There were 15 respondents (45% response rate), with significant experience in the EB field (>10 years [67%]). Journal of the Dermatology Nurses' Association, Get new journal Tables of Contents sent right to your email inbox, January-February 2009 - Volume 1 - Issue 1, Epidermolysis Bullosa: Wound Care Pearls for the Noninfected and Infected Wound, Articles in PubMed by Carol Schober-Flores, Articles in Google Scholar by Carol Schober-Flores, Other articles in this journal by Carol Schober-Flores, Privacy Policy (Updated December 15, 2022). Individuals with EB have extensive skin erosions, and when water comes into contact with their wounds, it is very painful. 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). Please enable scripts and reload this page. WebWound care: Because the primary symptom of epidermolysis bullosa is skin blistering, expert wound care is critical for all patients with EB. Moist environments promote wound healing, but too much moisture can hinder healing. 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). There may also be pocketing and tunneling around the wound. Epidermolysis bullosa in the Netherlands. Fourteen items (Table I) were generated through plenary discussion and sent for formal consensus. Quality of life in patients with epidermolysis bullosa. 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). What causes EB? These were further refined and grouped into 5 main themes (assessment and management of factors that impair healing, patient-centered concerns, local wound care, development of an individualized care plan, and organizational support) and 17 specific recommendations. may email you for journal alerts and information, but is committed Federal government websites often end in .gov or .mil. A multicenter study of percentage change in venous leg ulcer area as a prognostic index of healing at 24 weeks. An easy rule of thumb to remember is, if it is a dry wound, add moisture, but for a wet wound, dry it out. There was more than 85% agreement for all the proposed items. EB is a prototype of an orphan disease. Neuropathic pain often responds to tricyclic agents, particularly second-generation agents. 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. Objectives: 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. Epidermolysis Bullosa A centralized, interprofessional approach with care coordination including open communication with the general practitioner and home-care team is the most effective way of caring for these patients. Baranoski, S., & Ayello, E. (2004a). If a dressing product does adhere, you can very easily remove or "deglove" an individual's skin, which will result in a very traumatic and painful dressing change. Second, if used on a chronic basis, there is a potential for a gram-negative organism to grow. This is called critical colonization (Gardner & Frantz, 2004). wound Another option for a supply company that bills for Medicaid, Medicare, and private insurance is CCS Medical. Fine JD, Johnson LB, Weiner M, Suchindran C. Assessment of mobility, activities and pain in different subtypes of epidermolysis bullosa. 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%). Therefore, dressings are utilized not only for wound healing but also for wound protection (Figure 4). Therefore, you never want to apply a dressing product that might potentially adhere to the wound or to any loose tissue surrounding the wound. Perhaps the dressing adhered to and traumatized the wound bed with its removal, or the cleansing of the wound was too aggressive. EB is a complex multisystem disease; therefore communication among various health care professionals is paramount. Epidermolysis bullosa (EB), often referred to as the butterfly disease, is a group of rare genetic conditions characterized by skin that is delicate and fragile as butterfly wings. The multivalent wound care ointment provides a novel option for an effective control of bioburden and moisture management in patients with EB. Inclusion in an NLM database does not imply endorsement of, or agreement with, Biopsy of wounds that enlarge rapidly, have increased pain, change in appearance on serial photographic documentation, or feel different is recommended50. This phase is rich in growth factors. However, this scar will never be as strong as uninjured skin. Junctional EB causes blistering between the epidermis and dermis (the lower layer of the skin) Woo KY, Sibbald RG. Ultrasonic mist is a noncontact, low-intensity, low-frequency ultrasonic treatment (Extended Care Professional News, 2007). Epidermolysis Bullosa and Chronic Wounds: A Model Accessibility The 11 attendees (physicians and nurses) were selected based on their EB clinical and research expertise and background in wound care, wound-healing biology, infectious diseases, and bone-marrow transplantation. Extended Care Professional News (ECPN). 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. Wound Dermatological aspects of wound care. Wound care in the EB population poses unique challenges: clinical variability requires an individualized management plan; availability of a myriad of wound care products complicates the decision process and there is a high overall cost to the family and health units. 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. With the new, disease-modifying cellular therapies that are currently emerging,52 it is also important to maximize the chances of each patient being a potential candidate for these therapies (Table VIII). Fine JD, Johnson LB, Weiner M, Li KP, Suchindran C. Epidermolysis bullosa and the risk of life-threatening cancers: the national EB registry experience, 1986-2006. This tissue disintegrates, and the wound enlarges in either depth or circumference. Abstract Background: In epidermolysis bullosa simplex (EBS), epithelial structural fragility results in blisters and erosions. In: Krasner DL, Sibbald RG, Rodeheaver GT, editors. Epidermolysis bullosa (EB) is a genetic blistering skin disorder. Anand KJ. