Materiały źródłowe

• #1 Overview of the management of epidermolysis bullosa – UpToDate

  https://www.uptodate.com/contents/overview-of-the-management-of-epidermolysis-bullosa

  Epidermolysis bullosa (EB) is a heterogeneous group of hereditary mechanobullous diseases characterized by varying degrees of skin and mucosa fragility caused by mutations that affect skin structural proteins. […] The management of patients with EB is largely supportive and includes wound care and prevention and treatment of complications. […] Treatment is largely supportive and includes wound care, control of infection, nutritional support, and prevention and treatment of complications. […] The management of patients with EB involves a multidisciplinary team usually composed of a dermatologist, an EB nurse with specialized expertise in wound care, primary care provider, occupational therapist, nutritionist, and social worker.

• #2 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9006505/

  Epidermolysis bullosa (EB) is a disabling and chronic genodermatosis characterized by mucocutaneous fragility with blister formation after minimal trauma. […] No specific treatment is available; however, a multidisciplinary management is mandatory in order to treat the lesions, to prevent complication, and to give a psychological support to the patient and family members. […] The multidisciplinary team has been progressively established; the dermatologists act as patient case manager, in collaboration with the pediatrician, endocrinologist, dietician, dentist, plastic surgeon, digestive surgeon, geneticist, psychologist and a dedicated nurse. […] An adequate management of EB patients require a multidisciplinary approach with an educational plan to guarantee an appropriate treatment and to support and accompany patients and their families since birth along life.

• #3 Epidermolysis bullosa: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/epidermolysis-bullosa-treatment

  People diagnosed with epidermolysis bullosa are often referred to a specialized treatment center when possible. At these centers, you’ll find dermatologists, pediatric dermatologists, nurses, dieticians, psychologists, and others who have expertise in managing this rare condition. […] The doctor (or team) caring for a patient with epidermolysis bullosa develops a treatment plan carefully tailored to the patient’s needs. This care plan is written and given to the family and others who will care for the patient. […] When the condition is moderate or severe, the patient requires professional medical help, often from a team of medical professionals with different areas of expertise. A nurse may go to the home of a patient to help with treatment. […] Wound care involves changing bandages and dressings, usually daily.

• #3 Epidermolysis bullosa: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/epidermolysis-bullosa-treatment

  When treating wounds, wearing a new pair of clean medical gloves each time can help prevent an infection from developing in a wound. […] Great care is taken when removing bandages and dressings, which are usually changed one at a time. […] Each wound is then gently cleansed with a mild, non-toxic solution like saline, treated with medication if necessary, and covered with a new dressing and bandage. […] Pain management: Pain is a common symptom. Many things can cause pain, including the blisters and raw skin, dressing changes, an infection, swallowing, eating, or just moving. […] Before bathing and wound care, it may be necessary to give pain medication. […] To prevent new wounds, your dermatologist may recommend using padded bandages or wearing loose-fitting clothes. […] Your dermatologist can give you detailed advice that can help avoid new injuries.

• #4 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-022-01252-3

  Epidermolysis bullosa (EB) is a disabling and chronic genodermatosis characterized by mucocutaneous fragility with blister formation after minimal trauma. […] No specific treatment is available; however, a multidisciplinary management is mandatory in order to treat the lesions, to prevent complication, and to give a psychological support to the patient and family members. […] The multidisciplinary team has been progressively established; the dermatologists act as patient case manager, in collaboration with the pediatrician, endocrinologist, dietician, dentist, plastic surgeon, digestive surgeon, geneticist, psychologist and a dedicated nurse. […] An adequate management of EB patients require a multidisciplinary approach with an educational plan to guarantee an appropriate treatment and to support and accompany patients and their families since birth along life.

• #5 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-022-01252-3

  The dynamic educational plan adopted in our hospital showed good clinical and psychological outcome in our population, allowing adherence to treatment, reducing the frequency of complications and improving life expectancy and quality of life. […] Before discharging the patient, parents/caregivers should be given a specific and personalized training in order to guarantee an appropriate treatment at home. […] The dermatologist and the nurse teach the parents how to handle the baby and to breastfeed him/her, how to remove the dressings avoiding trauma, to break the blister roof, and to remove the crusts in order to reduce itching, and to recognize wound characteristics, the respective management and dressing, to dress the fingers in patients with recessive dystrophic EB to delay syndactyly.

• #6

  https://www.nursingcenter.com/journalarticle?Article_ID=849738&Journal_ID=849729&Issue_ID=849731

  ABSTRACT: Epidermolysis bullosa (EB) is a lifelong genetic skin disorder. It is characterized by extreme skin fragility resulting in chronic erosions and blisters. […] Wound care plays a crucial role in the care of these individuals. It becomes a part of their daily routines. Wound care has two goals, wound protection and wound healing. […] Good wound care practices can improve an individual’s quality of life. The ultimate goal is to allow these individuals the opportunity to live as normal a life as possible. Remember, we are children only once, and we need to give these children the opportunity to live the life of a child.

• #7 Epidermolysis bullosa

  https://dermnetnz.org/topics/epidermolysis-bullosa

  EB usually occurs at birth or shortly after. […] The primary aim is to protect the skin and stop blister formation, promote healing, and prevent complications. […] A team of medical specialists is usually required for overall care. […] The following are some general measures used in caring for a patient with EB. […] Avoid activities that induce friction on the skin. This includes the handling of infants and children alternative handling techniques are easily learnt from a trained health care professional. […] Maintain a cool environment and avoid overheating. […] Use foam padding or sheepskins to help reduce friction on pieces of furniture such as beds, chairs and infant car seats. […] Choose clothing (including nappies) and footwear that is light, has no irritating seams or detail, (eg, zips and tight elastic).

