Thursday, May 16, 2013

Clinical Presentation of Various Types of EDS

We already discuss that earlier experts divided Ehlers-Danlos Syndrome (EDS) into 11 subtypes — I through XI. To simplify these classifications scientists recognized the subtypes according to signs and symptoms and arranged them into six different types:

Hypermobility type (formerly type III)
This is the most common form of EDS. It may affect as many as one in 10,000 - 15,000 people. Signs and symptoms include:
E Loose, unstable joints
E Chronic joint pain

Classical type (formerly types I and II)
This type probably affects less than 1 in 20,000 - 40,000 people. Signs and symptoms include:
E Highly elastic, velvety skin
E Fragile skin that bruises/tears easily
E Slow and poor wound healing leading to scarring
E Noncancerous fibrous growths on pressure areas, such as elbows and knees; fatty growths on the shins and forearms
E Loose joints prone to dislocation and may delay the development of large-motor skills

 Vascular type (formerly type IV)
This is one of the most serious forms of EDS. It affects an estimated 1 in 100,000 - 200,000 people. Signs and symptoms include:
E Fragile blood vessels and organs that are prone to rupture
E Thin, fragile skin that bruises easily
E Veins visible beneath the skin
E Distinctive facial features, including protruding eyes, thin nose and lips, sunken cheeks and small chin
E Loose joints usually limited to the fingers and toes
Kyphoscoliosis type (formerly type VI)
This is another uncommon form of EDS. Fewer than 60 cases have been reported worldwide. Signs and symptoms include:
E Progressive curvature of the spine (scoliosis)
E Fragile eyes that are easily damaged
E Severe, progressive muscle weakness

Arthrochalasia type (formerly known types VII A and B)
Arthrochalasia is also very rare. Worldwide only about 30 cases have been reported with the followings signs and symptoms:
E Very loose joints and dislocations involving both hips, which may delay the development of large-motor skills
E Stretchy, fragile skin prone to bruising
E Early-onset arthritis
E Increased risk of bone loss and fracture

Dermatosparaxis type (formerly called type VIIC)
This form is also very rare. Only about 10 cases have been reported worldwide. Signs and symptoms include:
E Extremely fragile and sagging skin
E Loose joints, which may delay development of large-motor skills in children. 

Other types

The remaining subtypes ~ types V, VIII, IX, X, and XI ~ are classified as “other”. These are rare, and some are not well-defined. Some characteristics of these types:

E Type V has characteristically fragile skin, although bruising and loose joints are uncommon. 

E Type VIII involves teeth and gums as well as skin and joints (periodontal form).

E Type IX may cause chronic diarrhea and light headedness due to low blood pressure in addition to other signs and symptoms.

E Types X and XI are characterized by loose joints and are very similar and have sometimes been considered one type rather than two.

E Type X may lead to problems with blood clotting. 

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