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). AMD, Advanced micro devices; MRSA, methicillin-resistant Staphylococcus aureus; NSAID, nonsteroidal anti-inflammatory drug; PC/C, percutaneous catheter; PHMB, polyhexamethylene biguanide; VRE, vancomycin resistant enterococcus. Background: Epidermolysis bullosa is a rare, often severe, genetic disorder characterized by fragility of the skin and mucous membranes. HHS Vulnerability Disclosure, Help However, topical antibiotics are not recommended to be used long-term. The recommendations were translated into a survey, and sent to other EB experts to generate consensus using an online-based modified Delphi method. Evidence-based medicine: how to practice and teach EBM. This is achieved by using dressings with occlusive, semi-occlusive, absorptive, hydrating, and hemostatic characteristics, depending on the wound characteristics and drainage (Tables IV to toVI).VI). Epidermolysis bullosa - Treatment Managing painful chronic wounds: the wound pain management model. Always remember when assessing a wound to observe not only the wound itself but also the surrounding tissue or the periwound area. Older patients tend to have more chronic ulcers that are critically colonized and infected and there is increased likelihood of colonization with antibiotic-resistant bacteria. However, all types of EB have one thing in common, and that is their skin fragility resulting in chronic wounds and erosions. [Accessed June 1, 2011]; Price P, Fogh K, Glynn C, Krasner DL, Osterbrink J, Sibbald RG. To date, there are no specific wound care guidelines or any evidence that address the wound care challenges of the EB population. Their wounds remain in the inflammatory phase of healing and often have difficulty progressing beyond that phase. Wound healing involves three phases of healing. Your message has been successfully sent to your colleague. WebBasic Care Tips for Epidermolysis Bullosa (EB): A Parents Guide By Lorraine Spaulding Edited by Anna L. Bruckner, M.D. The publisher's final edited version of this article is available at, Evaluate EB typespecific involvement and comorbidities, Monitor hemoglobin levels: ideally normal, minimally >80 g/L, Low hemoglobin consider: Iron supplementation, transfusions(s), Low albumin: eg, protein supplements, feeding tube, Address other specific subtype involvement, Superficial critical colonization and abnormal inflammation, Deep/surrounding tissue infection/generalized inflammation caregiver, Evaluate and manage EB typespecific involvement (simplex, junctional, dystrophic, Kindler syndrome) and comorbidities, Assess and address poor nutritional status, World Health Organization pain ladder for nociceptive pain, Neuropathic pain: consider tricyclics, gabapentin, pregabalin, Combine nonsedating H-1 antihistamine in morning with sedating preparations at night, Consider liquid quick-onset preparations for breakthrough (especially liquid formulations), Build confidence with patient and circle of care individuals, to increase adherence, Explore support from established EB centers, Longest length widest width at right angles, Saline, water, or acetic acid (0.25%-1.0%), Consider baths, whirlpool with salt, bleach, other antimicrobials. Bathing for individuals with epidermolysis bullosa. Over the past decade specialized EB clinics have opened in 16 countries worldwide, providing an interprofessional model of care with input from many allied health professionals (eg, nurses, physicians, surgeons, occupational therapists, physical therapists, social workers, dietitian, music therapists).53-67 Isolated cases can be overwhelming to health practitioners particularly when referral to an established EB center is not feasible. The pain can occur at rest from blisters and denuded skin, secondary infection, friction, and shearing with physical movements.24 Pain can also be exacerbated during dressing changes, bathing, and other activities of daily living. Is the wound extending and is there fragile bleeding tissue or wound pocketing? Edward Barrett (Canada), Anna Bruckner (United States), Maya El Hachem (Italy), Louise Fret-Lalonde (Canada), Gerry Kelly-Mancuso (United States), Michelle Lee (Canada), Andrew Lin (Canada), Anne Lucky (United States), Celia Moss (United Kingdom), Dedee Murrell (Australia), Annmarie Ormonde (Italy), Francis Pallison (Chile), Agnes Schwieger (Germany), Rosemarie Watson (Ireland), Karen Wiss (United States). Juan HY, Zhou AE, Hoegler KM, Khachemoune A. Arch Dermatol Res. Wound care has two goals, wound protection and wound healing. WebEpidermolysis bullosa (EB) is a severe blistering skin disorder. 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. Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Signs of local infection such as increased redness, local pain, odor, and exudate should be documented for each problematic wound. In S. Baranoski & E. Ayello (Eds.). There is no ideal management strategy for dealing with anemia in patients with EB. Shinkuma S, Natsuga K, Nishie W, Shimizu H. Epidermolysis bullosa in Japan. To optimize nutritional status, patients with severe forms of EB may require a gastrostomy tube. Wound care for these individuals is a big part of their daily routines, but it is important to remember that many of the individuals we work with are children. Your child will be seen by up to ten providers specializing in EB.
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