• #8 Epidermolysis bullosa – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/symptoms-causes/syc-20361062

  Epidermolysis bullosa has no cure, but mild forms may improve with age. Treatment focuses on caring for blisters and preventing new ones. […] It’s not possible to prevent epidermolysis bullosa. But these steps may help prevent blisters and infection. […] Handle your child gently. Your infant or child needs cuddling, but be very gentle. To pick up a child with epidermolysis bullosa, place the child on soft material and give support under the buttocks and behind the neck. Don’t lift the child from under the arms. […] Take special care with the diaper area. If your child wears diapers, remove the elastic bands and avoid cleansing wipes. Line the diaper with a nonstick dressing or spread it with a thick layer of zinc oxide paste. […] Keep the skin moist. Gently apply moisturizer as needed throughout the day.

• #8 Epidermolysis bullosa – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/symptoms-causes/syc-20361062

  Dress your child in soft clothes. Use soft clothing that’s simple to get on and off. It may help to remove labels and put on clothing seam-side out to reduce scratching. Try sewing foam pads into the lining of clothing by elbows, knees and other pressure points. Use soft special shoes, if possible. […] Prevent scratching. Trim your child’s fingernails regularly. […] Encourage your child to be active. As your child grows, encourage activities that reduce the risk of skin injury. Swimming is a good option. For children with mild forms of epidermolysis bullosa, they can protect the skin by wearing long pants and sleeves for outdoor activities. […] Cover hard surfaces. Consider padding a car seat or bathing tub with sheepskin, foam or a thick towel. Soft cotton or silk can be used as a top layer over the padding.

• #9 Neonatal guidelines for suspected Epidermolysis Bullosa (EB) | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/new-baby-epidermolysis-bullosa-eb/

  Handle with care – avoid rubbing or friction/shearing forces which can result in skin damage. […] We advise against wearing gloves when handling babies with fragile skin. If policy dictates wearing gloves then apply 50:50 white soft paraffin: liquid paraffin or Emollin Spray to the fingertips to prevent friction with the skin. […] Avoid sliding your hands under the baby as shearing forces may cause skin damage. Gently roll the baby onto their side and then roll back onto your hands and lift. […] Ensure baby is clothed, with dressings on before holding or lifting. Avoid handling naked babies which can result in accidental damage. […] Do not rub area as it may result blisters or skin loss. […] Secure cannula with a Soft Silicone Tape (Siltape, Mepitac or 3m Kind Removal Silicone Tape). If Soft Silicone Tape not available, use adhesive tape but remove following guidelines below.

• #9 Neonatal guidelines for suspected Epidermolysis Bullosa (EB) | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/new-baby-epidermolysis-bullosa-eb/

  These guidelines are intended to support healthcare professionals and parents caring for neonates with suspected EB on the neonatal or post-natal ward. Infants with EB are safest nursed in their local neonatal unit (there is no need to transfer to specialised centres). […] Handling and feeding techniques require modification when nursing an affected neonate, otherwise clinical intervention may result in unnecessary blistering or skin loss. […] The EB nursing team will also support clinical staff unfamiliar with EB to care for these fragile patients. Multiple outreach visits may be required for neonates with significant skin loss/severe EB. […] If recommended products outlined in this guideline are not available – discuss with the EB nurses for advice on adaptation or alternatives. These suggestions are for immediate care and may be changed by the EB nurses during their first and subsequent visits.

• #9 Neonatal guidelines for suspected Epidermolysis Bullosa (EB) | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/new-baby-epidermolysis-bullosa-eb/

  Ensure adequate analgesia given 30 minutes prior to wound care (avoid PR medication if possible as this can blister the anal margin). […] Carefully remove soiled dressings using the SMARs or Emollin spray if stuck. […] If wearing gloves please ensure they are well lubricated and change when soiled. If not wearing gloves, ensure adequate hand washing between each limb. […] Avoid any bathing until inter-uterine and birth damage has healed. […] Blisters are not self-limiting and will enlarge if not lanced. This can result in further tissue damage, pain or infection if left untreated. […] Cleanse with 50% liquid paraffin, 50% soft white paraffin mix or Emollin Spray and gauze. Avoid using baby wipes which can irritate the skin. […] Ensure baby is dressed as soon as possible, as clothing offers protection from damage when being handled. […] Protect infants lips with Vaseline (for breast and bottle feeding). […] If NG feeding is essential, use tube suitable for long-term feeding and secure with Soft Silicone Tape, or contact the EB nursing team to learn about the Lasoo technique.

• #10 Epidermolysis bullosa – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/diagnosis-treatment/drc-20361146

  Your health care provider can show you how to care for blisters properly and advise you on ways to prevent them. Ask about safe ways to drain blisters before they get too large. Ask about recommended products for keeping the affected areas moist. This helps with healing and preventing infection. […] In general, take these steps: […] Wash your hands before touching blisters or changing dressings. […] Control pain. About 30 minutes before a dressing change or other painful procedure, older children and adults may take a prescription-strength pain medication. […] Cleanse skin daily. To cleanse a wound, soak it for 5 to 10 minutes in a mild solution of salt and water. […] Puncture new blisters. This prevents them from spreading. Use a sterile needle to puncture each new blister in two spots.

