Author(s): Abid Nawaz1, Vinesh Kumar2, Nauman Shaukat1, Aslam Khan3, Bilal Awan, Safia Shaikh4, Aqsa Iqbal
Hermansky-Pudlak Syndrome (HPS) is a rare autosomal recessive disorder that
presents with oculocutaneous albinism, bleeding disorders, and immunodeficien-
cy. Granulomatous colitis and pulmonary fibrosis are two major complications
of this syndrome. On rare instances, HPS can involve the heart and lungs. This
report discusses a 32-year-old man who presented with oculocutaneous albinism
and immunodeficiency along with renal, pulmonary, and pancreatic complica-
tions. Pancreatic atrophy is a unique finding in our patient, which has not been
reported in the literature. The purpose of our case report is to bring into light
unusual complications of HPS so that timely action could be taken to avoid the
progression of complications