CTICasesLangerhans Cell Histiocytosis
Imaging findings
High-resolution chest CT in a young female smoker demonstrates multiple, bilateral upper-lung-predominant nodules and thin-walled cysts of bizarre, irregular shapes, with relative sparing of the lung bases. Histopathology shows cd1a-positive Langerhans cells and eosinophils forming nodules and stellate scars.
Key takeaways
Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by the proliferation of Langerhans cells in the distal bronchioles, forming granulomatous nodules that cavitate to form thin-walled, irregular cysts. The classic distribution is upper and middle lung zone predominance with sparing of the costophrenic angles. cd1a immunohistochemical staining is diagnostic.
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