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

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

Background

Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. These lesions are characterized by circumscribed fibrotic areas involving different levels of the dermis, subcutis, and, sometimes, underlying soft tissue and bone. Although the clinical course of the disease is often benign, widespread lesions and disabling joint contractures may lead to significant complications.

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Pathophysiology

Localized fibrosing disorders include several clinical and histopathological conditions that are similar to the skin involvement of systemic sclerosis, but the systemic features are absent. Localized fibrosing disorders can be classified into several subtypes that include morphea, generalized morphea, and linear scleroderma, in which facial involvement is termed en coup de sabre. Linear scleroderma and morphea can coexist in the same patient. Other fibrosing conditions mentioned in this article include retroperitoneal fibrosis, mediastinal fibrosis, and Dupuytren contracture.

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Epidemiology

Frequency

United States

The frequency of morphea ranges between 3.4 cases per million adults to 2.7 cases per 100,000 population, depending on the report.

International

No particular geographic distribution has been noted.

Mortality/Morbidity

  • The clinical course of the disease is often benign, but widespread lesions and disabling joint contractures may lead to significant complications.
  • Localized fibrosing disorders are only rarely life threatening, but they can severely affect quality of life, particularly in children.

Race

  • Morphea seems to be more prevalent in whites than in blacks.

Sex

  • Morphea is more common in women than in men, with a female-to-male ratio of 3-4:1.
  • Morphea may appear or worsen during pregnancy.

Age

  • Children are more likely than adults to develop localized forms of scleroderma. Because of the impact on growth, these lesions can result in major facial or limb asymmetry, flexion contractures, and disability.
  • Linear scleroderma tends to affect children and adolescents.
  • Morphea en plaque is more common in adults.
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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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  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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