Particular body sites may present difficulties when faced with selecting a skin care or dressing regime. For example, digits are difficult to dress. Similarly, flexural sites such as the axillae and groin present problems for dressing retention. A variety of soft, conformable strip or tubular bandages in appropriate sizes may help to some extent, although even these may move and cause additional shearing stress to the skin, especially when dressings become soaked with heavy exudate. Perianal or vulvar wounds are particularly challenging to manage; using a barrier or lubricating preparation, as a cream, ointment, or spray, may help reduce adherence to other skin sites and clothing, as well as protect the skin from irritation and bacterial contamination. The addition of a topical local anesthetic, such as 2 % lignocaine gel, to ulcerated areas around the perineum, may be useful prior to passing urine or feces. The diaper area in infants and small children is another difficult site to dress due to frequent soiling by urine and feces. Using soft hydrogel dressings with or without copious barrier preparations such as zinc oxide may mitigate some of the trauma to this area. The ears and scalp are other difficult areas to dress, particularly in babies with JEB-Herlitz or older children or adults with RDEB with chronic wounds. A variety of primary dressings may be used, but they may prove difficult to keep in place. Conformable bandages or tubular bandages made into a snug-fitting cap may be helpful in this situation.

In addition to issues dressing the diaper area, infants and children with EB may present special challenges with dressings [13]. It is common for children to go through times as toddlers where they will remove any dressings that are applied to the skin, with the potential for causing more skin damage. As babies become more mobile, particularly those with DEB, bony prominences such as the knees and ankles may need protection as the child starts to crawl and walk. Some of the silicone dressings are ideally suited to provide atraumatic coverage but stay in place at these sites.

Children may have difficulty cooperating with dressing changes. Relaxation, biofeedback, distraction, and guided imagery are non-pharmacologic methods that may help children better tolerate their dressing changes [14, 15].

EB patients frequently have open areas of skin at different body sites, and individual wounds themselves may be chronic. As a result, wounds are usually colonized with bacteria (most commonly Staphylococcus aureus, Streptococcal species, or Pseudomonas aeruginosa). If the balance of host immunity versus virulence of the colonizing organism shifts, however, wounds may become critically colonized, whereby they stop healing and become “stuck [16].” With a further shift of the balance, wounds may become frankly infected, showing the characteristic features of further breakdown, increasing pain, redness, and exudate. While infection of an EB wound often prompts treatment with systemic antibiotics, some milder infections or critical colonization may be readily amenable to topical measures to reduce bacterial load [10]. In general, antimicrobial preparations such as chlorhexidine, benzalkonium chloride, lipid-stabilized hydrogen peroxide, and octenisan are preferred over topical antibiotics to which bacteria may become resistant. A number of antimicrobial dressings which fulfill the general requirements for EB wounds are also available which may be helpful in this situation, including polyhexamethylene biguanide (PHMB), iodine, medical-grade honey, and silver dressings [17, 18]. Caution should be exercised, however, with longer-term use of silver sulfadiazine cream or silver-containing dressings since absorption may lead to elevated serum silver levels [10].

Pain is a universal feature of EB (see below), although it may vary considerably depending on the extent of wounds and the presence of associated infection and inflammation [19]. Care should be given to addressing underlying background pain from chronic wounds, versus more acute wound pain and procedural pain that may be associated with bathing or dressing changes. It should be remembered that even babies and small children experience considerable pain from EB wounds, and these needs should be addressed, even with strong opioid analgesia if required. Generally speaking, the World Health Organization pain ladder approach should be adopted, with a stepwise approach using acetaminophen with or without NSAIDs for mild pain, adding mild opiates for moderate pain, or stronger opiates such as morphine for more severe pain [20].

In addition to systemic agents, topical analgesics may be helpful for a limited number of more painful wounds. These include dressings that provide slow release of ibuprofen and the use of topical morphine (10 mg morphine mixed in 15 g of hydrogel) applied directly to the painful wound at each dressing change [21].

Itch is a troublesome problem for many people with EB, either from intact skin or from wounds as they are healing. Topical measures such as cooling the skin or using soothing gel-based dressings may be helpful, but often a systemic approach may be necessary. Antihistamines (both sedating and nonsedating) or doxepin may provide some relief. Some patients may derive benefit from agents used for neuropathic pain or from ondansetron [19].

The high incidence of developing squamous cell carcinoma (SCC) in EB, notably severe generalized recessive dystrophic EB, is an additional challenge for EB wound care. In extensive primary SCCs, particularly in advanced disease where excision is not an option, and cutaneous or regional nodal metastases, wounds are often highly exudative and may also be friable, bleeding easily at dressing changes. The use of dressings designed to absorb exudate and hold it away from the surrounding skin may be particularly helpful, as may alginate dressings to reduce or stop bleeding. Malignant wounds may also pose problems of odor, and in this situation charcoal-containing or honey dressings may be useful [22].

In patients with advanced disease toward the end of life, it may be necessary to adopt a pragmatic approach to doing wound dressings. If dressing changes are painful and distressing for the patient, it may be preferable to reduce the frequency of dressing changes or alter their timing and accept that there may be deterioration in the wounds as a result.

Both breastfeeding and bottle feeding are possible in neonates and infants with EB. Petroleum jelly or a similar ointment can be used to reduce friction on the lips and facial skin, preventing new erosions. If there is difficulty with feeding due to erosions of the oral mucosa or a weak suck, a specialized feeder, such as the Haberman Feeder, can be helpful.

Nasogastric (NG) feeds are not routinely recommended, but can be used in rare circumstances. The tube can induce internal friction, irritating the nostrils, hypopharynx, and esophagus. Patients with RDEB are at highest risk and NG tubes should be avoided if possible in RDEB. NG feeds may be used for particular cases as a short-term treatment in other forms of EB. The tubes have to be secured using nonadhesive dressings.

Gastrostomy tube (GT) feeding is increasingly becoming the method of choice for children requiring long-term nutritional supplementation. Growth retardation often occurs early in the course of severe generalized RDEB, even despite regular guidance from a dietician. At least 50 % of the patients with severe generalized RDEB might require nutrition via a GT [33], and the use of GT feeds has become common in most of EB reference centers worldwide.

We have reported our long-term experience of GT feeding in young patients with severe generalized RDEB [28]. At GT feeding onset, mean body weight Z-score was —2.3 ± 1.0, mean height Z-score was −1.1 ± 1.1, mean weight-for-height was 81 ± 11 %, and mean height-for-age was 95 ± 4 %. GT feeds provided 91 ± 29 % and 205 ± 100 % of the recommended dietary allowance (RDA) for energy and proteins, respectively. Mean gain in weight Z-score was 1.2 ± 1.3, height catch-up was 1.0 ± 1.1, weight-for-height reached 92 ± 15 %, and height-for-age was 98 ± 5 %.