CTICasesParaneoplastic Pemphigus with Castleman's Disease and Constrictive Bronchiolitis
Imaging findings
A very large, intensely enhancing mediastinal mass was identified, consistent with unicentric Castleman's disease (hyaline vascular type). Initial chest imaging was normal. Subsequent chest radiograph showed hyperinflated lung volumes, and follow-up CT revealed severe hyperinflation, diffuse bronchial wall thickening, bronchial dilatation (primarily proximal airways), and subtle mosaic attenuation indicative of diffuse air trapping, consistent with severe constrictive bronchiolitis. Later imaging also showed extensive bronchocentric opacities and bronchial disease due to a specific infection and treatment-induced hypogammaglobulinemia.
Key takeaways
Paraneoplastic pemphigus (PnP) is a severe autoimmune mucocutaneous disease. Tumors associated with PnP include non-Hodgkin lymphoma and Castleman's disease. PnP can cause significant lung involvement, including bronchiolitis obliterans, where autoantibodies target columnar epithelium in the bronchioles. This case demonstrates the dramatic and progressive obstructive airways disease associated with PnP, which can lead to severe lung dysfunction and potentially cystic changes.
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