Pursuit of Osler's sign

A 58-year-old male came to the ophthalmology outpatient department with complaints of blackish discoloration in both eyes, which he noticed 6 months back. He had a history of bilateral knee pain and stiffness for 6 months, for which he was admitted in the orthopedics ward and planned for total knee replacement, since he was diagnosed to have bilateral OA knee. On examination, his BCVA was 6/9 in both eyes. In the interpalpebral area, there was a grayish conjunctival and scleral pigmentation seen in both the eyes in the perilimbal area at the lateral rectus insertion, which showed typical Osler's sign suggestive of alkaptonuria. Cornea was clear with a quiet anterior chamber. The pupil was round and reacting to both direct and indirect light reflexes, and there were early cataractous changes in the lens. Fundus examination with an indirect ophthalmoscope showed normal optic disc and vessels in both eyes. This is a classic case of alkaptonuria having typical signs. Many cases of alkaptonuria were diagnosed only after the development of symptoms. Scleral pigmentation usually starts during the mid-twenties; hence, being aware of this, ocular sign in alkaptonuria can lead to an early diagnosis and better outcome.