• #11 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9006505/

  The dynamic educational plan adopted in our hospital showed good clinical and psychological outcome in our population, allowing adherence to treatment, reducing the frequency of complications and improving life expectancy and quality of life. […] Before discharging the patient, parents/caregivers should be given a specific and personalized training in order to guarantee an appropriate treatment at home. […] It is recommended to lance new blisters with a finger prick lancet or a sterile needle and drained to prevent their expansion, leaving the blister roof in place to reduce infection risk and pain. […] A psychologist is involved to work alongside the dermatologist and nurse to support families from the earliest days of the newborn’s life. […] The pediatrician together with the dietician explain the importance of hydration, quality and quantity of nutritional intake, check the blood exams and prescribes support therapies with vitamins and food supplements.

• #12 Epidermolysis bullosa: recognition and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/epidermolysis-bullosa-recognition-and-management

  Optimal management requires a multidisciplinary approach that focuses on the protection of susceptible tissues against trauma; use of sophisticated wound care dressings; aggressive nutritional support; and early medical or surgical interventions to correct extracutaneous complications, whenever possible. […] A patient-centred multidisciplinary approach is required for EB management, as the condition and medical needs vary in each patient. Currently there is no definitive treatment. For all patients, the principles of care to improve quality of life and minimise complications include avoiding trauma; good skin care; infection prevention; and symptom management. […] Blisters in EB are not self-limiting and will extend rapidly if left unattended. They can develop anywhere and may involve mucous membranes. Intact blisters should be lanced at their lowest point and drained to limit tissue damage.

• #12 Epidermolysis bullosa: recognition and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/epidermolysis-bullosa-recognition-and-management

  The underlying principle of skin and wound management is to select an atraumatic dressing to: Prevent blistering and skin damage; Minimise pain and bleeding during dressing changes; Prevent infection; Allow cooling of the blister sites; Encourage wound healing. […] Pain is a common complaint with all types of EB and can originate from blister sites, areas of skin loss and affected areas of the mucous membrane. Normal daily routines can involve several painful events; for example bathing, mobilising and dressing changes. […] Good skin care and oral hygiene and physiotherapy may delay the need for surgery. […] Malnutrition is common, owing to swallowing difficulties caused by blistering of the oral mucosa. Reduced food intake and increased metabolic demands from wound healing lead to failure to thrive, delayed puberty and anaemia, which may further affect wound healing and skin integrity.

• #13 A consensus approach to wound care in epidermolysis bullosa

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3655403/

  The objective of this study was to generate a list of recommendations that will allow practitioners to better manage the complex needs of this population. […] The first step is to create an inventory of the body surface area involved and the type of wounds (intact blisters, erosions/ulcers, chronic, exudative vs nonexudative wounds). […] Dressing choices should be individualized based on EB subtype, extent and wound location, dressing frequency, cost, and availability. […] Wound healing requires an appropriate wound surface moisture balance. […] A comprehensive assessment should result in an individualized wound care plan tailored to the individual, taking into consideration unique biopsychosocial needs. […] EB is not just a skin disorder; therefore treating a patient with EB requires involvement of a dedicated team with expertise in all aspects of care.

• #14 Epidermolysis Bullosa (EB) | Mölnlycke Advantage

  https://www.molnlycke.ae/education/wound-areas/other-wounds/epidermolysis-bullosa-eb/

  For sufferers of the incurable skin condition Epidermolysis bullosa (EB), protecting the delicate skin around lesions, addressing the bio-burden and exudate management are all key. […] Care for EB patients focuses on managing the condition as EB has no cure yet. […] Wounds need to be cared for and blisters pierced, drained and dressed. […] With so many wounds at different stages of healing, the management of EB is complex. Protecting the peri-wound skin, avoiding skin stripping, addressing the bio-burden and exudate management are all key factors to consider. […] Wound dressings – the skin is so fragile and dressing changes so frequent that atraumatic dressings are recommended to prevent further damage, pain or bleeding. […] Infection management – there is a high risk of infection due to large areas of open wounds. Antimicrobial cleansers, moisturisers and topical treatments are necessary to manage the wound bio-burden.

• #15 Epidermolysis Bullosa (EB) | Mölnlycke Advantage

  https://www.molnlycke.ae/education/wound-areas/other-wounds/epidermolysis-bullosa-eb/

  Blister management – EB blisters need careful management as they will extend rapidly if left unchecked. […] Dressing retention – if dressings slip, they can tear the fragile skin and cause the wounds to stick to clothing of bedding. The dressing should be held firmly in place with a retention bandage.

• #16 Care for Newborns with EB | debra of America

  https://www.debra.org/how/care-newborns-eb

  Care for Newborns with Epidermolysis Bullosa (EB) includes several important practices. […] At present, there is no specific treatment for EB. Current therapy is directed toward the prevention of skin trauma, prevention of infection, and the treatment of complications. […] Hand washing is the most effective measure to control infection. […] Daily dressing changes are recommended for your newborn with Epidermolysis Bullosa (EB) and can be coordinated with a bath or cleansing of the skin. […] It is important to chart the growth of an infant/child with EB. Such charts provide essential information for evaluating the adequacy of the diet. […] It is not unusual for nursing staff and parents to avoid handling a baby with Epidermolysis Bullosa (EB), with the hopes of minimizing blisters. Learning the correct way to hold the baby will instill confidence in the caregivers and allow the baby to receive the emotional support and closeness he/she needs. […] Standard treatment for EB patients is daily wound care, bandaging, and pain management. […] There is no right or perfect way to dress EB wounds; many families have had success with a wide variety of methods of wrapping and types of medical products.

