Pulmonary Langerhans cell histiocystosis

Case contributed by Dr Domenico Nicoletti

Presentation

Dyspnea and dry cough in a heavy smoker.

Patient Data

Age: 75 years
Gender: Male

 

Presence of reticular opacities, micronodules and cysts in the mid and upper zones with normal bases.
 

In both lungs, particularly in the mid-upper zones, there are numerous micro and centrilobular nodules, with sharp margins, partly cavitated, with symmetrical distribution together with thick-walled cysts ("cheerios" pattern). Among the nodules are appreciable reticular opacities. The bases are spared.

Bronchoscopy with histological findings

Case Discussion

Langerhans cell histiocytosis (also called histiocytosis X) usually affects young adults.  Almost all patients are smokers or former smokers.

Pulmonary manifestations may occur in an isolated form or be associated with the involvement of other organs or systems. The disease starts in a nodular form and if it progresses, it evolves into a cystic form.

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