[Pulmonary lymphangiomyomatosis].

The case of a woman of 48 years of age suffering from pulmonary lymphangiomyomatosis is presented. Chest radiographs showed the reticular infiltrate of diffuse interstitial lung disease, whereas high resolution CT illustrated multiple, well-defined cystic lesions distributed diffusely through the lungs without fibrotic changes or nodular densities. The patient underwent open lung biopsy and pleurodesis because of recurrent pneumothorax. Treatment with medroxyprogesterone was initiated.