• #17 Recessive Dystrophic Epidermolysis Bullosa (RDEB) | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/severe-recessive-dystrophic-epidermolysis-bullosa-rdeb/

  Cleaning with water can sting the blisters and sores and therefore we recommend cleansing with 50/50 ointment or Emollin emollient spray. Blisters should be lanced as above. A layer of barrier cream such as Proshield Plus barrier cream, reapplied at each nappy change, should protect blistered and vulnerable areas. Open wounds are covered with the dressing Intrasite Conformable. […] If there is extensive skin loss at birth, we recommend delaying bathing until healing has taken place. This is because bathing can be a painful process and it is difficult to protect the baby from further skin damage when all the dressings are removed at once. […] Open wounds must be dressed to encourage healing and stop them sticking to clothing. Suitable dressings include Urgotul (Urgo), PolyMem (Ferris) and a range of soft silicone dressings, which all provide protection for the skin and encourage wound healing.

• #17 Recessive Dystrophic Epidermolysis Bullosa (RDEB) | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/severe-recessive-dystrophic-epidermolysis-bullosa-rdeb/

  Babies may be reluctant to feed due to soreness from blisters in the mouth. A Haberman (Special Needs) feeder is often helpful. […] Blisters and wounds can be painful but childrens need for pain relieving medicines varies greatly. […] In all forms of RDEB, the hands become scarred. In those with RDEB-SG, the hands have a tendency to contract leading to the fingers becoming webbed and forced into a fist. This can be delayed by splinting the hands under the guidance of an EB occupational therapist. […] While those with RDEB pruriginosa experience itching most of all, this is a problem that affects all types of DEB. Inevitably, itching leads to scratching which damages the fragile skin. Wounds that are almost healed are particularly itchy.

• #18

  https://www.nhs.uk/conditions/epidermolysis-bullosa/treatment/

  Children with severe types of EB can experience blistering and irritation in and around their eyes. […] If your baby has blisters in their mouth, it can cause problems with feeding. Your treatment team can give you advice about how to overcome feeding problems. […] Surgery may be needed to treat some complications that can arise in severe cases of EB.

• #18

  https://www.nhs.uk/conditions/epidermolysis-bullosa/treatment/

  There’s currently no cure for epidermolysis bullosa (EB), but treatment can help ease and control symptoms. […] An important part of your child’s treatment plan will be practical advice about how to prevent trauma or friction to your child’s skin to reduce the frequency of blistering. […] Your treatment team will be able to advise about caring for your child’s skin. […] If you think your child has a skin infection, let your GP know as soon as possible. […] The blisters and wounds can be painful and make simple activities such as moving and walking difficult. […] Good dental hygiene, using a soft toothbrush and a mouthwash that contains fluoride (as well as regular visits to a dentist), is important. […] Your child’s fingernails and toenails may become thicker than normal and difficult to cut, especially if blisters form under the nail.

• #19 Epidermolysis Bullosa | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/epidermolysis-bullosa

  Blistering and sores can be painful, so expert pain management is often an important part of the treatment plan. […] Antibiotics may be needed if wounds become infected. […] Children with blistering and sores in the lining of the digestive system may need treatment for chronic constipation or diarrhea, nutrition and vitamin deficiencies, growth problems related to poor absorption of food, or for narrowing of the esophagus. Treatment may include changes in diet, vitamin and nutritional supplements, medication or the placement of a gastrostomy tube (G-tube). […] Children with more severe cases of EB often suffer from chronic anemia due to iron deficiency, chronic disease, and blood loss from open wounds. Depending on the severity of the anemia and the cause, it may be treated with vitamins or supplements, diet, antibiotics or blood infusions.

• #19 Epidermolysis Bullosa | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/epidermolysis-bullosa

  Dealing with a chronic disease like epidermolysis bullosa can be stressful and at times overwhelming for children and their families. Psychologists can provide coping strategies for dealing with pain and stress. […] Because a childs activities and range of motion may be restricted to prevent blistering, or because of the pain of blisters, therapy is often needed to help a child stay physically active and to maintain engagement in school and with friends. […] In severe cases, surgery may be needed to correct problems caused by epidermolysis bullosa, such as GI strictures or fused fingers or toes. […] At Childrens Hospital of Philadelphia, your childs care is managed through the Epidermolysis Bullosa Multidisciplinary Clinic, where they can receive coordinated treatment from all the specialists they may need.

• #19 Epidermolysis Bullosa | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/epidermolysis-bullosa

  Two localized gene therapies to treat EB have been approved for use by the Food and Drug Administration (FDA) and are offered at CHOP. […] Because of the nature of the disease and the many body systems it can impact, children with epidermolysis bullosa may need special support or treatment for a variety of different medical issues. Among the issues addressed in EB treatment are: […] The first line treatment for a child with EB is to minimize sources of friction on the skin that cause blistering. Your childs medical team can advise on ways of holding, clothing, and diapering your child to help prevent blisters. […] Expert wound care is critical for all patients with the disease. Special dressings and wound-care supplies may be needed, as contact with standard adhesives and dressings can cause friction and additional blistering.

