Ehlers-Danlos syndrome (EDS) type IV: review of the literature

Abstract

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of connective tissue heritable disorders. EDS type IV is a rare form that presents typical clinical signs, such as easy bruising and haematomas at sites of trauma, skin manifestations (translucent skin with visible veins), and joint hyperlaxity. To illustrate the dermatological features and describe an aggressive periodontitis, a symptom not yet reported in this EDS type, we present a case of a 23-year-old young man. This patient has been suffering from bruised skin, haematomas, and varicose veins in his legs. These lesions, typical of EDS type IV, were associated with trauma followed by slow and difficult cicatrization. Teeth loss and clinical attachment loss in all the remaining teeth, a symptom compatible with a severe destruction of the periodontal support, was reported after orthodontic treatment. The treatment is limited to control the disease and teeth loss. Considering this new clinical symptom associated with EDS type IV, we suggest that the use of orthodontic apparatus should be carefully considered in such patients.