Palmar pigmentation: An unusual presentation of alkaptonuria

Alkaptonuria (AKU) is a rare inherited autosomal recessive metabolic disorder, caused by the deficiency of homogentisic acid oxidase enzyme. Herein, we are reporting a case of AKU with bilaterally symmetrical palmar pigmentation and skeletal involvement. Histopathological examination revealed ochre colored pigment in the dermis whereas roentgenogram of the lumbar spine showed typical calcification of the intervertebral discs.