Blue sclera and ligamentous hyperlaxity - Connective tissue disease?

Abstract

Introduction: the bluish coloration of sclera, frequently, can be associated with multiple systemic syndromes like the ligamentous hyperlaxity, which is a connective tissue disease currentely affecting population, joints, tendons and also some organs, all due to tegumentary weaknesses. Objective: to describe all epidemiologic and clinical characteristics of joint hypermobility syndrome associated with blue sclera in pediatric patients. Method: a review of the literature was conducted on published articles from May 2002 up to August 2020. Databases checked were Scopus, Wiley Online Library, SciELO, Dialnet, EBSCO, PubMedMedline. Keywords used were: “blue scleras”, “joints”, “joint hypermobility syndrome”, “connective tissue”, all in spanish and english language. A total of 21 bibliographic references were selected. Results: the uvea is a highly pigmented tissue, and when this pigmented layer increase or when the sclera decrease, allows the uvea to show through the sclera, giving rise to blue sclera. If extraocular manifestations are obvious, so blue sclera becomes an additional data supporting the diagnosis of joint hypermobility syndrome, may even be the only guide to an accurate diagnosis. Conclusions: it becomes necessary, from an ophthalmologic point of view, consider blue sclera a possible sign of onset acute diseases, cause of severe visual loss and systemic syndromes like joint hypermobility syndrome.