Ehlers-Danlos Syndromeis

What Is Ehlers-Danlos Syndrome? :

Ehlers-Danlos Syndromeis a group of inherited connective tissue disorders, caused by faulty collagen (a protein in connective tissue).

 

Connective tissue helps support the skin, muscles, ligaments and organs of the body. People who have the defect in their connective tissue associated with Ehlers-Danlos Syndrome may have symptoms which include joint hypermobility, skin which is easily stretched and bruised, and fragile tissues. There are six major classifications of Ehlers Danlos Syndrome.

What Are The Six Types of Ehlers- Danlos Syndrome?:

Ehlers-Danlos Syndrome has been classified into six types:

 

  • Hypermobility
  • Classical
  • Vascular
  • Kyphoscoliosis
  • Arthrochalasia
  • Dermatosparaxis

 

Hypermobility Type of Ehlers-Danlos:

The primary symptom associated with the Hypermobility Type of Ehlers-Danlos Syndrome is generalized joint hypermobility which affects large and small joints. Joint subluxations and dislocations are a commonly recurring problem. Skin involvement (stretchiness, fragility, and bruising) is present but to varying degrees of severity, according to the Ehlers-Danlos Foundation. Musculoskeletal pain is present and can be debilitating.

Classical Type of Ehlers-Danlos :

The primary symptom associated with the Classical Type of Ehlers-Danlos Syndrome is distinctive hyperextensibility (stretchiness) of the skin along with scars, calcified hematomas, and fat-containing cysts commonly found over pressure points. Joint hypermobility is also a clinical manifestation of the Classical Type.

Vascular Type of Ehlers-Danlos:

The Vascular Type of Ehlers-Danlos Syndrome is considered the most serious or severe form of Ehlers-Danlos Syndrome. Arterial or organ rupture can occur which can lead to sudden death. Skin is extremely thin (veins can be seen easily through the skin) and there are distinctive facial characteristics (large eyes, thin nose, lobeless ears, short stature and thin scalp hair). Clubfoot may be present at birth. Joint hypermobility usually only involves the digits
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