Multisystem Langerhans Cell Histiocytosis (LCH)
Imaging findings
A chest CT demonstrates diffuse, irregularly shaped cysts, reticular opacities, and architectural distortion, with a marked upper and mid-lung zone predominance and sparing of the lung bases. Bone windows reveal destructive lytic and sclerotic lesions in the temporal/mastoid bones and the spine.
Key takeaways
Langerhans cell histiocytosis (LCH) can present as a multisystem disease in adults. While isolated pulmonary LCH is strongly associated with cigarette smoking and often regresses with smoking cessation, the presence of extra-pulmonary bone lesions (such as temporal, mastoid, or spinal involvement) indicates a clonal neoplastic process that may occur independently of smoking or represent the non-smoking multi-system variant of LCH.
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