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Epidermolysis Epidermolysis bullosa (EB) is a group of inherited diseases characterized by mechanical fragility of the skin and mucous membranes. Red and irritated tissue can possibly indicate infection or that the drainage is irritating the surrounding tissue. With good wound care practices, you will make a huge difference in the life of an individual who has epidermolysis bullosa. An official website of the United States government. Patients with more severe forms of EB are at an increased risk of squamous cell carcinoma (SCC). The Herlitz variant of junctional EB has a pathognomonic presentation with periorificial blistering, exuberant hypergranulation tissue, and periungual involvement with nail shedding. The current standard of care is supportive, which includes daily wound care, pain management, and protective bandaging. WebParticipants completed the Epidermolysis Bullosa Wound Care List (see additional information for the complete list). To prevent blister extension we recommend puncturing it (at multiple sites to facilitate optimal drainage) with a sterile needle to release the inner fluid. Effects on quality of life are substantial. A firm dehydrated eschar or soft slough requires debridement to remove senescent cells that are deficient in cellular activities and biofilms that maintain the inflammatory process.40 Debridement in the EB population should, whenever possible, involve nonphysical methods (hydrogel, calcium alginate dressings). The objective of this study was to generate a list of recommendations that will enable practitioners to better care for patients with EB. Using a product on a long-term basis can result in the development of these resistant organisms. Curr Opin Pediatr. The difference between colonization and infection is the interplay between the number and type of colonies and host resistance.41 In bacterial colonization, bacterial colonies do not interfere with healing. Another option for a supply company that bills for Medicaid, Medicare, and private insurance is CCS Medical. Fostering independence and safety during activities of daily living requires environmental modifications (special beds, seating in baths, wheelchairs, footwear). Sibbald RG. Epidermolysis bullosa This phase is characterized by the formation of a scar. Epidermolysis bullosa: Diagnosis and treatment - American A comprehensive assessment should result in an individualized wound care plan tailored to the individual, taking into consideration unique biopsychosocial needs (Table VII). The silver ions in silver products are activated when they come into contact with the wound drainage. To optimize nutritional status, patients with severe forms of EB may require a gastrostomy tube. However, topical antibiotics are not recommended to be used long-term. 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. Development of a therapeutic relationship involves appropriate support and education. Jones, V., Bale, S., & Harding, K. (2004). However, National Rehab does bill for Medicare and for some other private insurances, so they can also be a great resource as well. Biopsy of wounds that enlarge rapidly, have increased pain, change in appearance on serial photographic documentation, or feel different is recommended50. Calcium alginates are seaweed dressings that can assist in debriding and absorbing drainage (Jones et al., 2004). Infection and colonization in epidermolysis bullosa. Three or more criteria from STONEES suggest deeper/surrounding skin infection and need for systemic therapy. Mepilex Transfer is designed to be used on wounds with a large amount of drainage (Figure 8). Patients and parents experiences during wound care of These dressings are all silicone based. consensus approach to wound care in epidermolysis bullosa Tabolli S, Sampogna F, Di Pietro C, Paradisi A, Uras C, Zotti P, et al. Wounds that are kept in a moist environment heal faster and scar better (Schober-Flores, 2003). As noted previously, for a noninfected wound, it was recommended to add moisture to the wound by applying a topical ointment to the wound or dressing. Abstract Background Wound care is the cornerstone of treatment for patients with epidermolysis bullosa (EB); however, there are currently no guidelines to help practitioners care for these patients. Objectives An easy rule of thumb to remember is, if it is a dry wound, add moisture, but for a wet wound, dry it out. WebAt this first appointment, we want to establish a relationship with you and your child so that youll feel comfortable coming to us with any questions or concerns. Before deciding on a wound care management strategy it is important to take an inventory of the body surface area affected, and the types of skin involvement (intact blisters, erosions, chronic wounds). WebSurvey data from respondents self-reporting a diagnosis of EBS were analyzed for clinical and wound manifestations, medication use, and QOL (using Quality of Life in There may also be pocketing and tunneling around the wound. Thus, the severity