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04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Erdheim-Chester disease presenting with pneumothorax.
Masafumi Yamaguchi1, Tetsuhiro Shiota, Yoichiro Kobashi
1Division of Respiratory Medicine, Ako City Hospital, Ako, Japan. myama3265 @ gmail.com
Respiration; International Review of Thoracic Diseases
|September 22, 2011
Summary
Erdheim-Chester disease (ECD) can cause acute lung issues. This case highlights a rare presentation of ECD with a ruptured lung cyst causing pneumothorax, emphasizing the need for prompt diagnosis.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
- Pulmonary involvement in ECD often leads to chronic symptoms and non-specific radiographic findings.
Observation:
- A 50-year-old man presented with acute chest pain and dyspnea due to a ruptured lung cyst and pneumothorax.
- Radiography revealed pneumothorax, a large cystic lesion, and interstitial markings.
Findings:
- Surgical biopsy confirmed histiocytic infiltrates positive for CD68 and negative for S-100 and CD1a.
- Systemic evaluation revealed widespread symmetrical skeletal involvement, confirming the ECD diagnosis.
Implications:
- This case underscores the diverse and potentially acute pulmonary manifestations of ECD.
- Early recognition of atypical presentations like pneumothorax is crucial for timely ECD diagnosis and management.
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