Skin Features of EDS 

The Ehlers-Danlos syndromes (EDS) are a group of heritable connective tissue disorders. Connective tissue is found throughout the body and is an important component of the skin. Problems with connective tissue can cause changes in the skin.  

People with EDS may have skin characteristics such as: 

  • Skin hyperextensibility
  • Skin fragility 
  • Unusual skin texture 
  • Thin skin 
  • Delayed wound healing 
  • Abnormal scarring 

People with EDS may have skin characteristics such as: 

  • Skin hyperextensibility
  • Skin fragility 
  • Unusual skin texture 
  • Thin skin 
  • Delayed wound healing 
  • Abnormal scarring 

All types of EDS can cause changes in the skin, but not everyone with a type of EDS has skin features. One feature seen across all types of EDS is skin hyperextensibility, which means the skin can stretch beyond the normal range. Mild skin hyperextensibility may occur in people with any type of EDS. More severe skin hyperextensibility is observed in certain types of EDS. The type and severity of skin changes vary both by type and between individuals with the same type.

Skin Features of Each Type of EDS 

Type of EDS

Common skin features

  • Soft, mildly stretchy skin
  • Mild atrophic scarring
  • Stretch marks
  • Extensive skin involvement, usually apparent from a very young age
  • Very soft, stretchy, fragile skin
  • Severe atrophic scarring
  • Doughy skin texture 
  • Easy bruising 
  • Molluscoid pseudotumors 
  • Subcutaneous spheroids 
  • Easy bruising 
  • Thin, translucent skin with increased venous visibility 
  • Premature aging
  • Skin hyperextensibility 
  • Skin fragility 
  • Wide or atrophic scarring 
  • Easy bruising 
  • Premature aging 
  • Prominent vasculature 
  • Skin hyperextensibility 
  • Easy bruising 
  • Skin fragility 
  • Delayed wound healing 
  • Skin hyperextensibility 
  • Soft and doughy 
  • Thin and translucent skin 
  • Soft, velvety, and/or translucent skin 
  • Skin hyperextensibility 
  • Skin hyperextensibility 
  • Atrophic scars 
  • Easy bruising 
  • Skin hyperextensibility 
  • Easy bruising 
  • Skin fragility with atrophic scars 
  • Increased palmar wrinkling 
  • Skin hyperextensibility and velvety skin texture without atrophic scarring 
  • Easy bruising 
  • Extreme skin fragility with congenital or postnatal tears 
  • Progressively redundant, almost lax skin with excessive skin folds at wrists and ankles 
  • Increased palmar wrinkling 
  • Severe bruising with risk of subcutaneous hematoma 
  • Soft and doughy skin texture 
  • Skin hyperextensibility 
  • Atrophic scars 
  • Soft, doughy skin 
  • Atrophic scarring 
  • Skin hyperextensibility 
  • Soft skin 
  • Atrophic scarring 
  • Easy bruising 
  • Delayed wound healing 
  • Thin skin 

Hypermobile EDS (hEDS) »

Common skin features
Soft, mildly stretchy skin
Mild atrophic scarring
Stretch marks

Classical EDS (cEDS) »

Common skin features
Extensive skin involvement, usually apparent from a very young age
Very soft, stretchy, fragile skin
Severe atrophic scarring
Doughy skin texture
Easy bruising
Molluscoid pseudotumors
Subcutaneous spheroids

Vascular EDS (vEDS) »

Common skin features
Easy bruising
Thin, translucent skin with increased venous visibility
Premature aging

Periodontal EDS (pEDS) »

Common skin features
Skin hyperextensibility
Skin fragility
Wide or atrophic scarring
Easy bruising
Premature aging
Prominent vasculature

Kyphoscoliotic EDS (kEDS) »

Common skin features
Skin hyperextensibility
Easy bruising
Skin fragility
Delayed wound healing

Spondylodysplastic EDS (spEDS) »

Common skin features
Skin hyperextensibility
Soft and doughy
Thin and translucent skin

Brittle cornea syndrome (BCS) »

Common skin features
Soft, velvety, and/or translucent skin
Skin hyperextensibility

Arthrochalasia EDS (aEDS) »

Common skin features
Skin hyperextensibility
Atrophic scars
Easy bruising

Musculocontractural EDS (mcEDS) »

Common skin features
Skin hyperextensibility
Easy bruising
Skin fragility with atrophic scars
Increased palmar wrinkling

Classical-like EDS (clEDS) »

Common skin features
Skin hyperextensibility and velvety skin texture without atrophic scarring
Easy bruising

Dermatosparaxis EDS (dEDS) »

Common skin features
Extreme skin fragility with congenital or postnatal tears
Progressively redundant, almost lax skin with excessive skin folds at wrists and ankles
Increased palmar wrinkling
Severe bruising with risk of subcutaneous hematoma
Soft and doughy skin texture
Skin hyperextensibility
Atrophic scars

Myopathic EDS (mEDS) »

Common skin features
Soft, doughy skin
Atrophic scarring

Cardiac-valvular EDS (cvEDS) »

Common skin features
Skin hyperextensibility
Soft skin
Atrophic scarring
Easy bruising
Delayed wound healing
Thin skin

FAQs

Skin extensibility, or skin stretchiness, is measured by pinching and gently pulling the skin to measure the distance it can stretch. At this time, the doctor can also examine the thickness and texture of the skin. Skin extensibility is usually measured on the ventral (underside) of the forearm, the back of the hand, or the neck.  

