Ehlers-Danlos type VIII. Review of the literature

Abstract

Ehlers-Danlos type VIII is a rare disorder characterized by soft, hyperextensible skin, abnormal scarring, easy bruising, and generalized periodontitis with early loss of teeth. To illustrate the clinical dermatological and dental features, we present the case history of a 20-year-old patient who has suffered from poor healing of wounds at the shins and knees since childhood, which have developed into hyperpigmented atrophic scars. In the course of orthodontic treatment during the last 3 years, severe apical root resorption, gingival recession, and loss of alveolar bone were observed. Family history was noncontributory for any skin or tooth disorders. The typical clinical signs confirmed the diagnosis of Ehlers-Danlos syndrome type VIII. As there is no specific treatment for the disorder, management is limited to the symptomatic treatment of the dental disease. It seems advisable to consider carefully the indications for orthodontic treatment in patients with Ehlers-Danlos type VIII syndrome.