eMedicine Specialties > Rheumatology > Soft Tissue and Regional Rheumatic Disease

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

Author: Mariana J Kaplan, MD, Assistant Professor, Department of Internal Medicine, Division of Rheumatology, University of Michigan Medical School
Contributor Information and Disclosures

Updated: Dec 3, 2008

Follow-up

Further Outpatient Care

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

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.

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.

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.

Miscellaneous

Medicolegal Pitfalls

  • Distinguish localized scleroderma from systemic sclerosis in early stages.

Special Concerns

  • In children, lesions can have a major impact on growth and result in major facial or limb asymmetry, flexion contractures, and disability.
 


More on Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis

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Treatment & Medication: Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis
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References
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References

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

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Further Reading

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Keywords

localized fibrosing disorders, linear scleroderma, morphea, regional fibrosis, localized scleroderma, systemic sclerosis, generalized morphea, en coup de sabre, retroperitoneal fibrosis, mediastinal fibrosis, Dupuytren contracture, morphea en plaque, plaque morphea, guttate morphea, keloid morphea, lichen sclerosus et atrophicus, atrophoderma of Pasini and Pierini, bullous morphea, deep morphea, subcutaneous morphea, morphea profunda, disabling pansclerotic morphea of childhood, eosinophilic fasciitis

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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.

Medical Editor

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, 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 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; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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