A case of ochronosis with gout and Monckeberg arteries.

Alkaptonuria is an inborn error of amino acid metabolism. A defect in tyrosine metabolism that results in accumulation of homogentisic acid in connective tissue, especially cartilage, has long been known. Degenerative arthropathy, especially of the knee and spine, develops at a relatively early age in adults. Accumulation also occurs in heart valves, and there may be a predisposition to atherosclerosis. We describe a 72-year-old man with ochronosis, gouty arthritis of both hands, and monckeberg arteries.