Ehlers-Danlos syndrome type VIII with severe periodontitis and apical root resorption after orthodontic treatment.

نویسندگان

  • S Karrer
  • M Landthaler
  • G Schmalz
چکیده

Sir, Ehlers-Danlos syndrome (EDS) is a group of generalized disorders characterized by abnormalities of the connective tissue leading to fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility (1). There are many subtypes and variants of EDS; at least 10 types have been described on the basis of clinical symptoms and inheritance pattern. However, about half of patients with EDS do not ®t into 1 of the 10 types (2). Therefore, in 1997 a revision of the classi®cation of the Ehlers-Danlos syndromes was proposed, based primarily on the cause of each type (3). EDS type VIII was recognized by McKusick (4) in 1972 in a family with skin fragility, abnormal scarring, early tooth loss and severe periodontitis. Deformed roots and pulp calci®cations in EDS patients have been reported (5). Up to now the diagnosis of EDS type VIII has been based only on clinical criteria. Unlike for EDS types IV, VI and VII, no underlying biochemical defect has yet been detected in type VIII. We present the case of a young man with EDS type VIII, in order to draw attention to this rare condition and document its clinical dermatological and dental features.

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عنوان ژورنال:
  • Acta dermato-venereologica

دوره 80 1  شماره 

صفحات  -

تاریخ انتشار 2000