What is Ehler-Danlos Syndrome?

Ehlers-Danlos syndrome, or EDS, is an illness which was first described at the beginning of the 20th century by the doctors Edvard Ehlers in Copenhagen and Alexandre Danlos in Paris. Initially described and characterized by a skin which was fragile, hemorrhagic and stretchable, and an excessive articular mobility, the EDS is a genetic disease which involves an anomaly of the connective tissue.

The Ehlers-Danlos syndromes  (EDS) form a heterogeneous group of hereditary illnesses of the connective tissue, characterized by an articular hyperlaxity, a cutaneous hyperelasticity, and a tissular fragility. There are numerous forms of the EDS, distributed usually in 6 classes, although in practice, three forms are mainly used: classic, hypermobile and vascular. In these three forms, the disease manifests itself by similar symptoms, but with different intensity. 

The classic form is mainly associated with appearances on the skin, the hypermobile shape is characterized by more intense articular appearances and the vascular shape involves essentially arterial, intestinal and uterine appearances. It is in this last group that the most serious forms of the disease can be found, with a risk of ruptured arteries and internal organs.

EDS can declare itself at different stages of the life of an individual, most of the time during the childhood or during puberty.

Various epidemiological data place the prevalence of the disease between 1/5000 and 1/10 000 births, although these figures are discussed by certain doctors who plead for a much bigger incidence of the disease and question its classification as a rare disease. Depending on the mutation type involved, transmission can be autosomal dominant or recessive dominant. In certain very rare cases, the transmission can be linked to the X.

Hormones could also be an important factor in the expression of the symptoms, which would explain the strong share of women with EDS, despite a transmission mode which is not linked to the sex chromosome.

What are the symptoms of Ehler-Danlos Syndrome?

Several symptoms are associated with EDS:

• Fatigue
• Chronic and diffused pains which are resistant to the usual analgesic treatments. These pains can be articular (the most frequent) and periarticular, muscular, abdominal, and migraines…
• Proprioceptive and movement control disorders Arthrochalasis
• Alterations of the skin (thinness, fragility, stretch marks, difficult healing)
• Numerous and spontaneous bruises and hemorrhage

Other symptoms are also often associated with EDS: digestive, respiratory, dental, ophthalmology, audition or cognitive problems.

One of the essential markers of the illness is that it is family run, with appearances and levels which can be different in the same family.

EDS, unlike degenerative illnesses, is characterized by a state of fragility, and not a progressive deterioration of the patient’s state.

Factors can improve or deteriorate the state of a patient: traumas aggravate the illness, feminine hormones play an important role (the illness can be worse during puberty and after giving birth, and sometimes improve during a pregnancy), and heat often improves symptoms.

How can Ehler-Danlos Syndrome be treated?

There are no cure for Ehler-Danlos Syndrome, yet the symptoms can often be taken care of.

Ligament or digestive surgery must be considered with great precautions. Patient care can be done through re-education, orthosis, compression clothing, and the use of certain molecules has improved the comfort and state of some patients.

The skin must be treated with consideration and protected from physical aggression, sun, heat, and cold. It must be hydrated effectively.

To that end, we recommend using Skintifique's Moisturizing Balm, which provides particularly efficient skin moisturizing and soothing, while displaying a very clean formulation that contains only 10 ingredients in total !