Alkaptonuric ochronosis presenting as palmoplantar pigmentation

We describe a 37-year-old woman who presented with palmoplantar pigmentatio n, thickening and pitting of 4 years duration. Bluish pigmented patches wer e seen over the sclera of her eyes. Her lumbar spine showed typical calcifi cation of the intervertebral discs. Addition of Benedict's reagent to a uri ne sample of the patient gave rise to greenish brown precipitate and browni sh black supernatant. Alkalinization of urine turned it black. A biopsy of the palmar lesion demonstrated irregular breaking up, swelling and homogeni zation of collagen bundles in the reticular dermis. Yellow-brown (ochre col oured) pigment was seen lying within the collagen bundles and also freely i n the deeper dermis confirming our clinical diagnosis of alkaptonuric ochro nosis. To the best of our knowledge this is probably the second report of a lkaptonuria presenting with palmoplantar pigmentation.