The life expectancy of a patient with Ehlers-Danlos syndrome (EDS) depends on the type of EDS and the patient's symptoms. EDS is a slowly progressive disease. EDS symptoms vary by type and range from mildly loose joints to life-threatening complications. There are 13 defined types of EDS. Apart from these 13 types, there are several other types identified as EDS. However, these are rare and aren’t well-defined.
The vascular type is typically a more severe form of EDS and is often associated with a short lifespan. Patients with vascular EDS have an average life expectancy of 48 years. About 80% of patients might have a critical event by the age of 40 years. The lifespan of a patient with kyphoscoliosis EDS is reduced due to two reasons:
- Involvement of the blood vessels
- Restrictive lung disease
Patients with classical and hypermobility types of EDS may have a longer lifespan. Patients with these forms of EDS live healthy but somewhat restricted lives.
What is Ehlers-Danlos syndrome?
Ehlers-Danlos syndrome (EDS) is an inherited group of conditions that affect the connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. The condition is caused by mutation of the gene, causing abnormalities in the structure, production, and/or processing of collagen (the protein present in the connective tissues).
What are the symptoms of EDS?
Joint hypermobility and extremely fragile skin are the shared features of most forms of Ehlers-Danlos syndrome (EDS). There might be features that may overlap in different types. Other symptoms of EDS vary with subtypes and are described below:
Hypermobile EDS: This type of EDS is characterized by
Classical EDS: This type of EDS is characterized by
- Stretchy, smooth, and silky skin with easy bruising.
- A flat, depressed scar.
- Tumors over the elbow.
- Delayed motor development.
- Hypotonia (decreased muscle tone).
Vascular EDS: This type of EDS is characterized by
- A fragile intestine and uterus that are prone to rupture.
- A short stature.
- Thin hair.
- Large eyes.
- A thin nose.
- Lobeless ears.
- Pneumothorax (collapse of the lung).
Kyphoscoliosis EDS: This type of EDS is characterized by
- Hypotonia at birth.
- Delayed motor development.
- Progressive scoliosis (sideways curvature of the spine from birth).
- Osteopenia (a low bone density).
Arthrochalasia EDS: This type of EDS is characterized by
- Hip dislocation (present from birth).
- Kyphoscoliosis (a combination of outward and sideways curvature of the spine).
- Dermatosparaxis EDS: Some of the features include:
- Saggy, redundant skin, especially on the face.
Classical-like EDS (clEDS): Features include:
- Spontaneous ecchymoses (bruises or discolorations of the skin resulting from the blood underneath)
- Spondylodysplastic EDS: Some of the features include:
- Short stature
- Muscle hypotonia
- Bowing of limbs
Cardiac-valvular EDS: This type of EDS can lead to
- Severe heart valve abnormalities.
- Skin problems.
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