Klin Monbl Augenheilkd 2020; 237(04): 417-418
DOI: 10.1055/a-1112-7292
Der interessante Fall
Georg Thieme Verlag KG Stuttgart · New York

Rare Hyperpigmentation of the Conjunctiva and Sclera: Ochronosis

Seltene Verfärbungen der Bindehaut und Sklera: Ochronose
Ioannis Lamprakis
1  Heuberger Augenklinik, Olten, Switzerland
Torsten Schlote
2  Ambimed Tagesklinik, Basel, Switzerland
› Author Affiliations
Further Information

Publication History

received 26 September 2019

accepted 20 January 2020

Publication Date:
24 April 2020 (online)


Alkaptonuria is a rare autosomal recessive disorder in which the metabolism of tyrosine is not properly functioning [1], [2] as a result of loss of homogentisate 1,2 dioxygenase (HGO) activity [3]. It has an estimated prevalence of 1 – 9/1.000.000 [4], [5], [6]. Literature suggests that the most common clinical finding is hyperpigmentation of the sclera [7], followed by conjunctival pigmentation and pigmented chamber angle, whereas recurrent anterior uveitis is a rare finding. Here, we present a case of a patient with this clinical condition.