Adult lipid storage disorders with pulmonary involvement are rare and
usually diagnosed at autopsy. We report a patient with splenomegaly an
d reticulonodular pattern on lung computed tomography. Bronchoalveolar
lavage was performed and revealed the presence of lipid-containing fo
amy cells, with the demonstration of both periodic acid-Schiff (PAS) a
nd scharlach red stain positive vacuoles in the cytoplasm of alveolar
macrophages. The same cells were found in bone marrow biopsy. As in ot
her rare disorders, bronchoalveolar lavage may be of diagnostic value
in lipid storage disorders with pulmonary involvement.