Isolated Pulmonary Langerhans Cell Histiocytosis

Isolated pulmonary involvement in Langerhans Cell Histiocytosis (LCH) is rare in childhood. The authors report a 6-y- old boy presenting with recurrent pneumothorax, whose CT thorax showed diffuse pulmonary cystic lucencies bilaterally. Biopsy of the lesions confirmed pulmonary LCH with Cd1a and S 100 positivity.