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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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[Scleroderma-like disorders].

S Deshayes1, H de Boysson1, L Geffray1

  • 1Service de médecine interne, hôpital Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.

La Revue De Medecine Interne
|February 7, 2016
PubMed
Summary
This summary is machine-generated.

Skin hardening and thickening can indicate various diseases beyond scleroderma. Differentiating these scleroderma-like disorders is crucial as their treatments and prognoses differ significantly.

Keywords:
Eosinophilic fasciitisFasciite avec éosinophilieScleredemaScleroderma-like disordersScleromyxedemaScléromyxœdèmeSclérœdèmeSyndrome sclérodermiforme

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Area of Science:

  • Dermatology
  • Rheumatology
  • Internal Medicine

Background:

  • Skin hardening and thickening are common findings with diverse etiologies.
  • These signs can be associated with immune-mediated, metabolic, neoplastic, toxic, or genetic diseases, and protein deposits.
  • Distinguishing these conditions from scleroderma is essential for appropriate management.

Purpose of the Study:

  • To review scleroderma-like disorders that present with skin hardening and thickening.
  • To highlight the characteristics of these conditions.
  • To emphasize the importance of differentiating them from true scleroderma.

Main Methods:

  • Literature review of scleroderma-like disorders.
  • Analysis of clinical features, diagnostic criteria, and treatment approaches.
  • Comparison with established scleroderma diagnostic markers.

Main Results:

  • Scleroderma-like disorders share skin manifestations with scleroderma but lack specific markers like Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies.
  • These alternative diagnoses include a range of systemic and localized conditions.
  • Failure to identify these mimics can lead to misdiagnosis and inappropriate treatment.

Conclusions:

  • The absence of definitive scleroderma markers necessitates a thorough investigation for alternative scleroderma-like disorders.
  • Accurate diagnosis of these mimics is critical for guiding patient treatment and predicting prognosis.
  • This review provides a framework for identifying and managing these challenging conditions.