Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis Follow-up

  • Author: Mariana J Kaplan, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 16, 2015
 

Further Outpatient Care

See the list below:

  • Periodic follow-up is recommended to detect early spread of lesions or other chronic complications.
Next

Deterrence/Prevention

See the list below:

  • Early evaluation for prevention and treatment of joint and muscle contractures and deformities is important.
  • Cases that involve children with severe facial deformities should be referred to a plastic surgeon early for prompt evaluation and consultation about possible treatment.
Previous
Next

Complications

See the list below:

  • Joint contractures, deformities, and severe limb atrophy can occur in patients with linear scleroderma.
    • Severe atrophy of underlying muscle and bone also can occur.
    • Disabling pansclerotic morphea of childhood can include claw deformity of the hands.
    • Arthralgias, osteoporosis, flexion contractures, and other bone changes are common.
  • A 1.5- to 7-cm leg length discrepancy occurs in 20% of patients with linear scleroderma.
  • Psychosocial problems related to adjusting to deformities and disfiguration can occur.
  • Loss of eyebrow or eyelashes, ptosis, pseudooculomotor palsy, uveitis, asymmetry of the tongue, and altered dentition and dental caries can occur in patients with morphea en coup de sabre.
  • Overt seizures may occur in patients with Parry-Romberg syndrome.
  • Aplastic anemia, thrombocytopenia, and hemolytic anemia have been reported in patients with eosinophilic fasciitis.
  • Intense pain may be present, particularly in disabling pansclerotic morphea of children and in en coup de sabre lesions, probably due to cutaneous nerve involvement.
  • In patients with generalized morphea, contractures may occur in limbs, and mobility may be restricted. Marked chest wall involvement may cause difficulty in breathing due to constriction of the thorax.
  • In patients with mediastinal and retroperitoneal fibrosis, complications are related to obstruction of vascular structures, bronchi, or ureters.
  • Complications in Dupuytren contracture are related to progressive hand deformities.
Previous
Next

Prognosis

See the list below:

  • In general, localized and regional fibrosing disorders carry a good prognosis.
  • Plaquelike lesions tend to improve with time. The duration of the activity usually is 3-5 years, but some lesions persist longer.
  • Residual pigmentation persists long term in about one-third of the patients.
  • Linear lesions tend to persist longer than plaque lesions.
  • Contractures may limit joint movements and may lead to clawing of the hands.
  • Facial hemiatrophy persists.
  • Individuals with the severe inflammatory subtypes, en coup de sabre linear scleroderma, or Parry-Romberg disease can have severe functional limitations.
  • In mediastinal and retroperitoneal fibrosis, the prognosis is related to the amount of irreversible obstruction.
Previous
 
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 Federation for Medical Research, Central Society for Clinical and Translational Research, Clinical Immunology Society, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, 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, American College of Rheumatology

Disclosure: Nothing to disclose.

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

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

  3. Namazi MR. Imiquimod: a potential weapon against morphea and fibromatoses. J Drugs Dermatol. 2004 Jul-Aug. 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. 1999 Jan. 26(1):11-7. [Medline].

  5. Dehen L, Roujeau JC, Cosnes A, et al. Internal involvement in localized scleroderma. Medicine (Baltimore). 1994 Sep. 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. 1999 Feb. 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. 1986 Jun. 104(6):849-57. [Medline].

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

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

  10. Jablonska S, Blaszczyk M. Sclerodermalike diseases. Clin Dermatol. 1994 Jul-Sep. 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. 1998 Jan. 38(1):21-6. [Medline].

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

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

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

  15. Schumacher HR. Multifocal fibrosclerosis. 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. 1997 Nov. 9(6):562-70. [Medline].

Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.