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Langerhans Cell Histiocytosis and Emphysema

Imaging findings

High-resolution CT of the chest in a young female trauma patient demonstrates multiple bizarre, irregularly shaped, thin-walled cysts alongside centrilobular and paraseptal emphysema. The cysts are predominantly located in the upper and middle lung zones, with relative sparing of the extreme lung bases and costophrenic angles.

Key takeaways

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by the combination of centrilobular nodules and irregularly shaped, branching cysts. Although PLCH predominantly affects the upper and mid-lung zones, it can occasionally present with atypical lower-lobe or lingular cysts, and must be distinguished from lymphangioleiomyomatosis (LAM) and emphysema in young female smokers.

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