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Updated: May 22, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Churg-Strauss syndrome: a disabling disease].
M C André1, A Fraga, M Duarte Reis
1Clínica Universitária de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal.
Acta Medica Portuguesa
|April 25, 2012
Summary
Churg-Strauss syndrome (CSS), a rare vasculitis, can present with unusual skin symptoms. Early diagnosis and multidisciplinary care are crucial for managing this condition effectively.
Area of Science:
- Rheumatology
- Dermatology
- Neurology
Background:
- Churg-Strauss syndrome (CSS) is a rare small-to-medium vessel vasculitis.
- It is characterized by asthma, eosinophilia, and systemic vasculitis.
Observation:
- A 51-year-old female presented with bullae, pain, paresthesias, and foot insensitivity.
- Rapid deterioration included gait loss and peripheral eosinophilia.
- Histopathology revealed extravascular eosinophils; bone marrow showed increased eosinophils without neoplastic infiltration.
Findings:
- Electromyography indicated mononeuritis multiplex affecting sciatic and femoral nerves.
- The patient met American College of Rheumatology and Chapel Hill Consensus criteria for CSS.
- Treatment with corticosteroids, cyclophosphamide, and rehabilitation yielded positive clinical and laboratory results.
Implications:
- Highlights the importance of recognizing atypical cutaneous manifestations for early CSS diagnosis.
- Emphasizes the critical role of a multidisciplinary team in managing this multisystemic disease.
- Suggests prompt intervention can lead to favorable patient outcomes.
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