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.