Abstract

Pulmonary Langerhans’ cell histiocytosis (LCH) is an uncommon intertitial lung disease characterized by tissue infiltration with CD1a expressing Langerhans cells. It occurs more commonly in heavy smokers and young adults. We present two patients with this rare entity who were diagnosed in our institution. The first patient was a 19-year-old man, with a smoking history of 1.5 packs/year. He admitted to hospital with the symptoms of breathlessness, cough, bilaterally chest pain as the result of bilaterally spontaneous pneumothorax. The second patient was a 20-year-old man, with a smoking history of 5 packs/year. He admitted to hospital strong cough, and breathlessness with exercise. Computerized tomography of chest showed cystic lesions at mid and upper zones in the first patient, where diffuse cavitary nodules at all zones in the second patient. The disease was diagnosed by open lung biopsy. The protein S-100 and CD1a surface antigens were found positive by the immunohistochemical staining of the biopsy specimens of both patients.