A unique presentation of Pulmonary Langerhans Cell Histiocytosis

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a diffuse lung disease that primarily affects young adults, with cigarette smoking playing a significant role in developing the disease. Patients with PLCH present with characteristic CT chest findings of small irregular nodules and upper zone cysts. Previously, larger nodules greater than 10 mm and cavitation have only been reported a few times in the literature. We describe the case of a 69-year-old male who presented with dyspnea, non-productive cough and weight loss, who was found to have multiple cavitary nodules on CT imaging of the chest. Histopathologic sampling of the lung revealed Langerhans cells which stained positive for S100 and CD1a, consistent with a diagnosis of PLCH. The patient was counselled to quit smoking as the mainstay of treatment. In 3-month follow-up his symptoms had largely resolved, with evidence of decreased nodule size on repeat CT imaging.