Pulmonary ceroidosis

Abstract

We describe a patient with pulmonary ceroid histiocytosis. Skin pigmentation, chest x-ray film and laboratory findings were normal. Only pulmonary function tests were abnormal (TLC = 63 percent, DLco = 52 percent). Based on these functional data, the patient was submitted to a lung biopsy by thoracotomy. Brown pigmented histiocytes were shown occupying alveolar spaces. Similar brown pigmented hepatocytes were seen in the liver biopsy.