• #20 Epidermolysis Bullosa (EB) Care of Neonates | NHSGGC

  https://clinicalguidelines.scot.nhs.uk/ggc-paediatric-guidelines/ggc-paediatric-guidelines/neonatology/epidermolysis-bullosa-eb-care-of-neonates/

  This guideline covers the immediate care of neonates with Epidermolysis Bullosa (EB), or neonates provisionally diagnosed with EB. […] Do not nurse in an incubator unless required for other medical conditions, such as prematurity, as heat and humidity can exacerbate blistering. […] Use a greasy emollient commonly known as 50/50 ointment (50% liquid paraffin/50% white soft paraffin) to cleanse napkin area in preference to water, this aids cleansing without further trauma and will reduce pain. […] Avoid bathing until inter-uterine and birth damage have healed and helps prevent damage from infant being handled naked. […] Use Latex Orthodontic feeding teat (commonly available in wards and cleft lip service) to avoid friction to oral mucosa. […] Analgesia prior to dressing change. […] Dressing type: Polymeric membrane Brand: Polymem. This is the first choice dressing for severe neonatal wounding/critical colonisation/infection.

• #21 Epidermolysis Bullosa (EB): Definition, Causes, Symptoms, Treatments

  https://www.apollo247.com/blog/article/epidermolysis-bullosa-causes-symptoms-treatment-prevention

  Epidermolysis Bullosa is a rare genetic disorder that affects the skin and mucous membranes, causing them to be extremely fragile and prone to blistering. […] Living with EB can be challenging, but there are several Epidermolysis Bullosa treatment options available to help manage the symptoms and improve quality of life. […] Daily wound care routines are of utmost importance in managing EB. Here are some key practices to incorporate into your routine: Gently clean the affected areas with mild soap and warm water. […] Apply prescribed ointments or creams to keep the skin moisturized and protected. […] Use non-stick dressings or bandages to prevent further damage to the skin. […] Avoid adhesive dressings that may cause more friction and damage. […] Pain management for individuals with Epidermolysis Bullosa (EB) requires a multifaceted approach, considering the chronic nature of the condition and the potential pain associated with blistering, wound care, and complications.

• #21 Epidermolysis Bullosa (EB): Definition, Causes, Symptoms, Treatments

  https://www.apollo247.com/blog/article/epidermolysis-bullosa-causes-symptoms-treatment-prevention

  Since individuals with EB have fragile skin that is prone to blistering and can be more susceptible to infections, implementing infection prevention measures is crucial to maintaining skin health and overall well-being. […] Preventing complications and promoting a good quality of life for individuals with EB require a comprehensive and multidisciplinary approach. […] Psychological and emotional support is also essential for both patients and their families. Dealing with a chronic condition like EB can be emotionally challenging. Seek support from healthcare professionals, support groups, and counselling services to help cope with the emotional impact.

• #22 Epidermolysis Bullosa | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/epidermolysis-bullosa/

  Physical and occupational therapy are encouraged to help maintain range of motion and to work out modifications for daily-life activities. […] Children’s Colorado offers the only center in the region for babies, kids and teens with epidermolysis bullosa. Our specially trained and experienced clinicians provide the delicate, individualized care that kids require. […] Patients typically participate in physical and occupational therapies to address issues of mobility, equipment needs, feeding and hand function. […] Decisions regarding pain management during procedures and exams are made with the family and the EB team to determine what is best for each child. Patients have access to anesthesia services and a pain management team to minimize pain for procedures and exams, and a full-time EB nurse who assists with in-hospital dressing changes.

• #22 Epidermolysis Bullosa | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/epidermolysis-bullosa/

  Epidermolysis bullosa (EB) is a family of inherited skin diseases characterized by fragile skin that is prone to breakdown and blistering. […] Unfortunately, there is no cure for EB. Treatment mainly centers on wound care and early diagnosis and treatment of complications. […] Treatment centers on wound care and managing other symptoms of EB. It is important to remember that there is no one-size-fits-all approach to treating EB. A patient might need all of these approaches, or just a few: […] Frequent bandaging in order to keep blisters clean and protected is a very essential and important aspect of EB care. Some blisters might also need to be lanced (popped open) and drained. […] Medication may be used to treat pain and itching associated with EB. […] Soaking in baths with bleach or pool salt can also be comforting for patients with EB.

• #23 Epidermolysis bullosa – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/diagnosis-treatment/drc-20361146

  Apply treated dressings. Spread petroleum jelly or other thick moisturizer on a nonstick bandage. […] Wrap blistered hands and feet daily. With some severe forms of this condition, daily wraps help prevent contractures and fusion of the fingers and toes. […] Watch for signs of infection. If you notice heat, pus or lines leading from the blister, talk with your health care provider about prescription antibiotics. […] Keep it cool. Blistering is often worsened by heat and warm conditions. […] A varied, nutritious diet promotes growth and development in children and helps wounds heal. […] Talk with your health care provider about how you or your child can get all the needed nutrients and vitamins. […] Treating and preventing blisters and worrying about complications can be stressful. You may find it helpful to share concerns and experiences with families in similar circumstances.

• #23 Epidermolysis bullosa – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/diagnosis-treatment/drc-20361146

  Ask your health care provider to suggest stress-reduction resources or consult with a psychologist to learn stress-reduction techniques. […] Centers that specialize in the diagnosis, evaluation and treatment of people with epidermolysis bullosa may belong to a network called EB Clinet. […] Visiting such a center regularly can improve quality of life and reduce hospitalizations from complications for people with epidermolysis bullosa. […] Call your health care provider immediately if you see signs of infection around a blister.

