Introduction: Histiocytoses represent a heterogeneous group of diseases characterized by abnormal proliferation of histiocytes. This rare condition primarily affects young smokers, most commonly in the age range of 20 to 40 years, and may present as either a localized or multiorgan form.
Aim: The aim of this article is to present a rare clinical case of a patient with Langerhans cell histiocytosis, predominantly involving the lungs and skin.
Materials and Methods: An 18-year-old patient presented with a clinical picture of progressing dyspnea lasting two weeks, bilateral chest pain, subcutaneous emphysema in the chest and right arm, and imaging findings of bilateral spontaneous pneumothoraces, necessitating emergency pleural drainage. The patient denied excessive tobacco smoking, environmental exposures, and accompanying illnesses. Computed tomography of the chest revealed extensive disseminated involvement of the lung parenchyma.
Results: The patient was sequentially drained on the left and right sides using Bülau drains. Due to persistent air leak and the inability of the lung to fully expand, active aspiration and the use of the Heimlich valve were implemented in outpatient settings. Video-assisted thoracoscopic surgery (VATS) on the right confirmed the diagnosis of Langerhans cell histiocytosis, and pleurodesis was performed through parietal pleural scarification.
Conclusion: Our experience with this rare condition demonstrates the challenges in managing recurrent spontaneous pneumothoraces and prolonged air leaks in Langerhans cell histiocytosis with significant bilateral lung involvement.
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