Diffuse pulmonary lymphangiomatosis in a 26-year-old man

Abstract

Diffuse pulmonary lymphangiomatosis is a very uncommon disease, and is caused by abnormal development and proliferation of the lymphatic system. It is seen predominantly in children, exceptionally in adults, and affects both sexes equally. The diagnosis is usually made by biopsy. We report a case of a 26-year-old patient who presented with a 12 month history of progressive chronic cough and dyspnea. His chest CT image mimicked interstitial lung disease. Histopathologic examination showed marked thickening of the pleura with numerous dilated irregular thin-walled lymphatic vessels. The diagnosis was confirmed to be diffuse pulmonary lymphangiomatosis by lung tissue biopsy.