• #24

  https://journals.lww.com/jdds/fulltext/2022/26010/skin_cleansing_and_wound_care_practice_in_patients.3.aspx

  Epidermolysis bullosa (EB) is a group of genodermatoses characterized by fragile skin that may progress to erosions, blisters, and open nonhealing wounds. Understanding home skincare practices and topical products use among patients with EB is necessary to optimize management outcomes and quality of life. […] Education is needed for EB patients and their families on lancing of blisters and topical antibiotic use, especially in light of increasing antibiotic resistance. Dressing modification for EB patients living in the Gulf region countries, particularly during hot and humid months, may be helpful. […] Regular bathing, particularly with the use of additives, can be helpful to reduce bacterial colonization and pain associated with EB wounds. […] Dressing choices in EB patients should be individualized depending on patient age, EB subtype, extent of wound lesion, dressing frequency, cost, and availability. Wound healing is best achieved using atraumatic dressing based on the wound characteristics and drainage.

• #24

  https://journals.lww.com/jdds/fulltext/2022/26010/skin_cleansing_and_wound_care_practice_in_patients.3.aspx

  Most of our patients required assistance during dressing changes, regardless of their EB type. […] Educating patients and their families in blister care and involvement in EB care may be valuable. Modification in dressing layers may be considered in managing EB patients in the Middle East region, particularly during summer months. […] We also suggest possible dressing modifications for EB patients living in the Gulf region countries, particularly during hot and humid months.

• #25 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-022-01252-3

  An appropriate wound management is mandatory. […] A psychologist is involved to work alongside the dermatologist and nurse to support families from the earliest days of the newborn’s life. […] We offer to all families, as well as to the children and adolescents psychological support. […] The pediatrician together with the dietician explain the importance of hydration, quality and quantity of nutritional intake, check the blood exams and prescribes support therapies with vitamins and food supplements. […] The physiotherapist teaches the advantages of physiotherapy and suggests the appropriate sport activities to prevent/delay contractures and osteoporosis. […] The dentist educates the parents to the oral hygiene since the first months of life in order to prevent/delay scarring, caries and parodontopathy.

• #26 Epidermolysis Bullosa | Doctor

  https://patient.info/doctor/epidermolysis-bullosa-pro

  Treatment of infections. […] Corneal erosions require antibiotic ointment and cycloplegic agents to reduce ciliary spasm and provide comfort. Avoid using tape to patch the eye, as it may cause blistering of the skin under the adhesive. […] Good dental hygiene and regular dental checks are recommended. Many patients with JEB and DEB develop dental caries because of enamel defects. Oral mucosal involvement can accompany severe forms of JEB and DEB. Avoid harsh mouthwashes containing alcohol. Normal saline rinses can help to clean the mucosal surfaces. […] Because of the extensive skin damage, a diet high in calories, protein and vitamins is required. Malnutrition is especially liable in the dystrophic types. A soft diet helps to reduce oral and oesophageal erosions. […] Surveillance of chronic lesions is important as they are susceptible to undergoing malignant change to squamous cell carcinoma. Such change often occurs at multiple sites. Surgical excision is required. These lesions tend to be aggressive.

• #27 Epidermolysis bullosa | Altru Health System

  https://www.altru.org/health-library/conditions/epidermolysis-bullosa

  A varied, nutritious diet promotes growth and development in children and helps wounds heal. If blisters in the mouth or throat make it difficult to eat, here are some suggestions: For babies with mild epidermolysis bullosa, breastfeeding is fine. […] Treating and preventing blisters and worrying about complications can be stressful. You may find it helpful to share concerns and experiences with families in similar circumstances. […] Centers that specialize in the diagnosis, evaluation and treatment of people with epidermolysis bullosa may belong to a network called EB Clinet. Such centers are staffed with doctors, nurses, social workers and rehabilitation specialists who provide specialized care for people with this condition.

• #28 Care for EB Wounds | debra of America

  https://www.debra.org/how/care-eb-wounds

  Wound care is part of the supportive care given to those with Epidermolysis Bullosa (EB). […] The following list of suggested methods of wound care has been compiled from several sources and addresses all forms of EB. […] It is important to note that no one wound care product works for every person or every wound. […] A blister, erosion, or wound should be bandaged if: Protection will prevent trauma and friction, Dressings reduce the pain, The area is draining or bleeding, Infection is present. […] When wounds are healing slowly or appear not to be healing, the wound bed should be examined by the treating medical provider. […] Wound healing may be delayed or disrupted due to: Blood flow, Anemia, Iron stores, Infection, Steroids, Skin Cancer. […] It is important to work with a Registered Dietitian to make sure that the appropriate amount of nutrition is being consumed. […] Use dressings that help the healing process. Non-adherent dressings are needed in EB. […] Monitor the Wounds for Changes. […] If the wound is not healing, the wound care plan may need to be changed.

• #29 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9006505/

  The physiotherapist teaches the advantages of physiotherapy and suggests the appropriate sport activities to prevent/delay contractures and osteoporosis. […] The dentist educates the parents to the oral hygiene since the first months of life in order to prevent/delay scarring, caries and parodontopathy. […] We offer to all families, as well as to the children and adolescents psychological support. […] Follow up of the patient depends on EB subtype and patients age to prevent and early treat complications. […] The feeling of joy of having a newborn turns into fear, guilt, shock, denial and helplessness because of the heavy burden of caring for a seriously ill child. […] An early therapeutic alliance between the medical professionals and the family facilitates adaptation to the disease, which can stabilize and protect the family unit, the relationship between all members, and the psychophysical development of the child.

