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Related Experiment Videos

Internal involvement in localized scleroderma

L Dehen1, J C Roujeau, A Cosnes

  • 1Department of Dermatology, Hôpital Henri Mondor, Université Paris XII, Créteil, France.

Medicine
|September 1, 1994
PubMed
Summary
This summary is machine-generated.

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Internal organ involvement in localized scleroderma (morphea) is often silent and mild, typically not affecting prognosis. Male gender, more lesions, and hypergammaglobulinemia predict internal involvement.

Area of Science:

  • Rheumatology
  • Dermatology
  • Internal Medicine

Background:

  • Localized scleroderma, also known as morphea, can present with or without linear scleroderma.
  • Internal organ involvement in morphea is a concern, but its frequency, impact, and predictors require further elucidation.

Purpose of the Study:

  • To determine the frequency, prognosis, and predictors of internal organ involvement in patients with localized scleroderma.
  • To differentiate morphea from systemic scleroderma based on internal involvement patterns.

Main Methods:

  • A cohort of 76 patients with localized scleroderma was studied.
  • A subset of 53 patients underwent systematic investigation for internal involvement.
  • Statistical analysis was used to identify predictors and assess prognosis.

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Main Results:

  • Internal involvement was detected in 16 of 53 systematically investigated patients.
  • Most internal involvement (14/16) was asymptomatic and mild, affecting esophageal function and pulmonary gas exchange.
  • Only 2 patients had severe symptomatic visceral disease; one developed systemic scleroderma.
  • Predictors of internal involvement included male gender, increased number of lesions, and hypergammaglobulinemia.
  • Seroimmunologic abnormalities showed no predictive value.

Conclusions:

  • Mild esophageal and pulmonary involvement are common but usually silent in morphea and do not impair prognosis.
  • Routine screening for internal involvement in asymptomatic morphea patients is not justified.
  • Morphea and systemic scleroderma appear to be distinct disease entities.