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

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

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

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  • 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

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

Sex

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  • 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

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  • 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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