Hermansky-Pudlak Syndrome (HPS) is a rare, inheritable disorder characterized by the
classic triad of oculo-cutaneous albinism, platelet dysfunction, and ceroid deposition. An
associated complication is pulmonary fibrosis with progressive restrictive lung disease. This
report discusses the lung involvement often seen in this condition correlated with
radiography, computed tomography, high-resolution computed tomography, and the
underlying pathology, by means of two such afflicted siblings. The elder died of respiratory …