Pulmonary Langerhans cell histiocytosis and multiple system involvement: A case report

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is a relatively rare type of lung disease, common in middle-aged smoking men. It is characterized by proliferation and infiltration of Langerhans cells, and the formation of multiple parabronchial mesenchymal nodules in lung tissue, and may lead to organ dysfunction. There are no typical symptoms and signs, and it is easily misdiagnosed or missed, and therefore deserves clinical attention and further discussion. CASE SUMMARY: We describe the case of a nonsmoking 46-year-old man with PLCH diagnosed based on clinical manifestations of fever and dry cough, with a history of hypothyroidism and diabetes insipidus for 9 years. Computed tomography (CT)- and CT-guided puncture examinations revealed no abnormalities, and he ultimately underwent thoracoscopic biopsy to confirm the diagnosis. The pathological diagnosis was PLCH. Thyroid function was maintained by medication. Pituitary magnetic resonance imaging showed that the pituitary stalk had become thinner. CONCLUSION: LCH often involves multiple systems. Moreover, the pathogenesis is not clear, clinical manifestations lack specificity, and diagnosis requires special attention. Diagnosis of PLCH can significantly benefit from comprehensive multidisciplinary analysis.