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About EDS

Classifications:

Ehlers-Danlos Syndrome is a group of connective tissue disorders, all of which result from genetic defects in collagen production.  There are 13 types of collagen in our bodies – all affecting different parts and systems.   The types of Ehlers-Danlos Syndrome are broken down according to symptoms and also by the gene involved in causing the collagen deficiency.

This information is only a brief summary of the characteristics of the types; NOT a diagnostic tool.  It is VERY important to remember that many of these types can only be confirmed by genetic analysis and that many types share symptoms.  There is a lot of crossover of symptoms between types and even within one type there can be a wide range of expression of symptoms.

Classical Type

Formerly known as:  EDS Type I & II

Diagnostic Criteria:

Major                                                        

  • Skin hyperextensibility                  
  • Widened atrophic scars                      
  • Joint hypermobility                             
  • Family History

Minor 

  • Smooth, velvety skin
  • Molluscoid psuedotumors
  • Subcutaneous spheroids
  • Complications from joint hypermobility
  • Muscle hypotonia, delayed gross motor development
  • Easy Bruising
  • Manifestations of tissue hyperextensibility
  • Surgical complications (postoperative hernias)                   

                                 

Hypermobility Type

Formerly known as:  EDS Type III

Diagnostic Criteria

Major:

  • Joint hypermobility
  • Soft skin with normal or only slightly increased extensibility
  • Absence of fragility or other significant skin or soft tissue abnormalities

Minor:

  • Positive family history of EDS
  • Recurrent joint dislocations or subluxations
  • Chronic joint, limb, and/or back pain
  • Easy bruising
  • Functional bowel  disorders (functional gastritis, irritable bowel syndrome)
  • Neurally mediated hypotension or postural orthostsatic tachycardia
  • High, narrow palate
  • Dental crowding

Vascular Type

Formerly known as:   EDS Type IV

Diagnostic Criteria

Major:

  • Arterial rupture
  • Intestinal rupture
  • Uterine rupture during pregnancy
  • Family history of the vascular type of EDS

Minor:

  • Thin, translucent skin (especially notable on the chest/abdomen)
  • Easy bruising
  • Characteristic facial appearance (thin lips and philthrum, small chin, thin nose, large eyes)
  • Acrogeria (an aged appearance to the extremities, particularly the hands)
  • Hypermobility of small joints
  • Tendon/muscle rupture
  • Early-onset varicose veins
  • Arteriovenous carotid-cavernous sinus fistula
  • Pneumothorax/pneumohemothorax
  • Chronic joint subluxations/dislocations
  • Congenital dislocation of the hips
  • Clubfoot
  • Gingival recession

Kyphoscoliotic Type

Formerly known as: EDS Type VI

(Information on the Kyphoscoliotic Type of EDS will be posted soon)

Less Common Types:

There are three more official types of Ehlers-Danlos Syndrome, Arthrocalasia, Dermatosparaxis, and Tenascin-X Deficient type.  I will have more specific information on these less common  types available soon. 

For more information on all types of Ehlers-Danlos Syndrome, please visit Ehlers-Danlos Network C.A.R.E.S., Inc by clicking the image below