title: [Ehlers-Danlos syndrome--20 years experience with diagnosis and classification at the university skin clinic of Heidelberg].
abstract: BACKGROUND
  The Ehlers-Danlos syndrome encompasses a group of hereditary disorders of the connective tissue, characterized by hyperextensible skin, joint hypermobility; and varying degrees of vessel and tissue fragility. The main forms are classical, hypermobile, vascular, kyphoscoliotic A/B, arthrochalasis A/B and dermatosparaxis types.
  
  
  METHODS
  We report our experience in diagnosis and classification of Ehlers-Danlos-syndrome, especially with the combination of clinical and morphological criteria, at the Department of Dermatology of the University of Heidelberg with more than 600 patients between 1984 and 2004.
  
  
  RESULTS
  We classified those types of EDS which are characterized by regular and characteristic ultrastructural changes in the dermal components, primarily collagen, including the classical, hypermobile and vascular types as well as the less-common arthrochalasis and dermatosparaxis types. The combination of clinical and morphologic features facilitates the selection of candidate genes for molecular genetic investigation.
  
  
  CONCLUSIONS
  Besides the skin, skeleton and vessels, many other organ systems such as eyes and intestine, can be affected in Ehlers-Danlos syndrome. Accordingly, interdisciplinary cooperation (pediatrics, surgery, orthopedics, rheumatology, neurology, genetics) is necessary. As the connective tissue of the skin is accessible for biopsy and diagnostic investigation, dermatologists should be trained in the diagnostic approach and classification of this syndrome.
authors.affiliation: Department of Dermatology, University of Heidelberg, Germany. Sylvia_Proske@med.uni-heidelberg.de
authors.forename: Sylvia
authors.initials: S
authors.lastname: Proske
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Hartschuh
authors.forename: Alexander
authors.initials: A
authors.lastname: Enk
authors.forename: Ingrid
authors.initials: I
authors.lastname: Hausser
mesh_terms: D000046:Academic Medical Centers / Q000706:statistics & numerical data; D003880:Dermatology / Q000706:statistics & numerical data; D004535:Ehlers-Danlos Syndrome / Q000145:classification* / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D005858:Germany; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D012189:Retrospective Studies