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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
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.
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.
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