Pulmonary tuberous sclerosis treated with tamoxifen and progesterone.

Tuberous sclerosis is a rare disease and pulmonary manifestations are extremely rare. Like pulmonary lymphangioleiomyomatosis, tuberous sclerosis of the lung affects only women of childbearing age and carries a very poor prognosis. The radiological and pathological features of the two conditions in the lung are indistinguishable and some authors therefore consider lymphangioleiomyomatosis to be a forme fruste of tuberous sclerosis,1 although others believe that the relationship is unclear.2