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Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion
Shoji Hirasaki1, Kazutoshi Murakami, Tatsuya Kanamori
1Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan. s.hirasaki@kousei-hp.or.jp
Internal Medicine (Tokyo, Japan)
|April 17, 2012
Summary
Weber-Christian disease (WCD), a rare panniculitis, can present with thoracic involvement like mediastinitis and pleural effusion. Early corticosteroid treatment effectively managed symptoms in a unique case study.
Area of Science:
- Internal Medicine
- Dermatology
- Pathology
Background:
- Mediastinitis and pleural effusion are typically associated with infectious or malignant processes.
- Weber-Christian disease (WCD), or nodular nonsuppurative panniculitis, is a rare lipo-inflammatory disorder.
- Thoracic involvement is an uncommon manifestation of Weber-Christian disease.
Observation:
- A 53-year-old male presented with high fever, mediastinitis, and left pleural effusion.
- Subcutaneous nodules on lower extremities were diagnosed as panniculitis via thoracoscopy-assisted excision.
- Histological findings confirmed panniculitis in the periaortic area.
Findings:
- Corticosteroid therapy rapidly resolved the patient's fever.
- Follow-up imaging demonstrated significant improvement in mediastinitis and pleural effusion.
- The case highlights the potential for WCD to mimic more common causes of thoracic inflammation.
Implications:
- Weber-Christian disease should be considered in the differential diagnosis of unexplained mediastinitis, even with prominent thoracic findings.
- This case expands the known clinical spectrum of Weber-Christian disease.
- Prompt diagnosis and management with corticosteroids can lead to favorable outcomes in WCD with thoracic involvement.
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