Ehlers-Danlos syndrome is a group of inherited disorders of collagen metabolism.
Types of Ehlers-Danlos syndrome and their main clinical features:
There are at least 10 subtypes with a variable expression of skin fragility, hyperelasticity of the skin and hypermobility of the joints.
The major features differentiating more common types of Ehlers-Danlos syndrome are listed.
- Type I Hyperextensible skin, hypermobile joints and papyraceous scars.
- Type II Hyperextensible skin, hypermobile joints and papyraceous scars, but more mild.
- Type III Hypermobile joints with minimal skin abnormality.
- Type IV Thin fragile skin and blood vessels, but normal joints.
- Type V Resembles type II, but ecchymoses more marked.
- Type VI Soft skin, keratoconus and intra-ocular hemorrhage.
- Type VII Extreme joint hypermobility often with congenital hip dislocation.
- Type VIII Hyperelastic skin and joints with periodontitis.
- Type IX Bladder diverticula, inguinal hernias, mild skin changes, skeletal defects.
- Type X Mild skin and joint changes, but prominent bruising.
(after J.L. Burton, 1992)
- There is no effective treatment.
- Fragile skin that has been torn may be approximated with tissue glue, as sutures hold only very poorly.
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