Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Treatment & Management

  • Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Aug 19, 2011
 

Medical Care

  • Most patients with plaque morphea experience spontaneous remission and require no specific treatment. Treatment depends entirely on the severity of the findings. Intralesional injections of corticosteroids might be helpful in early stages.
    • The progression of Dupuytren contracture varies, ranging from little or no change over many years to rapid progression and complete flexion contracture of one or more digits.
    • The treatment of mediastinal and retroperitoneal fibrosis has not been well studied, although corticosteroids, immunosuppressive agents, and tamoxifen appear to be effective.
  • If the lesions spread (as in generalized morphea), anti-inflammatory or immunosuppressive medications may be indicated.
  • Although numerous therapeutic agents have been used for morphea, treatment remains unsatisfactory.
  • Daily antimalarial agents may be beneficial, especially when lesions are highly inflammatory.
  • In linear scleroderma and deep morphea, aggressive treatment, including systemic corticosteroids, may be necessary. Topical corticosteroids may also be useful.
  • Occasionally, other disease-modifying agents, including d-penicillamine, azathioprine, sulfasalazine, methotrexate, and cyclophosphamide, are necessary to control a severe inflammatory process.
  • Plasmapheresis may be useful in some patients, but no randomized controlled trials have been published.
  • Reports indicate that patients with severe localized scleroderma have been treated successfully with psoralen plus ultraviolet light of the A wave length (PUVA) bath photochemotherapy.[1]
    • Low-dose UVA1 phototherapy can be highly effective for sclerotic plaques, even in patients with advanced localized fibrosing disorders with rapidly evolving lesions despite conventional therapy.
    • Patients are usually irradiated with 20 J/cm2 UVA1 for 12 weeks, with a cumulative UVA1 dose of 600 J/cm2.
  • A recent study reported that occlusive treatment with tacrolimus ointment can be useful in localized scleroderma.[2] Imiquimod has also been suggested as a potential treatment for morphea and fibromatoses, but more studies are needed.[3]
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Surgical Care

  • Tendon-lengthening procedures and surgical release of joint contractures are sometimes necessary.
  • Amputation may be necessary as a consequence of severe flexural deformity.
  • Often, patients with en coup de sabre or Parry-Romberg syndrome require surgical reconstruction of the face and scalp.
  • Reports indicate that en coup de sabre lesions have been repaired effectively with a combination of an expanded skin flap and a hydroxyapatite implant.
  • When actual contractures occur in Dupuytren contracture, surgical intervention is desirable. Limited fasciotomy is effective in most instances. More radical procedures, including amputation, are necessary in rare cases. Palmar fasciotomy is a useful and more benign procedure. Surgical management is often required to treat the complications of both retroperitoneal and mediastinal fibrosis.
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Consultations

  • Patients with the linear and deep types of morphea require physical therapy to prevent joint deformities and skin contractures. Heat treatment and massage might be helpful.
  • Psychotherapy for people with deformities and disfiguration is very important.
  • Early evaluation by a reconstructive or plastic surgeon is important for patients with en coup de sabre lesions or Parry-Romberg disease.
  • Evaluation by a hand surgeon may be indicated in patients with Dupuytren contracture for consideration of releasing the contractures.
  • Surgical consultation may be considered in patients with mediastinal and retroperitoneal fibrosis if obstructive lesions occur.
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Activity

  • Physical therapy is very important for patients prone to develop joint and muscle contractures and deformities. Joint mobility should be maintained.
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Contributor Information and Disclosures
Author

Mariana J Kaplan, MD  Assistant Professor, Department of Internal Medicine, Division of Rheumatology, University of Michigan Medical School

Mariana J Kaplan, MD is a member of the following medical societies: American Association of Immunologists, American College of Rheumatology, American Federation for Medical Research, American Medical Association, Central Society for Clinical Research, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Kristine M Lohr, MD, MS  Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

References

  1. Todd DJ, Askari A, Ektaish E. PUVA therapy for disabling pansclerotic morphoea of children. Br J Dermatol. Jan 1998;138(1):201-2. [Medline].

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

  3. Namazi MR. Imiquimod: a potential weapon against morphea and fibromatoses. J Drugs Dermatol. Jul-Aug 2004;3(4):362-3. [Medline].

  4. Mizutani H, Yoshida T, Nouchi N, et al. Topical tocoretinate improved hypertrophic scar, skin sclerosis in systemic sclerosis and morphea. J Dermatol. Jan 1999;26(1):11-7. [Medline].

  5. Dehen L, Roujeau JC, Cosnes A, et al. Internal involvement in localized scleroderma. Medicine (Baltimore). Sep 1994;73(5):241-5. [Medline].

  6. Eguchi T, Harii K, Sugawara Y. Repair of a large "coup de sabre" with soft-tissue expansion and artificial bone graft. Ann Plast Surg. Feb 1999;42(2):207-10. [Medline].

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

  8. Ghersetich I, Teofoli P, Benci M, et al. Localized scleroderma. Clin Dermatol. Apr-Jun 1994;12(2):237-42. [Medline].

  9. Gilkeson GS, Allen NB. Retroperitoneal fibrosis. A true connective tissue disease. Rheum Dis Clin North Am. Feb 1996;22(1):23-38. [Medline].

  10. Jablonska S, Blaszczyk M. Sclerodermalike diseases. Clin Dermatol. Jul-Sep 1994;12(3):437-48. [Medline].

  11. 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].

  12. Mathisen DJ, Grillo HC. Clinical manifestation of mediastinal fibrosis and histoplasmosis. Ann Thorac Surg. Dec 1992;54(6):1053-7; discussion 1057-8. [Medline].

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

  14. Schachter RK. Localized scleroderma. Curr Opin Rheumatol. Dec 1989;1(4):505-11. [Medline].

  15. Schumacher HR. Multifocal fibrosclerosis. In: Cecil Textbook of Medicine. WB Saunders Co; 2000:1561-62.

  16. Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. Nov 1997;9(6):562-70. [Medline].

 
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This photograph shows morphea en plaque on the trunk of a patient. There is a distinctive border separating the plaque from the surrounding normal skin (reproduced with permission of Mayo Clinic Proceedings).
This photograph shows generalized morphea on the trunk of a patient (reproduced with permission from Mayo Clinic Proceedings).
CT scan of the abdomen showing the typical paraaortic mass of retroperitoneal fibrosis.
 
 
 
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