Ehlers-Danlos syndrome: more complicated than imagined

• Ehlers-Danlos syndrome was described by 2 physicians together:
  • the French dermatologist Dr Henri-Alexandre Danlos (1844-1912). He was at the Hôpital St-Louis in Paris and was a pioneer in the use of radium in systemic lupus erythematosus and skin lesions of tuberculosis.
  • the Danish dermatologist Dr Edvard Ehlers (1862-1937). He trained in Breslau, Berlin, Vienna and Paris and was perfectly fluent in French. That is why he described the condition at the turn of the century with Dr Danlos. Dr Ehlers was more known during his life for his work on leprosy.
• Ehlers-Danlos syndrome (types I and II) in its classical form is autosomal dominant and characterized by hyperextensive skin, easy subluxations and joint dislocations.  This form is due to mutations on genes COL5A1, COL5A2, COL1A1.
• More than what was described there were 10 known forms of Ehlers-Danlos syndrome. Since 1997 a new classification of the syndrome delineates them according to observations:
  • Hypermobility (type III)
  • Classical (types I and II)
  • Vascular (type IV)
  • Kyphoscoliosis (type VI)
  • Arthrochlasia (type VIIA and VIIB)
  • Dermatosparachsis (type VIIC)

Contributors

Dr Christophe Hsu – dermatologist. Geneva, Switzerland

Source of information:

-Crissey JT el al. Dermatology and Dermatologists (2002). Parthenon Publishing

-Wikipedia