title: 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.
authors.affiliation: Department of Oral Pathology, Federal University of Rio Grande do Sul, Porto Alegre, Brazil. cristianobadauy@bol.com.br
authors.forename: Cristiano Macabu
authors.initials: CM
authors.lastname: Badauy
authors.forename: Sabrina S
authors.initials: SS
authors.lastname: Gomes
authors.forename: Manoel
authors.initials: M
authors.lastname: Sant'Ana Filho
authors.forename: José Artur Bogo
authors.initials: JA
authors.lastname: Chies
mesh_terms: D000328:Adult; D016301:Alveolar Bone Loss / Q000209:etiology; D003288:Contusions / Q000175:diagnosis; D004535:Ehlers-Danlos Syndrome / Q000145:classification*; D006406:Hematoma / Q000175:diagnosis; D006801:Humans; D008297:Male; D017622:Periodontal Attachment Loss / Q000209:etiology; D016388:Tooth Loss / Q000209:etiology; D014648:Varicose Veins / Q000175:diagnosis