• #30 Epidermolysis bullosa: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001457.htm

  Epidermolysis bullosa (EB) is a group of disorders in which skin blisters form after a minor injury. It is passed down in families. […] The goal of treatment is to prevent blisters from forming and avoiding complications. Other treatment will depend on how bad the condition is. […] Follow these guidelines at home: Take good care of your skin to prevent infections. Follow your provider’s advice if blistered areas become crusted or raw. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your provider will let you know if you need a bandage or dressing, and if so, what type to use. […] Take good care of your oral health and get regular dental check-ups. It is best to see a dentist who has experience treating people with EB. […] Do exercises a physical therapist shows you to help keep your joints and muscles mobile.

• #31 Epidermolysis bullosa | Children’s Health Queensland

  https://www.childrens.health.qld.gov.au/health-a-to-z/epidermolysis-bullosa

  Dental care is important in all children with EB. Blistering does occur in the mouth and the use of an extra soft, childrens sized toothbrush will make brushing more comfortable. Early referral along with regular visits to your dentist for professional cleaning and fluoride treatments is highly recommended for prevention of dental cavities. […] Bathing newborns is not recommended until early birth damage has healed. Bath oil and gentle soap free wash is recommended for all children with EB. Salt can be added to baths when your child has extensive blistering and may help with any pain experienced during the bathing process. Salt should be added at a rate of 90g per 10 litres of water to achieve a 0.9 per cent solution. Please speak to your EB nurse for further information regarding bathing and salt use.

• #32 Epidermolysis bullosa: Tips dermatologists give parents

  https://www.aad.org/public/diseases/a-z/epidermolysis-bullosa-self-care

  Dryness in the eyes can lead to a scratched cornea, but using eye drops or eye ointments may prevent this and other eye problems. […] Encourage play and movement. It’s understandably hard to encourage your child to play and move about when the skin is so fragile, but children with EB should move as much as they can. It is better for a child to get a blister now and again than to be too afraid to move. Moving around helps to: Prevent constipation. […] Reduce the shortening and thickening of muscles, tendons, and other tissues, which can make it difficult if not impossible to move the affected area of the body. […] Stop muscle wasting, as muscles can shrink and weaken without movement. […] Make appointments for full-body skin exams. Regardless of the type of EB your child has, dermatologists recommend full-body skin exams beginning at age 10. Problems can develop when wounds: Occur again and again

• #33 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-022-01252-3

  The plastic surgeon is involved in case of pseudosyndactyly or of squamous cell carcinoma. […] Follow up of the patient depends on EB subtype and patients age to prevent and early treat complications. […] The family must reconstruct its identity accepting the disease, reorganizing daily life, creating a routine of care in which parents should feel capable and effective, equipped to sustain the care by themselves. […] An early therapeutic alliance between the medical professionals and the family facilitates adaptation to the disease, which can stabilize and protect the family unit, the relationship between all members, and the psychophysical development of the child. […] The child becomes more aware of his or her illness, and experiences medication as a very traumatic moment. […] The relationship with the healthcare staff, who increasingly speak directly to the child, can support him/her in the search for new motivations, reshaping the goals and negotiating with him/her the treatment, as far as possible. […] Compliant child and parents let reduce the frequency of complications at an early stage and allow better quality of life and life expectancy.

• #34 Epidermolysis bullosa | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/epidermolysis-bullosa

  Epidermolysis bullosa has no cure, but mild forms may improve with age. Treatment focuses on caring for blisters and preventing new ones. […] Your health care provider can show you how to care for blisters properly and advise you on ways to prevent them. Ask about safe ways to drain blisters before they get too large. Ask about recommended products for keeping the affected areas moist. This helps with healing and preventing infection. […] A varied, nutritious diet promotes growth and development in children and helps wounds heal. If blisters in the mouth or throat make it difficult to eat, here are some suggestions: […] Treating and preventing blisters and worrying about complications can be stressful. You may find it helpful to share concerns and experiences with families in similar circumstances. […] Centers that specialize in the diagnosis, evaluation and treatment of people with epidermolysis bullosa may belong to a network called EB Clinet. Such centers are staffed with doctors, nurses, social workers and rehabilitation specialists who provide specialized care for people with this condition.

• #35 Caring for a child with Epidermolysis Bullosa: a scoping review on the family impacts and support needs :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-29-number-2/caring-child-epidermolysis-bullosa-scoping-review-family-impacts-and-support-needs

  Aims Epidermolysis Bullosa (EB) is a rare genetic disorder characterised by recurrent skin blistering. Wound care and nursing are critical to everyday lives of EB patients. The aim of this review was to identify the support needs of parents of a child with EB and to assess the impact EB has on the family unit, irrespective of subtype of condition severity. […] Wound management and nursing are an important aspect of support for families with a child with EB; however, many health professionals do not adequately understand the impact EB has on the individuals and the family unit. […] The characteristics of severe EB demand higher than average levels of support compared to other children with chronic needs as disease management is focused on symptom control and extensive wound care. […] The most common needs identified were emotional needs, followed by practical needs, social needs and physical needs. Many parents also reported a lack of informational and psychological support.

