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[Eosinophilic fasciitis (Shulman's syndrome)]
J Lattmann1, H Adam, A von Hochstetter
1Departement für Innere Medizin, Universitätsspital Zürich.
Deutsche Medizinische Wochenschrift (1946)
|November 30, 1990
Summary
Eosinophilic fasciitis (Shulman syndrome) is a rare condition causing painful limb swelling. Early prednisone treatment effectively reduced symptoms and eosinophilia, though long-term management is often required.
Area of Science:
- Rheumatology
- Dermatology
- Immunology
Background:
- Eosinophilic fasciitis (Shulman syndrome) is a rare connective tissue disorder characterized by inflammation and thickening of the fascia.
- It presents with painful swelling, typically in the limbs, and is associated with peripheral blood eosinophilia.
Observation:
- A 36-year-old male presented with progressive, firm, and painful swelling of his upper and lower limbs.
- Laboratory findings included elevated erythrocyte sedimentation rate, mild anemia, thrombocytosis, elevated gamma-globulin, and significant peripheral blood eosinophilia (44%).
- Histopathological examination of a lower leg biopsy revealed characteristic findings of eosinophilic fasciitis.
Findings:
- Diagnosis of eosinophilic fasciitis was confirmed by clinical presentation, laboratory results, and histological findings.
- Initial treatment with high-dose oral prednisone (100 mg/d) led to clinical symptom regression and resolution of eosinophilia.
- Gradual dose reduction of prednisone was necessary, with temporary increases required to manage persistent symptoms and prevent recurrence, indicating a relapsing-remitting course.
Implications:
- Early diagnosis and prompt treatment with corticosteroids are crucial for managing eosinophilic fasciitis.
- The case highlights the potential need for long-term, adjusted corticosteroid therapy to achieve sustained remission.
- This case contributes to understanding the clinical course and therapeutic challenges associated with Shulman syndrome.