Concomitant pulmonary and rib lesions in a 44-year-old male nonsmoker

A 44-year-old male nonsmoker presented to the respiratory department with progressive left chest pain for 20 days. He had previously been healthy with normal immunity. He had no cough, fever, breathlessness, or sputum production. Physical examination revealed local tenderness on the left 10th posterior rib. Chest computed tomography showed bilateral multiple pulmonary nodules, thin-walled and thick-walled cavities and left 10th rib lytic lesion (Fig. 1a). Whole-body bone scan showed a central defect with the peripheral uptake of the “donut”-like change sign on the left 10th rib (Fig. 1b). Laboratory tests and bronchoscopy examination revealed no abnormalities. Lung puncture biopsy was difficult due to the small pulmonary lesions. Percutaneous CT-guided rib core biopsy was performed.

What is the diagnosis?

Langerhans cell histiocytosis involving both lungs and single rib

In patients not receiving active immunosuppressive therapy, the most likely culprits for multiple pulmonary and rib lesions are primary lung cancer, chronic infectious or inactive granulomata, or even the underlying systematic disease itself []. Langerhans cell histiocytosis (LCH) may affect any organ such as bone, skin, lung, lymphoid organs, and so on. It is easy to be misdiagnosed due to its various clinic features and laboratory results. Pulmonary Langerhans' cell histiocytosis (PLCH) occurs predominantly in young smokers. The disease is characterized by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways []. Chest CT plays an outstanding role in diagnosis. Imaging manifestations of small intralobular nodules, cavitated nodules, and thin-walled or thick-walled cysts prompted suspicion of PLCH. Several invasive procedures including bronchoalveolar lavage, transbronchial biopsy and lung biopsy can be used to diagnose pulmonary lesions. The type of diagnostic modality should be individualized. Detection of CD-1a cells in bronchoalveolar lavage fluid and transbronchial biopsies are not sensitive. Studies have shown a statistically significant decline for the diagnostic accuracy of CT-guided lung biopsy for lesions 10 mm or smaller [].When the lung puncture biopsies were uncertain for diagnosis or difficult due to too small pulmonary lesions, CT-guided percutaneous core rib biopsies can be performed for patients with indications. Although LCH is not fatal in all cases, delayed diagnosis or treatment can result in serious impairment of organ function.

None.