• #36 Caring for a child with Epidermolysis Bullosa: a scoping review on the family impacts and support needs :: Cambridge Media Journals

  https://journals.cambridgemedia.com.au/wpr/volume-29-number-2/caring-child-epidermolysis-bullosa-scoping-review-family-impacts-and-support-needs

  The daily burden of care and missed opportunities to spend quality time together often had a negative impact, with one study reporting nearly half of all parent relationships ending in separation or divorce. […] Parents reported feeling overwhelmed, exhausted and mentally fatigued, highlighting the greater need for better community support services providing respite. […] The need for better informational support was most evident in the groups of parents caring for a newborn where a definitive diagnosis was pending, and parents caring for a child with lethal EB, resulting in delayed referrals to specialists in some cases. […] The psychological needs of parents are related to the level of social and family support, relationship status and severity/progression of the child’s disease.

• #37 Multidisciplinary care for patients with epidermolysis bullosa from birth to adolescence: experience of one Italian reference center

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9006505/

  The child becomes more aware of his or her illness, and experiences medication as a very traumatic moment. […] It is useful to involve the child so that he/she becomes part of the self-care, collaborating with his/her parents and becoming an „expert” in the disease. […] The relationship with the healthcare staff, who increasingly speak directly to the child, can support him/her in the search for new motivations, reshaping the goals and negotiating with him/her the treatment, as far as possible. […] In the adolescent period, it is very difficult to maintain adherence to the treatment, because they manifest a total rejection of their body, they realize that the pathology is chronic and worsening and they tend to be incompliant. […] An adequate disease multidisciplinary management, the involvement of both parents since the beginning of taking care, the constant psychological support and the appropriate and dynamic therapeutic patient education allow a greater adherence to treatment.

• #38 Epidermolysis Bullosa: Symptoms, Causes, Diagnosis, and Treatment

  https://www.verywellhealth.com/epidermolysis-bullosa-overview-4589859

  Blisters are the key symptom of EB. […] One of the most important things to do when managing EB is to prevent the formation of blisters as much as possible. […] Wound care is a very important aspect of treatment for EB. This will help prevent serious infection and allow for more rapid healing. Many people with EB will have wound dressings that need to be changed every day or two. A nurse may be able to provide you will special education and assistance in managing this problem. […] People with EB may need pain medications to lessen their discomfort. Different medications may also help to lessen itching. […] In 2023, the Food and Drug Administration (FDA) approved two drugs for treating EB: Vyjuvek (beremagene geperpavec) for the treatment of wounds in people aged 6 months and older who have DEB and mutations in the COL7A1 gene. […] Fulsuvez (birch triterpenes) to treat wounds caused by dystrophic and junctional EB in individuals aged 6 months and older.

• #39 Management of Skin Lesions in Patients with Epidermolysis Bullosa by Topical Treatment: Systematic Review and Meta-Analysis

  https://www.mdpi.com/2227-9032/12/2/261

  Currently, advanced therapies are being adopted to improve healing with the goal of correcting the underlying genetic pathology or mitigating its effects. […] These therapies ultimately aim to correct the absence or reduction in anchoring proteins located at the dermal-epidermal junction. […] Our work includes two trials that evaluated the use of fibroblasts. […] In conclusion, we identified isolated trials exploring the use of henna, tissue engineering grafts, and the investigation of other drugs to alleviate symptoms or reverse the blistering process. […] More clinical trials are needed to identify treatments that can reduce or prevent the onset of symptoms that significantly impact the quality of life of patients with epidermolysis bullosa.

• #40 Epidermolysis bullosa

  https://dermnetnz.org/topics/epidermolysis-bullosa

  Pierce, drain and dress blisters to promote healing (this should be done only by people who have received training on wound care). […] Many traditional adhesive tapes and dressings may be unsuitable for people with EB especially those with the more severe forms (eg, RDEB) as their removal can cause additional trauma to the skin. Use of advanced wound care products such as low-adherent silicone tapes and dressings is recommended. […] Oleogel-S10, containing birch triterpenes or birch bark extract, has been shown to accelerate the healing of chronic wounds in junctional and dystrophic epidermolysis bullosa. […] When EB affects other parts of the body, various cares and treatments are adopted. For example, a soft diet when the oesophagus is involved or using stool softeners for constipation, or if the patient has anal blisters. […] Clinical practice guidelines are available on the DEBRA International website including those on Oral Health care, Wound Care, Pain Management, Cancer Management.

• #41

  https://link.springer.com/article/10.1007/s13671-024-00437-9

  Epidermolysis bullosa (EB) is a heterogeneous group of rare inherited disorders that cause skin and mucous membrane fragility. Patients with EB have compromised wound healing due to the underlying genetic defect, which can be further complicated by related comorbidities. […] Wound healing in EB patients is challenging due to multiple wounds of varying duration and healing ability. In the absence of a cure, the goal of treatment is to minimize the risk of blister formation, optimize wound care, pain, and itch relief, and address specific complications. […] In this review, we discuss wound healing challenges, wound care strategies stratified by EB subtypes, as well as investigational cell-based, protein replacement, and gene therapies. […] A consensus approach to wound care in Epidermolysis Bullosa. […] Evaluation of wound care options in patients with recessive dystrophic epidermolysis bullosa: a costly necessity. […] Best practice guidelines for skin and wound care in Epidermolysis Bullosa. […] Wound management in Epidermolysis Bullosa.

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