Pediatric Epidermolysis Bullosa Treatment & Management

Updated: Feb 07, 2017
  • Author: Surasak Puvabanditsin, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

The treatment of epidermolysis bullosa (EB) is primarily preventive and supportive. Once blistering has occurred, the blister should be punctured with a sterile needle or a blade. This may prevent the accumulation of fluid and pressure and may thus prevent the blister from extending. Complete and gentle drainage of the fluid, accomplished by leaving the roof of the blister intact and by covering the affected area with white petrolatum–impregnated gauze, helps to promote an environment most optimal for healing. If the blister repeatedly refills with fluid, it should be drained several times.

Open wounds should be covered with nonadherent dressings such as petrolatum-impregnated gauze, hydrogels, fenestrated silicone dressings or absorbent foam silicone dressings. Tape and any significant pressure to the skin must be avoided. Dressings can be held in place with rolled gauze (such as Kerlix), with tape applied only to the dressing itself or by stockinette (such as Surgifix or Spandage).

Some authors recommend daily application of polymyxin, bacitracin, or silver sulfadiazine topical ointments to treat open or partially healed wounds, which should be covered with petrolatum-impregnated gauze or nonadherent synthetic dressing. Gentamicin soaks (480 mg/L saline), acetic acid soaks (white vinegar), and the addition of small amounts of bleach to the bath water (eg, 1/8 cup per full tub) have been used to decrease the overgrowth of pseudomonas and staphylococcal organisms.

Corrective gene therapy is the ideal therapy for epidermolysis bullosa, but much more research is required before it can be developed and used in clinical practice. Cell-based therapies using fibroblasts and bone marrow cells have attracted considerable attention. [36]

Mental health

Chronic anticipatory anxiety is a common problem in children with epidermolysis bullosa, especially as it relates to frequent wound care changes. There may be a negative effect on development, mental health, education, family relationships, and, potentially, brain function. Approximately 80% of patients with epidermolysis bullosa experience psychiatric symptoms, particularly anxiety, depression, and behavioral problems. Psychiatric screening should be incorporated into the multidisciplinary approach to the patient with epidermolysis bullosa. [37]

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Surgical Care

Surgical procedures can correct the deformities of epidermolysis bullosa caused by repeated episodes of blistering and scarring of the hand. Ablative fractional laser surfacing has shown positive preliminary results in the management of dentition, pseudosyndactyly, and wounds. Unfortunately, most therapeutic improvements are often temporary because the recurring scars often require repeated procedures. [38] Esophageal dilatation or insertion of a gastrostomy tube may be required if esophageal strictures develop.

Patients with limited donor sites for a skin graft may need advanced therapy with bioengineered skin products. Several products (eg, composite cultured skin [CCS], Graftskin, Dermagraft) have been used in the treatment of patients with epidermolysis bullosa.

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Consultations

A multidisciplinary team should perform a review in patients with epidermolysis bullosa to address the following issues:

  • Regular skin care and dressing

  • Pain management

  • Nutrition

  • Monitoring of blood levels

  • Physiotherapy

  • Dental treatment

  • Occupational therapy

  • Videofluoroscopy, barium swallow study, or both

  • Echocardiography

When necessary, an ophthalmologist, a gastroenterologist, and plastic surgeon should be consulted.

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Diet

Patients with extensive cutaneous injury require increased energy (caloric) and protein intake. Vitamin and iron supplements are advised if nutritional compromise is present. If esophageal strictures develop, a blenderized diet is recommended.

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Activity

Patients should avoid unnecessary trauma to the skin. Wearing loose-fitting clothing and soft, well-ventilated leather shoes is advisable. Because increased ambient temperatures exacerbate most forms of epidermolysis bullosa, a cool environment is important.

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Prevention

A water mattress and soft fleece covering help to limit the friction and trauma that lead to blistering.

Avoid directly taping to the skin.

Keeping the palms and soles cool and dry during hotter weather helps to minimize blistering, especially in the Weber-Cockayne type of epidermolysis bullosa simplex (EBS).

The baby should not be placed in an incubator unless reasons of prematurity prevent open crib, as heat and humidity place the baby at risk for blistering. [39]

Suctioning, nasally and orally, should be avoided. If necessary, a soft catheter should be used with minimal suction pressure. [39]

The umbilical cord should be secured with ligature, not a plastic clamp that can cause friction. [39]

The name band should be placed on clothing and not in contact with skin. [39]

Electrodes should be lubricated, with smallest diameter, and secured with a nonadhesive dressing. [39]

Thick padding should be applied between patient and blood pressure cuff. [39]

Encourage skin-to-skin contact with caretakers. [39]

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Long-Term Monitoring

Preventive measures are the key to successful management of epidermolysis bullosa (EB). Epidermolysis bullosa requires cooperation of patients, parents, and physicians caring for the patient.

Medical surveillance for involvements of kidney, urinary tract, eye, and GI tract should become part of the routine evaluation of children and adults with epidermolysis bullosa.

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