Epidermolysis Bullosa Commands Aggressive Tx

PHILADELPHIA — When it comes to the management of epidermolysis bullosa, patient preference for bathing and dressings is important, follow-up is key to the treatment of associated anemia, and lesions suspicious for squamous cell carcinoma should undergo early biopsy and be treated aggressively, according to Dr. Dedee F. Murrell, an expert in the care of patients with this painful condition.

Dr. Murrell focused on these areas during her presentation on the management of epidermolysis bullosa (EB) at the annual meeting of the Society for Pediatric Dermatology. In Australia, she is the chair of the dermatology department at St. George Hospital in Kogarah, New South Wales, and a consultant dermatologist for EB at Sydney Children's Hospital in Randwick, New South Wales.

Dressings and Bathing

There is no consensus on how to bathe infants with EB. "There's still debate about whether you should immerse the baby completely in the bath or change the dressings limb by limb," she said. Although the limb-by-limb method may be easier to teach parents, one drawback is that the baby never learns to be comfortable with the bath setting.

"We've found it very useful in Sydney to use heated-up normal saline baths for these newborns. It makes [the water] comfortable and doesn't sting their skin when they go in the bath," she said. Some older children move on to showering, but most do not like the feeling of water splashing on their skin and prefer bathing.

"We tend to recommend saline baths for bigger patients," she noted. Patients are advised to add 1 kg of swimming pool salts to the bath water. Swimming pool salts are cheaper to use than table salt. Sometimes patients are advised to add a half cup of bleach to reduce colonization of the skin.

When their dressings are changed, it is important for patients or their caretakers to pop the EB blisters to reduce the extension of the blisters. Dr. Murrell and her colleagues usually recommend using a large-bore (such as a 19-gauge) sterile needle, which is inserted parallel to the skin surface so that it doesn't hurt the underlying skin. It's better to rupture the blister in the lowest part, so that it does not reaccumulate fluid when the patient stands. Next, press gauze over the blister to drain and collect the fluid. "Some patients prefer scissors and others even use scalpels, but I think there are [fewer accidents] with the needles," she said.

As for the optimal dressing, "I believe, personally, that the ideal dressings for EB are still to be found," said Dr. Murrell.

For now, nonstick, silicone-based dressings appear to be the best option. However, most of these require secondary dressings of variable thickness to absorb the exudate, depending on the wound. Some products are available with combined primary and secondary dressings.

Most dressings need bandages to hold them in place. "You need special training and experience not to wrap them too tightly and cause new blistering by too much pressure at the edge of these dressings," she said. For children—especially those with recessive dystrophic EB (RDEB), whose fingers are eroded—parents need to be taught how to wrap the fingers individually. Parents also need to be taught how to lift children with EB to avoid causing more blisters.

Silver-impregnated dressings are also an option, particularly the newer ones that are silicone based. However, these cannot be used continuously for a long period of time because of the possibility of argyria.

Anemia

"It turns out that it's not just iron-deficiency anemia these patients have, but anemia of chronic disease as well," said Dr. Murrell. Patients with EB have persistently open wounds and repeated infections. As a defense mechanism, cytokines reduce the availability of iron so that infectious organisms cannot use it. Reduced iron availability, coupled with loss of iron through the skin, leads to low serum iron and altered iron metabolism. The inflammatory response in these patients also leads to blunted erythropoietin response. Cytokines, such as interferon-gamma and tumor necrosis factor–alpha, interfere with bone marrow response to erythropoietin.

"Malnutrition doesn't assist us in the management of anemia either. That's another problem these patients face," said Dr. Murrell. Patients with EB have low protein and albumin levels, and may also have low vitamin C levels. Protein has been shown in animals to lower the body's production of erythropoietin. In addition, iron deficiency can cause mucosal changes that reduce the intestine's ability to absorb iron. As a result, patients with EB need parenteral iron. Intravenous parenteral iron is preferable because intramuscular injection is painful and these patients often have low muscle mass.