Epidermolysis bullosa (EB): symptoms, treatment, and care

This is the most common type of EB accounting for 70% of all cases. EBS symptoms vary in severity. With EBS the missing protein and fragility occurs within the top layer of the skin known as the epidermis. 

Find out more about EB simplex

Can be less severe or severe (depending on whether it is dominant or recessive). The missing protein and fragility occur below the basement membrane, which is a thin, dense layer that lines most human tissue. 25% of all EB cases are Dystrophic EB. 

Find out more about Dystrophic eb

A rare form of EB that accounts for just 5% of all cases. JEB symptoms vary in severity and are caused by a missing protein in the skin. Fragility occurs within the structure that keeps the epidermis and dermis (the inner layer of the two main layers of skin) together – the basement membrane.

Find out more about junctional eb

KEB. A very rare form of EB (less than 1% of cases). Named because of the defective gene being responsible for the information required to produce the protein Kindlin1. With KEB, fragility can occur at multiple levels of the skin. 

Find out more about KINDLER eb

• touch or friction can cause shearing of the skin and blisters to form, blisters are not self-limiting so need to be lanced regularly to avoid them enlarging.  
• the healing of the blisters can cause pain, severe itching, and scarring. 
• in some types of EB, blistering may mainly occur on the hands and feet which causes problems with walking/mobility, and other daily activities. 
• in severe forms of EB, internal blisters such as inside the mouth can make it difficult to swallow and there can be narrowing of the oesophagus (throat) and airways which would require medical intervention. 
• widespread blistering and wounds can cause the skin to become infected if not managed well. 
• some types of EB can experience extensive scarring, a change in the colour of the skin over time, and a higher risk of developing skin cancers.  
• the build-up of scar tissue can cause fingers and toes to fuse together which can require medical intervention. 
• EB can affect other organs within the body in addition to the skin including the bones and intestines, it can also lead to other medical complications, including constipation, particularly for children due to blistering around the bottom, and because it is a side effect of some types of painkillers. EB can also lead to anaemia. The effects of EB are multisystemic and in severe types of EB bone density can be severely impacted. 

Two of the most common symptoms associated with EB are pain and itch. These symptoms occur due to the frequent and sometimes extensive blistering that can be present all over the body and internally due to missing or abnormal protein(s) caused by a faulty or mutated gene, which means the skin doesn’t bind together as it should.  

There are currently no cures for EB but there are supportive measures/medications that are to help with pain and itch as well as other symptoms. Your EB healthcare specialist will be able to advise which treatments are suitable for you, but below is a general overview of the causes and treatments for these two common symptoms.

There are many complex reasons why people with EB experience pain and identifying the cause  is important so that pain reduction advice can be offered. If you are experiencing pain and require support, the specialist EB healthcare teams will be able to advise and support you with pain management. The DEBRA EB Community Support Team can also provide you with practical and emotional support including grants to fund items which may help with pain and itch.  

Common causes of pain with EB include: 

• blisters / blister healing. 
• areas of skin loss and open wounds. 
• lesions (an area of abnormal or damaged tissue) on mucous membranes, which are tissue which secretes mucus and lines cavities and organs, these include the mouth, eyelids, stomach, and the cornea (front part of the eye). 
• infections. 
• internal blistering. 
• trauma to the skin like a rub or bang. 
• overheating. 
• unknown causes or complications unrelated to the skin. 
• application of incorrect dressings or topical treatments.  
• dressings changes. 
• sensitivity to products such as laundry detergents and deodorants. 
• clothing materials. 

Once you know why you are experiencing pain (even if there are unknown causes), you can work with your EB healthcare specialist on a pain reduction plan. Below are some general tips on minimising pain for people living with EB, however, what works for one person may not work for another so you should always seek advice from your EB healthcare specialist for your individual situation. 

Itching is an unpleasant sensation that provokes scratching. For people living with EB, itching can be very painful. Scratching can be difficult to resist and can cause further skin trauma and lead to the breakdown of wounds that are nearly healed. Scratching can also lead to an inflammatory reaction, which further strengthens the itch sensation. 

Common causes of itch in patients with EB 

• healing blisters.  
• dry skin. 
• overheating. 
• inflammation. 
• persistent skin damage due to re-occurrence of blisters in the same area.  
• some opiates/opioids (pain relief drugs) can increase itch. 
• sensitivity to products such as laundry detergent, deodorants, and other products which meet the skin. 
• stress can increase itch – see useful links for information and resources to help you deal with stress. 
• anaemia which can be a side effect of EB that results in itching. 
• unknown, or a combination of causes. 

