eMedicine Specialties > Pediatrics: General Medicine > Dermatology

Epidermolysis Bullosa: Treatment & Medication

Author: Surasak Puvabanditsin, MD, Assistant Professor of Pediatrics, UMDNJ-New Jersey Medical School; Associate Professor of Pediatrics, St George's University School of Medicine, Grenada; Associate Professor of Pediatrics, Seton Hall University School of Graduate Medical Education
Coauthor(s): Erik Brandsma, MD, Fellow, Division of Neonatology, Department of Peditrics, UMDNJ-Robert Wood Johnson Medical School; Nisha Patel, MD, Resident Physician, Westchester Medical Center, Maria Ferrari Children's Hospital; Rungtiwa Weerasethsiri, MD, Staff Physician, Department of Pediatrics, UMDNJ-New Jersey Medical School; Alexis A D'Elia, MD, Resident Physician, Department of Internal Medicine, Winthrop University Hospital
Contributor Information and Disclosures

Updated: Jul 30, 2009

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.

Surgical Care

  • Surgical procedures can correct the deformities of epidermolysis bullosa caused by repeated episodes of blistering and scarring of the hand. 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.

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.

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.

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.

Medication

  • Drug therapy is not currently a component in the standard of care for epidermolysis bullosa (EB).
  • See Treatment.

More on Epidermolysis Bullosa

Overview: Epidermolysis Bullosa
Differential Diagnoses & Workup: Epidermolysis Bullosa
Treatment & Medication: Epidermolysis Bullosa
Follow-up: Epidermolysis Bullosa
Multimedia: Epidermolysis Bullosa
References
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References

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Further Reading

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Keywords

epidermolysis bullosa, EB, bullous disorder, blister, blistering, skin erosion, epidermolysis bullosa simplex, EBS, junctional epidermolysis bullosa, JEB, dystrophic epidermolysis bullosa, DEB, Herlitz junctional EB, pyloric atresia, ectodermal dysplasia, Dowling-Meara syndrome, Köbner syndrome, Weber-Cockayne syndrome, Kallin syndrome, Mendes de Costa syndrome, Ogna syndrome, Carmi syndrome, Cockayne-Touraine syndrome, Pasini syndrome, Hallopeau-Siemens syndrome, Shabbir syndrome, Kindler syndrome, muscular dystrophy, failure to thrive, squamous cell carcinoma, SCC, treatment, diagnosis

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Contributor Information and Disclosures

Author

Surasak Puvabanditsin, MD, Assistant Professor of Pediatrics, UMDNJ-New Jersey Medical School; Associate Professor of Pediatrics, St George's University School of Medicine, Grenada; Associate Professor of Pediatrics, Seton Hall University School of Graduate Medical Education
Surasak Puvabanditsin, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Coauthor(s)

Erik Brandsma, MD, Fellow, Division of Neonatology, Department of Peditrics, UMDNJ-Robert Wood Johnson Medical School
Disclosure: Nothing to disclose.

Nisha Patel, MD, Resident Physician, Westchester Medical Center, Maria Ferrari Children's Hospital
Nisha Patel, MD is a member of the following medical societies: American Medical Student Association/Foundation and Phi Beta Kappa
Disclosure: Nothing to disclose.

Rungtiwa Weerasethsiri, MD, Staff Physician, Department of Pediatrics, UMDNJ-New Jersey Medical School
Rungtiwa Weerasethsiri, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Alexis A D'Elia, MD, Resident Physician, Department of Internal Medicine, Winthrop University Hospital
Alexis A D'Elia, MD is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Medical Editor

Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program of the Richmond SPCA; Pediatric Emergency Physician, Emergency Consultants Inc, Chippenham Medical Center
Kevin P Connelly, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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