Clyde B. Cope, M.D.; Paul Kassander, M.D.
JAMA. 1952;150(10):997-999. doi:10.1001/jama.1952.03680100039012.
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Ochronotic arthritis is a rare metabolic condition clinically characterized by a diagnostic triad1: (1) blue-black cartilages, especially of the ears and nose, brownish discoloration of the scleras, and gray-blue pigmentation of the skin; (2) urine that turns dark brown or black on standing or alkalinization; and (3) arthritis. There have been about 50 cases reported in the literature. Virchow,2 in 1866, described as postmortem findings the first case of ochronotic pigmentation and deforming arthritis. In 1904, Osler3 reported the first living patient with pigmented ears and scleras, rheumatic pains, Heberden's nodes, and nonglucose reducing substances in the urine. Because of the complexity and rarity of this clinical entity, none of the summaries in the literature can be considered complete or final. The most satisfactory reviews have been written by Oppenheimer and Kline4 and Smith.5

Hench6 has aptly stated that ochronotic arthritis resembles rheumatoid arthritis


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