Pulmonary Langerhans cell histiocytosis - a rare type of a rare disease

Introduction: Langerhans cell histiocytosis is a rare disease (0.4 in 100,000) with a still highly debated aetiology - either a reactive or a neoplastic process. It is most commonly divided into single-system and multisystem disease. The pulmonary form is part of the former group and occurs mostly in adolescent and adult smokers (2nd through 4th decade of life). Aim:  The aim of this report is to provide more clinical data of one rarely documented disorder. Materials and Methods: We present a 42-year-old male patient with a respiration-associated pain of unknown origin. His physical examination was unremarkable. A CT scan was indicated, revealing multiple thin-walled cystic lesions and multiple small nodules in the pulmonary parenchyma, predominantly in the middle and upper lung segments, which was suggestive of pulmonary Langerhans cell histiocytosis. Through a video assisted thoracoscopy, several of the nodules were resected and sent for biopsy. Results: The patient was discharged on the day after the operation. The biopsy confirmed the diagnosis of Langerhans cell histiocytosis. Since systemic corticosteroids were contraindicated in this particular case, the patient was counseled to cease smoking. On the follow-up CT a tendency for a reversal of the condition was observed. Conclusion: The pulmonary form of Langerhans cell histiocytosis is a rare disease, most commonly seen in relatively young smokers with non-specific presentation. The biopsy is the only definitive diagnostic procedure. The disease may resolve spontaneously or after smoking cessation. In more severe cases corticosteroids, chemotherapy or lung transplantation may be considered.