CTICasesPulmonary Langerhans Cell Histiocytosis (PLCH)
Imaging findings
Chest CT shows advanced end-stage cystic lung disease with confluent, bizarrely shaped, and upper-lobe predominant cysts mimicking severe emphysema, with relative sparing of the extreme lung bases. Pathology from the explanted lung demonstrates smoke-associated pigment-laden macrophages and stellate, medusa-head fibroinflammatory scars characteristic of Langerhans cell histiocytosis.
Key takeaways
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a smoking-related interstitial lung disease that typically presents with a combination of nodules and bizarrely shaped cysts in the upper and mid lung zones. In end-stage disease, the cysts can become confluent, mimicking severe panlobular or centrilobular emphysema.
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