In the most severe cases EB can be very visible and can affect multiple areas of the body, however in other cases, for example EB Simplex, which accounts for 70% of all EB cases, it can be less visible and affect only certain areas of the body such as the feet. EB can also be a dynamic disability which means the effects of the condition on the person can be changeable. For example, one person with EB may never need any mobility support and instead will make the necessary adjustments to help manage or prevent their symptoms, however another person with EB may have a need for a mobility aid on occasion, and for another they may have a frequent need for mobility support.

Our members have told us that EB can feel like a hidden disability which can create additional challenges because EB, in all its forms, can be hard to live with both physically and mentally without having to be questioned or being made to feel like you must explain what it is. This is why it is important that more people know and understand EB.

Find out more about EB as a hidden disability

For most people living with EB, or caring for someone with EB, wound care is a big part of daily life. Knowing how to manage blisters and different wound types, treat pain and itch, prevent, and treat infection and when to seek medical advice are all crucial in EB wound care. 

There is a range of support to help individuals and families deal with the challenges of living with EB including the EB healthcare centres of excellence where patients living in any of the four nations may be referred for regular healthcare and support. The EB healthcare specialists in these centres, some of which are part-funded by DEBRA UK, are highly knowledgeable and experienced in how to care for skin, including how to lance blisters and what treatments are available to relieve symptoms. You can ask to be referred to one of the centres through your GP. If your GP is uncertain about referring you or you are unsure about what to request, please contact our EB Community Support Team who will be able to help you and can provide a letter template to share with your GP. 

Below you can find information to help you in care for blisters and minimise skin damage, along with links to other useful resources. 

An important part of any treatment plan for people living with EB is preventing trauma or friction to the skin to reduce the frequency of blistering and therefore reduce pain, itch, and scarring. Everyone’s experience with EB is slightly different and advice will vary depending on the severity and type of EB. It is therefore recommended that you seek support from an EB healthcare specialist for your individual circumstances but as a guide it is recommended to: 

• minimise walking long distances and try to save your skin/feet for essential journeys where possible. 
• try and avoid bumps and scratches and try to avoid rubbing the skin – parents may need to adapt how they lift babies and children. 
• try to find comfortable clothes that don’t rub against the skin and where you can, avoid bulky seams, where possible, wear natural smooth fibres such as silk, bamboo and cotton as this can help reduce irritation. 
• keep the skin as cool as possible. 
• choose comfortable footwear that doesn’t have hard seams inside. Find out more in the footwear guide.
• use any aids and adaptations suggested by your healthcare team which could be simple solutions such as insoles or a perching stool, or mobility aids such as a wheelchair or grab rails in the bathroom. Always ask your EB specialist as some equipment or mobility aids may not be appropriate for your EB. 
• ask others to be considerate of your needs. 

People living with EB often describe the pain from their skin damage as being like third degree burns, and in some cases, there may be deep loss of skin over large areas. Specific care is required to limit pain, itch and other symptoms associated with blistering. If you’re not sure how to care for your skin, or that of someone in your care, always refer to a specialist. You can find contact details for the specialist centres as well as what to do in an emergency here.

The DEBRA EB Community Support Team can also provide practical advice including knowing your rights and the obligations of education providers and employers to ensure that pain relief can be administered at the correct times. Talking to those around you about EB may also help you to cope with the physical and mental health challenges of living with EB.  

To be able to manage the symptoms of EB you will require certain products and supplies. What you need will depend on the type and severity of your EB, and your EB healthcare specialist will be able to advise you but below is an indication of the equipment and supplies you are likely to need: 

• Scissors. Sharp scissors will be needed to cut and trim bandages, regular scissors also work for cutting dressings. Make sure you clean and sanitise scissors after each use. 
• Wound dressings. There are a wide range of dressings available for different types of EB and your EB healthcare specialist will be able to advise you about the most suitable dressings for you or the person you care for. In all cases it is important to use non-adhesive dressings that do not stick to the skin to minimise further damage. 
• Bandages. Bandages may be needed to ensure that the dressings stay in place because if dressings slip, they can tear the fragile skin or cause the wounds to stick to clothing or bedding. A retention bandage can help ensure that dressings stay in place.  
• Moisturisers. Itching can be a major problem in all forms of EB. As wounds heal, or as infections flare up, itching can become troublesome but keeping the skin well moisturised can really help. 
• Antimicrobial cleansers. There is a risk of infection with all types of EB due to often large areas of open wounds and thus antimicrobial cleansers, moisturisers and topical treatments are often necessary to reduce this risk. A topical treatment is a medication applied to a particular place on the body with the goal being that it treats the tissues to which it has been applied without significant effects at other sites. 

There are specialist suppliers who can deliver items such as these directly to your home, and many pharmacies who offer a prescription delivery service so check with your local pharmacy. One of these providers is Bullen Healthcare who have extensive experience supporting the EB Community. They consistently hold a large stock of products and supplies commonly prescribed to people living with EB and have even created a dedicated team to help with all EB queries and orders. To find out more about accessing medical supplies and getting the appropriate prescription medication, please contact the DEBRA EB Community Support Team.

