie V. Schaffer, MD, to the development and writing of this article.



More on Morphea

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Differential Diagnoses & Workup: Morphea
Treatment & Medication: Morphea
Follow-up: Morphea
Multimedia: Morphea
References
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References

  1. Saleh Z, Arayssi T, Saleh Z, Ghosn S. Superficial morphea: 20-year follow up in a patient with concomitant psoriasis vulgaris. J Cutan Pathol. Jul 7 2009;[Medline].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. McKenna DB, Benton EC. A tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines. Clin Exp Dermatol. Nov 1999;24(6):467-8. [Medline].

  7. 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].

  8. 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].

  9. Aberer E, Neumann R, Stanek G. Is localised scleroderma a Borrelia infection?. Lancet. Aug 3 1985;2(8449):278. [Medline].

  10. 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].

  11. Weide B, Walz T, Garbe C. Is morphoea caused by Borrelia burgdorferi? A review. Br J Dermatol. Apr 2000;142(4):636-44. [Medline].

  12. Weide B, Schittek B, Klyscz T, Schüz K, Stark M, Rassner G. 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].

  13. Wienecke R, Schlupen EM, Zochling 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].

  14. 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].

  15. 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].

  16. 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].

  17. Khandpur S, Singh S, Sharma VK, Gupta R, Singh MK. Linear morphea with secondary mucinosis. Indian J Dermatol Venereol Leprol. Jul-Aug 2009;75(4):388-90. [Medline].

  18. Pugashetti R, Zedek DC, Seiverling EV, Rajendran P, Berger T. Dermal mucinosis as a sign of venous insufficiency. J Cutan Pathol. Jul 10 2009;[Medline].

  19. 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].

  20. 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].

  21. Fitch PG, Rettig P, Burnham JM, et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol. Mar 2006;33(3):609-14. [Medline].

  22. 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].

  23. Seyger MM, van den Hoogen FH, de Boo T, de Jong EM. Low-dose methotrexate in the treatment of widespread morphea. J Am Acad Dermatol. Aug 1998;39(2 Pt 1):220-5. [Medline].

  24. 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].

  25. 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].

  26. 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. Dec 2000;43(6):1017-23.

  27. Koeger AC, Rozenberg S, Fautrel B. Effectiveness of topical calcitriol for localized scleroderma. J Rheumatol. Jan 1999;26(1):239-40. [Medline].

  28. Falanga V, Medsger TA Jr. D-penicillamine in the treatment of localized scleroderma. Arch Dermatol. May 1990;126(5):609-12. [Medline].

  29. Möhrenschlager M, Jung C, Ring J, Abeck D. Effect of penicillin G on corium thickness in linear morphea of childhood: An analysis using ultrasound technique. Pediatr Dermatol. Jul-Aug 1999;16(4):314-6. [Medline].

  30. Kreuter A, Gambichler T, Avermaete A, Jansen T, Hoffmann M, Hoffmann K. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol. May-Jun 2001;18(3):241-5. [Medline].

  31. Kreuter A, Hyun J, Stucker 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].

  32. 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].

  33. Daoud MS, Su WP, Leiferman KM, Perniciaro C. Bullous morphea: clinical, pathologic, and immunopathologic evaluation of thirteen cases. J Am Acad Dermatol. Jun 1994;30(6):937-43. [Medline].

  34. Davis DA, Cohen PR, McNeese MD, Duvic M. Localized scleroderma in breast cancer patients treated with supervoltage external beam radiation: radiation port scleroderma. J Am Acad Dermatol. Dec 1996;35(6):923-7. [Medline].

  35. Dutz J. Treatment options for localized scleroderma. Skin Therapy Lett. 2000;5(2):3-5. [Medline].

  36. 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].

  37. Falanga V, Medsger TA Jr, Reichlin M. Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea. Arch Dermatol. Mar 1987;123(3):350-3. [Medline].

  38. Falanga V, Medsger TA Jr, Reichlin M, Rodnan GP. Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med. Jun 1986;104(6):849-57. [Medline].

  39. 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].

  40. Ihn H, Fujimoto M, Sato S, et al. Increased levels of circulating intercellular adhesion molecule-1 in patients with localized scleroderma. J Am Acad Dermatol. Oct 1994;31(4):591-5. [Medline].

  41. Itin PH, Schiller P. Double-lined frontoparietal scleroderma en coup de sabre. Dermatology. 1999;199(2):185-6. [Medline].

  42. 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].

  43. Kencka D, Blaszczyk M, Jablonska S. Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea. Dermatology. 1995;190(3):203-6. [Medline].

  44. 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].

  45. 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].

  46. Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. Jan 2005;152(1):180-2. [Medline].

  47. Martin L, Piette F, Blanc P, et al. Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol. Nov 2005;153(5):932-6. [Medline].

  48. Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. Mar-Apr 2003;13(2):171-6. [Medline].

  49. McNiff JM, Glusac EJ, Lazova RZ, Carroll CB. Morphea limited to the superficial reticular dermis: an underrecognized histologic phenomenon. Am J Dermatopathol. Aug 1999;21(4):315-9. [Medline].

  50. Morison WL. Psoralen UVA therapy for linear and generalized morphea. J Am Acad Dermatol. Oct 1997;37(4):657-9. [Medline].

  51. 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].

  52. Nelson AM. Localized scleroderma including morphea, linear scleroderma, and eosinophilic fasciitis. Curr Probl Pediatr. Oct 1996;26(9):318-24. [Medline].

  53. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc. Nov 1995;70(11):1068-76. [Medline].

  54. Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. Jan 1997;24(1):73-80. [Medline].

  55. Rosenberg AM, Uziel Y, Krafchik BR, et al. Antinuclear antibodies in children with localized scleroderma. J Rheumatol. Dec 1995;22(12):2337-43. [Medline].

  56. 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].

  57. Sapadin AN, Fleischmajer R. Treatment of scleroderma. Arch Dermatol. Jan 2002;138(1):99-105. [Medline].

  58. 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].

  59. 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].

  60. Stege H, Berneburg M, Humke S, et al. High-dose UVA1 radiation therapy for localized scleroderma. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):938-44. [Medline].

  61. Takehara K, Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford). Mar 2005;44(3):274-9. [Medline].

  62. Uziel Y, Krafchik BR, Silverman ED, Thorner PS, Laxer RM. Localized scleroderma in childhood: a report of 30 cases. Semin Arthritis Rheum. Apr 1994;23(5):328-40. [Medline].

  63. Vierra E, Cunningham BB. Morphea and localized scleroderma in children. Semin Cutan Med Surg. Sep 1999;18(3):210-25. [Medline].

  64. 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].

  65. 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].

  66. 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].

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Further Reading

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Keywords

morphea, morphoea, guttate morphea, keloidal morphea, nodular morphea, bullous morphea, generalized morphea, linear morphea, linear scleroderma, localized scleroderma, plaque-type morphea, atrophoderma of Pasini and Pierini, en coup de sabre, progressive hemifacial atrophy, progressive facial hemiatrophy, Parry-Romberg syndrome, deep morphea, morphea profunda, subcutaneous morphea, eosinophilic fasciitis, Shulman syndrome, disabling pansclerotic morphea

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Contributor Information and Disclosures

Author

Kendra G Bergstrom, MD, Private Practice, Manhattan Dermatology
Kendra G Bergstrom, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Medical Editor

Peter Fritsch, MD, Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria
Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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