of the skin disorder can range from a mild seasonal blistering, which hardly interferes with a person's lifestyle, to a severely debilitating and even life-threatening skin disorder, which completely consumes an individual's life (Schober-Flores, 1999). Respondents included physicians (67%), nurses (17%), and allied health professionals (7%). Murrell DF. WebEpidermolysis Bullosa, or EB, is a rare genetic connective tissue disorder that affects 1 out of every 20,000 births in the United States (approximately 200 children a year are born with EB). aSection of Dermatology, Department of Pediatrics, Hospital for Sick Children and University of Toronto, cSt Thomas Hospital and Great Ormond Street Hospital for Children, London, dDepartment of Obstetrics and Gynecology, Institute of Wound Research, University of Florida, Gainesville, eBiomedical Engineering, University of Alberta, Edmonton, gClinical Research of the Blood and Marrow Transplantation Program and Stem Cell Institute, University of Minnesota, Minneapolis, bDivision of Infectious Diseases, Department of Pediatrics, Hospital for Sick Children and University of Toronto, hWomen's College Hospital and University of Toronto. Signs of deep and surrounding tissue infection (lymphadenopathy, fever, and malaise) require systemic antimicrobial therapy. Overview of the management of epidermolysis bullosa We propose using this paradigm for patients with EB (by eliminating the undermining and allocating suffering SU rather than S) in nonhealing wounds for developing a wound care plan and monitoring the response over time. P30 EY021721/EY/NEI NIH HHS/United States. Sepsis occurs when the quantities of bacteria have reached 10,000,000-100,000,000 (Gardner & Frantz, 2004). wound care Hettiaratchy S, Papini R. Initial management of a major burn, II: assessment and resuscitation. Treatment of skin cancers in epidermolysis bullosa. Liy-Wong C, Tarango C, Pope E, Coates T, Bruckner AL, Feinstein JA, Schwieger-Briel A, Hubbard LD, Jane C, Torres-Pradilla M, Zmazek M, Lara-Corrales I. Orphanet J Rare Dis. Background: Epidermolysis bullosa is a rare, often severe, genetic disorder characterized by fragility of the skin and mucous membranes. Ingen-Housz-Oro S, Blanchet-Bardon C, Vrillat M, Dubertret L. Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa. Before Practice guidelines: a new reality in medicine, I: recent developments. Patients' and parents' experiences during wound care of epidermolysis bullosa from a dyadic perspective: a survey study. Advanced wound care products, such as Dermagraft, can be utilized when other treatment modalities have failed. Most types of this condition start at birth or soon after. Wound cleansing is especially important in these individuals because it not only decreases the bacteria of the skin, but it can also prevent an infection from developing. The bacterial load may be reduced by bathing with diluted bleach, applying compresses, or using sprays with diluted vinegar.39 Lipid-stabilized hydrogen peroxide cream (Crystacide, DermaUK, Stotfold, UK) is well tolerated and effective when applied directly on the wound or contact dressing.39 Topical antibiotics/antimicrobials (eg, polymyxin B-gramicidin, fusidic acid, mupirocin, silver sulfadiazine) should be used only for short periods of time and rotated every 2 to 6 weeks to prevent resistance and sensitization.39 When using these agents, we recommend applying them on the dressing rather than directly on the skin to limit pain and trauma. The type of EB and the severity of the skin disorder is determined by the lacking gene (Schober-Flores, 1999). epidermolysis bullosa The redosable treatment, which will be sold by Krystal as Vyjuvek, is for dystrophic epidermolysis bullosa. Care for an individual diagnosed with EB can be challenging. Vahlquist A, Tasanen K. Epidermolysis bullosa care in Scandinavia. To our knowledge, this is the first attempt to develop guidelines of care for the EB population that focus on wound care, with a holistic approach that takes into account other patient-related factors, patient preferences, and the immediate and extended care teams. Federal government websites often end in .gov or .mil. Other wound-related outcomes such as pain reduction, decreased bacterial load, and need for dressing changes, or increased quality of life are more attainable. Epub 2022 Nov 7. eCollection 2023. These guidelines need to be periodically renewed to reflect new scientific and clinical practice knowledge. Percent change in wound area of diabetic foot ulcers over a 4-week period is a robust predictor of complete healing in a 12-week prospective trial. The wound care plan should be clearly outlined in a written document given to the family and copied to the family practitioner and home-care personnel. Shinkuma S, Natsuga K, Nishie W, Shimizu H. Epidermolysis bullosa in Japan. Pain control can be a problem for persons diagnosed with EB, especially during bath time. Epidermolysis bullosa care in the United Kingdom. wound Gardner, S., & Frantz, R. (2004). Epidermolysis bullosa: A nursing perspective. Dive Brief: The Food and Drug Administration on Friday approved the first topical gene therapy, clearing a treatment developed by Krystal Biotech for a rare genetic skin disease that causes severe skin blistering and has no cure. Effect of clinical guidelines on medical practice: a systematic review of rigorous evaluations. Wound contamination indicates that bacteria are on the wound surface but that they are not reproducing, replicating, or interfering with wound healing (Gardner & Frantz, 2004). Lastly, Clorox soaks are effective against all types of organisms, including gram-positive, gram-negative, and even fungal organisms. The last phase of healing is the remodeling phase. 