Whilst you may be able to minimise the risk of skin trauma, blisters are inevitable and sometimes spontaneous, appearing without any obvious cause. Blisters are also not self-limiting and may get bigger if left alone. Bigger blisters = bigger wounds, and so managing blisters is an important part of your skin care routine and lancing them as soon as possible is important. Please refer to the resources section (LINK TO ‘RESOURCES’) for further information on skin care. 

The aim is to prevent the blister getting bigger by draining off any fluid, leaving an opening large enough to stop the blister re-sealing and re-forming, whilst protecting the raw skin underneath. 

Below are some tips to help you manage your blisters:  

1. Lancing or ‘popping’ blisters with a hypodermic needle to allow drainage of blister fluid. This prevents the blister from enlarging and creating a larger raw area of damaged skin. 
2. Use a sterile needle – the size is important so speak to your healthcare provider to make sure you are using the correct size. You can ask your GP or EB specialist centre for a supply of sterile needles. Please note you will need a sharps box and a collection service for the disposal of needles. Information about disposing of needles.
3. Lance at the lowest point of the blister so that gravity can help drain fluid away. 
4. Gently apply pressure with a gauze or clean cloth to assist fluid drainage – some people prefer to use a clean syringe to remove fluid. 
5. Leave the roof of the blister intact to protect the raw skin underneath and reduce the possibility of infection. 
6. Remove any dead skin or debris from around the blister, whilst leaving the roof of the blister intact – dab don’t rub to limit skin damage. 
7. If part of the raw skin underneath is left exposed like an open wound, you may want to dress that area using non-stick dressings advised by your healthcare provider. Do not use normal sticky plasters as these can cause further damage to the skin. If a sticky plaster is used in error, there are adhesive removal products including sprays and wipes that can help limit any damage to the skin, these may be available to you on prescription through your GP or through pharmacies. It may also be useful to provide these products to your child’s school, childcare provider, or to carry around with you for use at appointments, e.g. when giving blood. 
8. It may be helpful to keep the wound moist as dryness can make itching worse. There are creams available to help with this. 

Your healthcare team will help advise you about blister care and can recommend dressings and products that are appropriate for your skin and type of EB. 

Blisters can also appear internally – in the mouth, anal area, and other mucous membranes (nose, mouth, lungs stomach), which can be distressing. Missing proteins that give skin its strength is expressed in different tissues within the body, including the membrane covering the eye and the tissue within the mouth and oesophagus. Please talk to your EB healthcare team for support on how to treat these types of blisters. 

In many cases, once a blister is lanced it will heal and no longer cause pain however some people can still experience pain and itch without the presence of blisters.  

An area of skin that has blistered may become even more fragile, especially following any re-occurrence of blisters in the same area. 

Sometimes a wound doesn’t heal, or heals but then breaks down again, which can be painful, and it can make the wound more susceptible to infection. This is known as a chronic wound. In these situations, talk to your EB healthcare specialist to identify why the wound isn’t healing so that they can help, e.g., by suggesting an alternative type of dressing, by using a cream, or dressing with anti-fungal/anti-bacterial properties, or by prescribing something to clearing the infection. There are also other factors that can affect how well your wounds heal including nutrition, sleep and reducing stress. Please contact your EB healthcare specialist or the DEBRA EB Community Support Team for wellbeing support. 

The frequent dressing changes required to manage EB can be extremely distressing and painful, yet they are a life-long and important part of regular, sometimes daily skin care, wound and blister management. 

Blisters should be lanced as soon as possible to prevent them causing further pain and damage. 

The time taken to complete dressing changes can vary greatly but for optimum pain reduction it is advised to complete dressing changes in the least time possible. There are different dressings available, and ensuring you have the most appropriate dressings is important, e.g. severely affected newborns need minimal handling and may require dressings that can remain in place for several days.  

Non-adhesive dressings are crucial to reduce further damage and pain. If an adhesive dressing is applied in error, there are adhesive removal products available to you on prescription through your GP or through pharmacies. Silicone based dressings are often easier to apply and remove than traditional, stickier dressings. Your EB specialist healthcare team will be best placed to advise. 

The EB specialist healthcare teams at the EB healthcare centres of excellence have extensive expertise in wound management and will be able to advise on the right treatment plan for you. Please contact your EB healthcare specialist or if you don’t have access to a specialist, the DEBRA EB Community Support Team can help you with a referral. 

It can be very difficult dealing with the pain associated with wounds and the blistering of the skin caused by EB however there are different options for pain relief depending on the severity of the symptoms experienced and the associated pain, these include creams, gels, and oral medication. 

