Pulmonary Langerhans cell histiocytosis

Case contributed by Liz Silverstone


Cough and dyspnea in a smoker.

Patient Data

Age: 25 years
Gender: Male

Numerous upper zone predominant elongated cysts, which accompany pulmonary artery branches and spare the subpleural lung. The costophrenic and anterior angles of the lung are spared. There are a few small lung nodules.

MinIPs show the extent of bronchial destruction and sometimes shows a branching pattern radiating from the hilum.


Case Discussion

The findings are typical of Langerhans cell histiocytosis, a reactive or neoplastic proliferative disease which affects young to middle-aged adults. A history of smoking is typical and the upper zone distribution is characteristic of inhalational lung disease. As in this case, the disease stabilized and symptoms improved with cessation of smoking.

Inflammatory peribronchial nodules are more common in early disease. Subsequently, irregular elongated cystic bronchial dilatation develops due to progressive destruction of the bronchial walls.

The diagnosis was made in this case by correlating the clinical and imaging findings without resorting to lung biopsy.

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