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Three-Dimensional Cell Culture Models to Investigate the Epithelial Barrier in Eosinophilic Esophagitis
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[Eosinophllic fasciitis (Shulman disease)].

L Arlettaz1, M Abdou, F Pardon

  • 1Service de génétique et d'immunologie, Institut Central/Hôpital du Valais. lionel.arlettaz@hopitalvs.ch

Revue Medicale Suisse
|May 19, 2012
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Summary

Eosinophilic fasciitis is a rare connective tissue disease causing symmetrical skin swelling and hardening, primarily in limbs. Corticosteroids are effective in over 70% of cases, with immunosuppressants used for remission.

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Area of Science:

  • Rheumatology
  • Connective Tissue Diseases
  • Dermatology

Background:

  • Eosinophilic fasciitis (EF) is a rare connective tissue disease first described in 1974.
  • Characterized by symmetrical skin swelling, eosinophilia, and progressive skin induration, primarily affecting limbs.
  • Facial and hand involvement and Raynaud phenomenon are typically absent; no autoantibodies are detected.

Purpose of the Study:

  • To summarize the key features, diagnostic methods, and treatment outcomes of Eosinophilic Fasciitis.
  • To provide an overview of this rare inflammatory condition.

Main Methods:

  • Diagnosis relies on clinical presentation, Magnetic Resonance Imaging (MRI), and histological examination.
  • MRI reveals fascial thickening and contrast enhancement.
  • Histology shows inflammation and thickening of the fascia with lymphocytic and plasmacytic infiltration.

Main Results:

  • Eosinophilic fasciitis presents with symmetrical limb swelling and progressive skin hardening.
  • Diagnostic imaging (MRI) and biopsy confirm fascial inflammation and thickening.
  • High-dose corticosteroids demonstrate efficacy in over 70% of patients.

Conclusions:

  • Eosinophilic fasciitis is a distinct entity diagnosed through a combination of clinical, imaging, and histological findings.
  • Corticosteroid therapy is the primary treatment, with immunosuppressive agents reserved for refractory cases.
  • Early diagnosis and treatment are crucial for managing this rare connective tissue disease.