For certain types of EB, such as EBS, over-the-counter painkillers may provide relief but please be aware that children under the age of 16 should never be given aspirin as there is a small risk it could trigger a serious condition called Reye’s Syndrome, this is advised by the NHS. 

Stronger options for managing pain may also be available via your EB healthcare specialist on prescription including morphine, which is often used prior to dressing changes, and oral sucrose solution for infants, where small amounts of sweet solutions (oral sucrose) are placed on the tongue to reduce procedural pain. This has been shown to be beneficial prior to and during procedures in newborn babies. 

Please discuss the use of painkillers, even over-the-counter ones with your EB healthcare specialist. 

Reducing the time it takes to do dressing changes, for example by using templates to cut dressings in advance reduces the time that the individual experiences distress and helps reduce their pain.  

Some people living with EB find that doing things they enjoy, such as listening to music, spending time with others, getting outside, playing games, or watching TV provide a useful distraction. Using mindfulness and breathing techniques amongst other wellbeing interventions can also be helpful. The healthcare teams at the EB specialist centres have extensive experience in pain management and can support you, however mild or severe your pain is, with pain management techniques or a crisis plan. 

The useful links section contains links to organisations that offer support on pain management techniques.  

You can also access tips and advice about managing chronic pain on the NHS website.

Open wounds or raw skin can become infected which then requires urgent treatment to prevent further pain and damage. Many infections can be prevented with thorough hand washing and clean equipment is essential when lancing blisters and changing dressings. 

The following may indicate the presence of an infection. 

If you experience any of these symptoms or you are concerned your wounds may be infected, you should seek a face-to-face medical review with your local GP or healthcare provider. 

• redness and heat around the area of skin. 
• the area of skin leaking pus or a watery discharge. 
• crusting on the surface of the wound. 
• a wound that is not healing. 
• a red streak or line spreading away from a blister, or a collection of blisters (may be more difficult to see on black or brown skin). 
• a high temperature (fever) of 38C (100.4F) or above. 
• an unusual odour. 
• increased pain. 

At the first sign of infection, please contact your GP or healthcare provider who will be able to provide a suitable treatment which may include antiseptic creams, antibiotics, gels, or specialist dressings. 

For longer term support and to aid wound healing you may be able to boost your immunity via nutrition and dietary supplements. Talk to your EB healthcare specialist to discuss the best nutritional plan for you, also visit our diet and nutrition section for delicious recipes from our EB specialist dietitians and members, bursting with healthy ingredients to provide high protein, nutrient-rich and, for some types of EB, high calorie meals, puddings, or snacks.

Good skin care is important to help reduce itch. Although the urge to scratch can be difficult to resist, some people find patting the area gently with a cool damp cloth, or taking a cool bath provides some relief. If the severity of itch necessitates it, there are medications available such as topical creams and ointments to help reduce itch. 

Ensuring you are hydrated, avoiding overheating, and being mindful of the products that contact your skin, will also help. 

Relaxation, breathing and mindfulness techniques can also provide relief, often in combination with medical and other behavioural treatments. Different solutions work for different people so it’s best to discuss your individual needs with your EB healthcare specialist . 

More information and tips on how to treat itchy skin.

EB wounds and blisters can heal with a scar. Scarring is part of the body’s natural healing process when tissue is damaged and can be mild, superficial, and temporary, or extensive and permanent. Generally, the more scar tissue there is, the more fragile that area can be. Padding for these vulnerable areas of skin can help to limit and slow further damage. 

Extensive scarring can lead to complications which may require surgery, your EB healthcare specialist will discuss with you what treatment options are available. 

If you have any concerns about scarring or need support, the EB specialist healthcare teams have extensive experience in this area and can discuss your concerns along with any emotional support you may need. 

Stress and a lack of sleep can negatively impact wound healing and the ability to deal with pain. However, these symptoms can be improved through stress management techniques, nutritional supplements, medication, meditation, mindfulness, and other wellbeing interventions. Your EB healthcare team will be able to support you to find the right treatment for you, you can also access additional resources here or visit here for more information. 

Living with EB can be difficult, but the DEBRA EB Community Support Team is here for everyone living with or directly affected by every type of inherited and acquired EB. The team can offer emotional, practical, and financial information and support at every stage of life. 

Joining DEBRA UK as a member is completely free, and membership gives you access to all the services offered by the DEBRA EB Community Support Team, plus other great benefits including bespoke events, where you can connect with members of the EB community in-person or on-line, discounted holiday breaks, advocacy, and expert financial information, support, and grants. 

Membership also gives you a voice and the opportunity to shape what we do; the research projects we invest in, and the services we offer for the whole EB community. Also by simply joining as a member you will be making a difference because the more members we have, the more data we have, which is crucial to support the EB research programme, and more members gives us a louder voice to help lobby the government, the NHS, and other organisations for the support needed to improve services for the benefit of the entire EB community.