Hand deformity in a patient with mucopolysaccharidosis type I (MPS I, Hurler-Scheie syndrome)

The hand of a 17-year-old male with Hurler-Scheie syndrome, showing severe contractures and deformation of the distal interphalangeal joints. Movement of the hand and fingers is limited. Thenar atrophy seen at the base of the thumb is due to severe carpal tunnel syndrome.