120 Pulmonary Langerhans’ Cell Histiocytosis

Clinical Presentation

38-year-old woman smoker with cough and dyspnea

Radiologic Findings

PA chest radiograph (Fig. 120.1) demonstrates normal lung volume without visible pulmonary abnormalities. HRCT (Figs. 120.2, 120.3) shows subtle bilateral upper lobe–predominant small pulmonary cysts with nodular irregular cyst walls and scattered small nodules with ill-defined borders (Fig. 120.2). Note the relative sparing of the lung bases (Fig. 120.3).

Diagnosis

Pulmonary Langerhans’ Cell Histiocytosis

Differential Diagnosis

• Infection

• Emphysema

• Bronchiectasis

• Lymphangioleiomyomatosis

Discussion

Background

Pulmonary Langerhans’ cell histiocytosis (PLCH) is a chronic, progressive interstitial lung disease that results from abnormal non-malignant proliferation of monoclonal Langerhans’ cells. Multiple organs may be affected, including bone, pituitary gland, mucous membranes, skin, lymph nodes, and liver.