Ehlers-Danlos Syndromes (EDS)

The Ehlers-Danlos syndromes (EDS) are a group of thirteen individual genetic conditions, all of which affect the body’s connective tissue.

Connective tissue lies between other tissues and organs, keeping these separate whilst connecting them, holding everything in place and providing support, like the mortar between bricks.

 

In EDS, a gene mutation causes a certain kind of connective tissue – the kind will depend on the type of EDS but usually a form of collagen – to be fragile and stretchy. This stretchiness can sometimes be seen in the skin of someone with EDS; individuals with the condition may also be able to extend their joints further than is usual – this is known as being hypermobile, bendy or double-jointed.

 

As collagen is present throughout the body, people with EDS tend to experience a broad range of symptoms, most of them less visible than the skin and joint differences.

 

These are complex syndromes affecting many systems of the body at once, despite this EDS is often an invisible disability. Symptoms commonly include, but are not limited to, long-term pain, chronic fatigue, dizziness, palpitations and digestive disorders. Such problems and their severity vary considerably from person to person, even in the same type of EDS and within the same family.

EDS Subtypes:

* Inheritance Pattern: AD = autosomal dominant; AR = autosomal recessive

The syndrome is named after two physicians, Edvard Ehlers from Denmark, and Henri-Alexandre Danlos from France, who identified it at the turn of the 20th century.

Edvard Laurits Ehlers (26 March 1863 Copenhagen – 7 May 1937) was a Danish dermatologist whose name was given to a group of genetic misfunctions of connective tissue, called Ehlers–Danlos syndrome (EDS).

 

Edvard Lauritz Ehlers grew up as the Mayor of Copenhagen's son and qualified in medicine in 1891. In the following years he went into further studies in Berlin, Breslau, Vienna and Paris. In Iceland he studied the decline of leprosy and was rewarded for his studies with a prize from the National Leprosy Fund in London. In 1906 he was appointed chief of the dermatological polyclinic at the Frederiks Hospital in Copenhagen. From 1911 to his retirement in 1932, Ehlers was director at the municipal hospital of Copenhagen.

Henri-Alexandre Danlos (March 26, 1844 – September 12, 1912) was a French physician and dermatologist born in Paris.

 

With Edvard Ehlers, the Ehlers–Danlos syndrome is named.

 

He studied medicine in Paris, and during the early part of his career performed research in the laboratory of Charles-Adolphe Wurtz (1817-1884). In 1881 he became médecin des hôpitaux, and four years later was chef de service at the Hospital Tenon in Paris. In 1895 he received an appointment at the Hospital Saint-Louis.

 

Danlos was pioneer in the use of radium for treatment of lupus erythematosus of the skin, and in 1901 with physicist Eugène Bloch (1878-1944), he was the first to apply radium on tuberculous skin lesions.

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