Intrathoracic lymphangiomatosis mimicking lymphangioleiomyomatosis in a young woman.

In this report, we describe a case of lymphangiomatosis that mimicked lymphangioleiomyomatosis in a 20-year-old woman. Lung biopsy specimens showed proliferation of anastomosing lymphatic channels in the visceral pleura and dilated peribronchiolar and septal lymphatic channels. During 8 years of follow-up, this process behaved like lymphangioleiomyomatosis with reticulonodular infiltrates and worsening obstructive and restrictive changes evident on pulmonary function tests.