Pulmonary Langerhans cell histiocytosis

Case contributed by Dr Yen-Chung Liu

Presentation

Rushed into the ED with sudden dyspnea and left sided chest pain.

Patient Data

Age: 25 years old
Gender: Male

Bilateral coarse reticular interstitial pattern with a large left pneumothorax and mediastinal shift to the right.

Computed tomography scan of the chest showed numerous thin-walled pulmonary cysts of varying sizes and shapes, some of which are confluent and result in large cystic lesions. There is more middle and upper lobe predilection. 

Case Discussion

Diagnosis was confirmed by bronchoscopic biopsy. 

Langerhans’ cell histiocytosis (LCH) is a group of disorders characterised by infiltration of multiple systems by Langerhans’ cell. The exact pathophysiology of this idiopathic disorder is unknown. Reticulo-micronodular infiltration is the commonest pattern observed by CXR, although the abnormalities vary with the stage of the disease 1.

CT, especially the HRCT plays an important role in the diagnosis of of PLCH . The typical HRCT pattern of PLCH is thick- and thin-walled cysts combination with small poorly limited nodules, cavitated nodules, which affect both the peripheral and the central parts of the lung fields 1

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Case information

rID: 35387
Case created: 4th Apr 2015
Last edited: 4th Sep 2017
System: Chest
Inclusion in quiz mode: Included

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