The differential for a diffuse cystic disease is remarkably short. Excluding emphysema which is not genuinely cystic the considerations are pneumatoceles (usually few/isolated, variable in size and larger), Langerhan’s Cell Histiocytosis (Histiocytosis X), lymphangioleiomyomatosis (LAM) (female and diffuse involvement, not sparing bases, pneumothorax or effusions often present ), tuberous sclerosis (equivalent to LAM), and neurofibromatosis (actually an exotically rare manifestation of this disease, non uniform, larger cysts).
The appearances are fairly typical for Langerhan’s with overall lung expansion, symmetrical disease and typical sparing of the bases. These patients are invariably smokers. At HRCT the same distribution is noted with sparing of the anterior medial lung in the bases also evident. Early in the disease small ill-defined nodules are also evident that progressively cavitate into thinner irregular and “bizarrely” shaped cysts. The nodules are usually not visible on CXR. Later in the disease, when the nodules have all resolved and the cysts become confluent, the appearances can be so uniform that it is difficult to differentiate from reticular however, lung volumes are preserved. While the disease is still at least partially nodular significant improvement can occur with smoking cessation. Bone disease is not common in the adult presentation of Langerhan’s as opposed to infant or childhood onset Langerhan’s disease.