A woman with bluish-coloured ears

A 60-year-old woman presented with typical features of alkaptonuria. C A S E R E P O R T A 60-year-old woman was referred by her general practitioner because of a bluish discoloration of both auricles, which had been present for some years (figure 1). A year earlier arthroscopy, performed because of pain in the right knee, showed dark-grey sediment on the cartilage but no further abnormalities. Except for the pain in the right knee, which was already decreasing, she had no symptoms of the skeletal system. The patient said that she had noticed that her urine showed a dark discoloration when the toilet had not been flushed for a number of hours. The family history revealed that two of her three brothers had also developed dark-coloured auricles. Besides the bluish discoloration of both auricles, physical examination revealed pigmentation of both sclerae (figure 2). No further discolorations of skin or cartilage were found. W H A T I S Y O U R D I A G N O S I S ? See page 212 for the answers to this photo quiz. A woman with bluish-coloured ears J. de Boer, G.W. van Dam, A.A.M. Franken Department of Internal Medicine, Academisch Ziekenhuis Groningen, PO Box 30001, 9700 RB Groningen, the Netherlands, e-mail: j.de.boer@int.azg.nl, Departments of Internal Medicine and Medical Photography, Isala Clinics, Location Sophia, Zwolle, the Netherlands * corresponding author P H O T O Q U I Z Figure 1 Ochronotic pigmentation of the conchae, anthelices and helices of the right and left ear, respectively Figure 2 Ochronotic pigmentation of the sclerae of the right and left eye, respectively