Ochronosis: a case of severe ochronotic arthropathy

Ochronosis involves primarily the large cartilaginous joint surfaces, ribs, intervertebral discs, ear cartilage etc. We report on a 52-year-old woman with typical alkaptonuric ochronosis with dark urine, blue-black pigmentation of the auriculae and hands, spondylarthropathy and severe hip and shoulder joints involvement. The differential diagnosis of this rare condition is discussed with a review of the literature.