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

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

Further Outpatient Care

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

  • 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.
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Complications

  • 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.
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Prognosis

  • 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.
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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

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  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].

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  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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