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Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PL...
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... admitted to our hospital. Vital signs at presentation include: PR=120/min, RR=30/min, BP=110/80 mmHg and T=37 o C. The patient was a soldier with no underlying disease and physical examination revealed no significant finding except bilateral diminished breath sounds. Chest radiography showed bilateral pneumothorax and honeycombing of the lungs (Fig. 1). Laboratory tests were ...
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Background: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinica...
Citations
Pulmonary Langerhans cells histiocytosis (PLCH) is a rare disorder of unknown etiology. It usually presents as an isolated lung disease in adults; however, involvement of other organ systems can occur occasionally. In the presence of characteristic findings, high-resolution computed tomography scan may be sufficient for a confident diagnosis of PLCH in an adult smoker. Pneumothorax is the most commonly reported pleural complication of PLCH, and pleural effusion is extremely rare. Herein, we present a case of advanced PLCH in an adult smoker presenting with concomitant exudative pleural effusion.
Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.
