Photodistributed Hereditary Ochronosis

To the Editor. —We describe an unusual distribution of skin pigmentation in a patient with hereditary ochronosis. Report of a Case. —A 48-year-old man was evaluated in our Department in February 1991 because of a 10-year history of blueblack hyperpigmentation on his face, ears, neck, and forearms. The Patient had previously undergone a work up for arthritic pain that primarily affected his back, shoulders, pelvis, and knees. The skin pigmentation started on his face and hands and progressed to the neck and forearms. He admitted to having dark brown urine and stained undergarments for as long as he could remember. He denied use of antimalarials or phenolic intermediates such as hydroquinone, carbolic acid, picric acid, and resorcinol. The findings from his physical examination revealed a sooty bluish-black hyperpigmentation on the face, especially the nose and malar eminences; the ears; the V-line of the neck; and the dorsa of the hands