[The pathology and clinical features of generalized lymphangiomyomatosis (author's transl)].

Generalized lymphangiomyomatosis with diffuse pulmonary involvement was diagnosed at post-mortem of a 26-year-old woman. The disease occurs only in women, during or after their reproductive phase, with chylous effusion, especially chylothorax, dyspnoea and chronic progression. It is a multitopic condition starting with a focal lesion, which has its origin in the smooth musculature of the lymphatics and probably develops in dependence on hormonal factors.