Pulmonary Langerhans cell histiocytosis

Case contributed by Dr Charlie Chia-Tsong Hsu


Panel beater who has smoked 30 cigarettes per day for the last 30 years. No respiratory symptoms.

Patient Data

Age: 45
Gender: Male

Diffuse bilateral nodular opacities and small cysts in the upper and mid zone. 

Red arrows point to lung cysts of varying morphology.

Yellow arrows point to peribronchiolar and centrilobular nodules.

Case Discussion

Early stage of pulmonary Langerhans cell histiocytosis is characteristically by small ground glass lung nodules with upper lobe predominance. Based on imaging appearance it is not possible to differentiate Langerhans cell histiocytosis from respiratory bronchiolitis (RB) or RB-ILB, the clinical entity. 

As the disease progresses irregular shaped lung cysts appears. The mixture of lung nodules and irregular shaped cysts is suggestive of Langerhans cell histiocytosis in a patient with smoking history.

In minority of patients the disease progresses. The late stage is characterized by severe emphysemas and is no different to other causes of emphysema. 

In summary the cystic/ nodular phase of Langerhan cell histiocytosis is pathognomonic  whereas early nodular disease overlap with RB and RBILD and late disease cannot be differentiated from various causes of severe emphysema. 

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Case information

rID: 21108
Published: 4th Jan 2013
Last edited: 14th Aug 2019
System: Chest
Inclusion in quiz mode: Included

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