Skin is hyperextensible if it stretches greater than: 

  • 1.5 cm on the forearms 
  • 1.5 cm on the back of the hands 
  • 3 cm on the neck 
  • 3 cm on the elbows 
  • 3 cm on the knees 
  • 1 cm on the palm of the hand 

Atrophic scars occur in some types of EDS. These scars form when tissue does not regenerate correctly and wounds heal at a lower level, causing them to appear sunken. 

Papyraceous scars are atrophic scars that have widened to become paper-thin. 

 

Translucent skin occurs because the skin is thin, which makes the deeper veins more visible. Thin, translucent skin is often seen in the general population, especially in the young, the elderly, and those with very light skin tones. Translucent skin does not necessarily mean a person has any type of EDS. 

Very translucent skin occurs most commonly in these types of EDS: 

Wound healing is usually achieved within 14 to 21 days. Delayed wound healing means it takes longer for a skin injury to heal. Anything greater than 3-4 weeks would be considered delayed wound healing. Delayed healing often worsens with age. Other factors that can affect the healing process include: 

  • Smoking 
  • Poor blood supply 
  • Diabetes 
  • Medications 

To improve skin healing, it can be helpful to: 

  • Consume a well-balanced diet 
  • Avoid smoking tobacco 
  • Avoid reinjury during all phases of the healing process 
  • Use support bandages to reduce swelling around a wound 
  • Tightly control the blood sugar if the person is diabetic 

Seek medical advice if the wound reopens or if there are signs of infection, such as: 

  • Increased redness 
  • Increased pain 
  • Pus 
  • Fever 
  • Increased swelling 

It is essential to promptly address and properly clean new wounds to reduce the risk of infection. Wound repair should minimize tension on the edges of the wound by using sutures or adhesive strips. Effective post-injury management may include: 

  • Protection from sun damage, which can increase the discoloration of scars 
  • Continued use of adhesive tapes for 1-2 weeks after suture removal 
  • Cool packs 
  • Topical creams 

If a scar has already formed, cosmetics can be used to improve their appearance. Medical tattooing, or scar camouflage, may also be an option. 

Everyone with EDS has different degrees of skin involvement and heals at different rates. Generally, stitches should be used on any wound that is under tension (likely to pull apart). This means most wounds will require stitches, especially wounds that are deep, jagged, or greater than 1 centimeter in length.  

There are many methods of applying stitches. The surgeon may use an “interrupted horizontal mattress suture” technique to help reduce the tension on the edge of the healing wound. Stitches should be applied in two layers (deep and surface sutures) and left in for roughly twice as long as they would normally be. 

Smaller lacerations on the face may be closed with skin glue and adhesive strips. Staples can be considered for scalp wounds, larger deep wounds, and surgery. 

People with EDS do not seem to have a greater risk of reacting to adhesives. However, some people, with or without EDS, are reactive to the adhesive material and can develop a local reaction (dermatitis). This can be avoided with lowallergy dressing. Strong adhesive dressings may damage fragile skin when removed, so this should be done carefully. 

It may be helpful to cover the sharp edges of furniture around the home to prevent wounds caused by bumping into furniture. This is particularly important if you have a young child with severe skin fragility because young children fall often. 

Padding can be worn over the knees, shins, and elbows when a child is learning to walk. Shin guards can provide protection when a child is at school or playing. Children with severe skin fragility should be counseled about the risks of participating in rough activities (horseplay) and contact sports. It is also important to encourage sun protection because sun damage can increase the appearance of scars. 

We are not aware of research on the outcomes of tattoos and piercings in people with EDS or HSD. In general, it is best to avoid unnecessary procedures, but many people with EDS and HSD have successfully had piercings and tattoos. People with only mild skin involvement may not have any problems getting tattoos or piercings. Other people with more severe skin involvement may have poor cosmetic outcomes. 

Unfortunately, there is no scientific evidence to support the use of supplements to help the skin in EDS. 

Molluscoid pseudotumors are spongy lumps that form over scars. These lumps often occur in areas that are easily knocked and bumped, such as the elbows and knees. Subcutaneous spheroids arise due to trauma and are small mobile lumps of hardened fat that form under the skin on the arms and legs. Molluscoid pseudotumors and subcutaneous spheroids are observed in classical EDS (cEDS). 

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