2022 Aug 13;17(1):314. doi: 10.1186/s13023-022-02461-z. Bruckner-Tuderman L. Epidermolysis bullosa care in Germany. Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa. Bathing for individuals with epidermolysis bullosa. Topical antibiotics can be used for wounds with critical colonization or for an infected wound. Diagnostic Dilemmas in Dermatology Column. Surface critical colonization and deep and surrounding skin infection are clinical diagnoses. Extracutaneous manifestations and complications of inherited epidermolysis bullosa, part II: other organs. Some of the Molnlycke products recommended for the noninfected wound are Mepitel, Mepilex lite, Mepilex, and Mepilex border (Molnlycke Healthcare, 2006; Figure 6). Some dressing options for the infected wound are foam products, such as Mepilex Transfer, Mepilex lite, and Mepilex, or silver products, such as Mepilex Silver, SilvaSorb, or Acticoat. The secondary bandage should be a dressing that can hold a fair amount of drainage; therefore, a foam bandage is preferable, such as Mepilex lite and Mepilex foam. Sprecher E. Epidermolysis bullosa care in Israel. Bodemer C. Epidermolysis bullosa in France: management in the national reference center for genodermatosis. The edge effect: current therapeutic options to advance the wound edge. Schober-Flores, C. (2003). the contents by NLM or the National Institutes of Health. It is important to understand how a wound heals, what an infected wound looks like, and what products are available. Epub 2022 Aug 10. This as a result will make their bath time something they look forward to rather than something they fear. Additional scientific research including effectiveness studies for everyday practice and expert consensus, may further refine these recommendations. Epidermolysis bullosa is an inherited mechanobullous disorder that is usually stable, but in some patients with underlying comorbidities, close monitoring or face-to-face management is required . When cleansing a wound, it is essential to remove the surface contaminants but not to traumatize the wound bed, and it is essential to choose the correct dressing products to promote wound healing but not to hinder it. Moreover, gastrointestinal upset and constipation are reasons for non-adherence. The experts were asked to rate each recommendation on a 4-point Likert scale (strongly disagree, slightly disagree, slightly agree, strongly agree). Lai-Cheong JE, McGrath JA. Fine JD, Eady RA, Bauer EA, Bauer JW, Bruckner-Tuderman L, Heagerty A, et al. Advanced wound care products are tissue-engineered skin equivalents developed from the neonate foreskin. Following the application of the wound care ointment, there was a reduction in wound surface area on central (96%) and distal mid-back (92%) by treatment visit three, and there was a 96% reduction on the left shoulder blade ulcer by treatment visit four. These bacteria are replicating and multiplying in quantities, which cause injury and impair healing. The standard of care for wound cleansing is to use solutions that are gentle and noncytotoxic.38 For patients with EB we recommend gentle cleansing with a saline solution, water, or dermol 500 (containing benzalkonium chloride 0.1%, chlorhexidine hydrochloride 0.1%). Epidermolysis Bullosa Multidisciplinary Clinic WebTypes of EB Several types of EB have been identified. Methods Allegaert K, Naulaers G. Gabapentin as part of multimodal analgesia in a newborn with epidermolysis bullosa. Drain blisters with sterile needle to prevent tracking, Consider nontraumatic conservative debridement of slough, Superficial critical colonization (NERDS) and abnormal inflammation, Deep/surrounding tissue infection (STONEES)/generalized inflammation, Autolytic debridement: alginates, hydrogels, Superficial critical colonization: silver, honey, PHMB, Moisture balance foams with silicone coatings to prevent trauma and pain, If wound is stalled or edge/other areas appear atypical, consider skin biopsy to rule out squamous cell carcinoma or other complications before considering active therapeutic options. Epidermolysis bullosa care in Mexico. For this reason, it is recommended to use Hibiclens only for short-term and only when indicated. It does accomplish its task of eliminating gram-positive organisms, but this "opens the door" for the gram-negative organisms. During this phase, the growth