Abstract

Alkaptonuria is a disorder of tyrosine metabolism due to deficiency of homogentisic acid oxidase enzyme which results in accumulation of homogentisic acid[1] . Excess amounts of homogentisic acid gets oxidized and gets deposited in connective tissue leading to OCHRONOSIS. Characterstic features of ochronosis include blue black discoloration of connective tissues including sclera, cornea, articular cartilage, heart valves, auricular cartilage, tendons and ligaments. Alkaptonuric ochronosis particularly interesting because it can be detected based only on clinical signs and medical history. We herein present a patient with typical signs and symptoms such as darkening of urine, pigmentation of sclera, nails, ear cartilage and manifesting arthritis in the fourth decade

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