[Pulmonary lymphangiomyomatosis and tuberous sclerosis--similarities and differences].

Two cases pulmonary lymphangioleiomyomatosis and tuberous sclerosis with pulmonary involvement were presented. Clinical picture of both diseases was similar. Breathlessness was the main symptom of lymphangioleiomyomatosis and there were signs of chylothorax. Chest x-ray interstitial changes disseminated in both lungs. High resolution computer tomography showed numerous cysts in both lungs. Proliferation of smooth muscle bundles was found in lung biopsy. Main symptoms of tuberous sclerosis were breathlessness on exertion and recurrent pneumothorax. Changes in chest x-ray, CT and lung biopsy were similar to found in lymphangioleiomyomatosis. Diagnosis of tuberous sclerosis was based on typical extrapulmonary signs and symptoms.