factors are stimulating the wound to fill it in with new granulation tissues, new blood vessels are being formed, and new endothelial cells are forming along the borders of the wound. There were 15 respondents (45% response rate), with significant experience in the EB field (<1 year [13%]; 1-5 years [13%]; 6-10 years [7%]; 11-15 years [7%]; and >15 years [60%]). Ideal methods of serial assessment of wounds in patients with EB are lacking. Itching at night may be related to body overheating and treated with sedating antihistamines (hydroxyzine) or a tricyclic with prominent H-1 antihistamine action (doxepin). The list was further refined by grouping items into main themes and specific recommendations (Table II). Soaking of each individual wound for 5 to 10 minutes or removing the dressings in the bathtub may help reduce pain and trauma associated with dressing changes. In contrast, bullous pemphigoid presents with basal keratinocyte hemidesmosomes in the dermoepidermal junction, which results in a split at the dermoepidermal junction and clinically forms tense blisters. During this time, the scar will lighten, soften, and blend with uninjured skin. The .gov means its official. Therefore, the goal for choosing a dressing product for individuals diagnosed with EB is to find a product that will not only lessen the duration of the inflammatory phase but also protect fragile tissue from breakdown during the proliferative and remodeling phases of healing (Cuzzell, 1995). This treatment not only cleanses the wound, but it also decreases the bacterial count of the wound, stimulates the growth factors, and decreases the pain of a wound. However, this scar will never be as strong as uninjured skin. Grimshaw JM, Russell IT. Tape has strong adhesives that will remove their skin when the tape is removed. WebEpidermolysis bullosa: Dressing care. Woo KY, Sibbald RG. In actuality, bacterial wound contamination and colonization are common to all healing wounds and are in fact prerequisites for the wound-healing process (Gardner & Frantz, 2004). Classic signs and symptoms of infection are significant periwound redness, swelling, tenderness, warmth, and pain. A wound size reduction of 20% to 40% in 2 and 4 weeks is quoted to be a reliable predictor of healing at 12 weeks.46-48 In addition, clinical observation of the edge of the wound is foretelling: nonhealing wounds often have a cliff edge instead of the purple tapered sandy shore beach of healable ones. If a wound is infected with gram-positive organisms, such as Staphylococcus aureus or streptococcus, a capful of Hibiclens can be very effective against eliminating those organisms (Schober-Flores, 2003). The diaper region is often particularly difficult to manage, as large denuded areas are difficult to protect from urine and feces. For nonresponders, gabapentin,29 pregabalin, or other antiepileptics may be helpful. Certain dressings can cause a distinct odor or can cause an increase of the drainage of the wound. To date, there are no specific wound care guidelines or any evidence that address the wound care challenges of the EB population. Patients with EB simplex (EBS) present predominantly with acral blisters exacerbated by heat and friction.6 Blistering can be more extensive in the generalized forms of EBS and recessive forms of EBS with suprabasal cleavage.6,7 Dowling-Meara form of EBS is characterized by grouped blisters that extend at the periphery resembling a string of pearls and acral blisters that lead to painful keratoderma. Whether the wound is actually infected or not, if there is a high bacterial count on the skin surface, the grafting process will not be successful. Blood sampling can be difficult as a result of poor venous access, therefore a more practical approach for assessing the overall nutritional status is monitoring of the growth curves in pediatric patients and body mass index in adults with EB. Does the wound have the characteristic symptoms of infection such as redness, odor, pain, and increased drainage? Fridge JL, Vichinsky EP. These products can dissolve and often become incorporated into the wound. wound care Another dressing option would be the restore products made from Hollister Wound Care. Narcissus Way, Denver, Colorado 80237. In S. Baranoski & E. Ayello (Eds.). Next, assess the moisture balance of the wound. However, the first step is to understand how a wound heals. Tabolli S, Pagliarello C, Uras C, Di Pietro C, Zambruno G, Castiglia D, et al. The frequency and severity of pain is often proportional to disease severity, with up to 50% of patients with the most extensive type of EB (RDEB) experiencing daily pain greater than 5 (0-10 scale).23 Although the cause of pain in EB is multifactorial, the skin and related EB lesions are by far the most significant source of pain. Therefore, EB is considered a multiorgan system disorder (Schober-Flores, 1999). We have brought together experts in the fields of EB, wound care biology, and clinical practice to provide the best available approaches to optimize wound care in patients with EB.