EHLERS DANLOS SYNDROME (EDS) or CUTIS HYPER ELASTICA DIFFERENT FORMS OF EDS

Classic EDS

• Type I EDS (Severe) and Type II EDS (Mild)

Clinical Manifestations-

• Hyperelasticity of skin (Rubber Person Syndrome)
• Hypermobile joints
• Affect Type V Collagen and Type I Collagen.
• arachnodactyly

 Hypermobile EDS (Type III EDS)

• Joint hypermobility

 Vascular EDS (Type IV EDS)- Most serious

• Type III collagen is affected

 Type V EDS

• Inherited as an X-linked trait

 EDS with Tenascin X defect seen in-

Type III EDS

EDS due to enzyme defect-

• Arthrochalasic EDS (type VII EDS) or Dermatosparaxis
• Kyphoscoliotic

According to Villefranche Classification, EDS –

SUBTYPE	DEFECT IN
Hypermobility	Type III collagen, Tenascin X
Classic	Type I and V collagen
Vascular	Type III collagen
Kyphoscoliosis	Lysylhydroxylase
Arthochalasis	Type I collagen
Dematosparaxis	ADAM metallopeptidase with thrombospondin type I motif

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