Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode I'm going

to be talking to you about Erla's Danlos syndrome. And you can find written notes on this topic at

zero definals.com slash EDS or in the rheumatology section of the zero definals pediatrics book.

So let's get straight into it.

Erla's Danlos syndrome, or EDS, is an umbrella term that encompasses a group of genetic conditions

that cause defects in collagen, resulting in hypermobility of the patient's joints,

and abnormalities in connective tissue, such as the skin,

bones, blood vessels and organs. There are several types of Earlers Danlos syndrome.

This section mostly focuses on hypermobile Earls Danlos syndrome.

So let's discuss the different types.

Hypermobile Earls-Danloss syndrome represents most cases of EDS in clinical practice and your exams.

It's the most common and the least severe type of earless-dan loss syndrome.

The key feature is joint hypermobility, but patients also have soft and stretchy skin.

The gene for hypermobile Erlos-Danis-Danlos syndrome has not been identified and there is no single mode of inheritance.

Classical Erlos-Danloid syndrome features remarkably stretchy skin that feels smooth and velvety to touch.

They also have severe joint hypermobility, joint pain and abnormal wound healing.

Notably, they can develop lumps over pressure points such as their elbows.

They're prone to hernius, prolapses, mitralreguditation and aortic root dilatation.

Inheritance is autosomal dominant.

Vascular Eurlost-Anloss syndrome is the most dangerous form of Erlostanloss syndrome.

The blood vessels are particularly fragile as a result of defective collagen.

Patients have characteristic thin, translucent skin that you can almost see through.

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