Skin Features of EDS 

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: 

• Vascular EDS (vEDS) 
• Brittle cornea syndrome (BCS) 
• Spondylodysplastic EDS (spEDS) 

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 low–allergy 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).