The Ehlers-Danlos syndrome

Abstract

A case study of a nineteen year old female illustrates the main features of the Ehlers-Danlos syndrome. This is a rare syndrome, the characteristic features of which are hyper elasticity and fragility of the skin, hyperextensibility of joints and fragility of blood vessels. Musculo-skeletal, ocular and internal manifestations are present in some cases. The main feature of this case was severe bleeding from minor trauma. The Kyphoscoliosis was first noticed in childhood but had become worse in her teens and she had evidence of respiratory insufficiency secondary to the marked chest deformity. No treatment is known. Surgical procedures may present a problem as the tissues are friable and difficult to suture. Meticulous techniques and pressure dressing are desirable.