Epidermolysis bullosa

Table of Contents


Expectations (prognosis)

The outlook depends on the severity of the illness. Mild forms of epidermolysis bullosa improve with age.

In the severe forms, scarring after blisters form may cause:

  • Contracture deformities (for example, at the fingers, elbows, and knees) and other deformities
  • Feeding and swallowing difficulties if the mouth and esophagus are involved
  • Fused fingers and toes
  • Restricted mobility from scarring

Very serious forms of epidermolysis bullosa have a very high mortality rate.

Secondary infection is common.


Complications
  • Anemia
  • Death (junctional epidermolysis bullosis has a high death rate, but other, milder forms may not reduce life expectancy at all)
  • Esophageal stricture
  • Eye disorders, including blindness
  • Infection, including sepsis
  • Loss of function in the hands and feet
  • Muscular dystrophy
  • Periodontal disease
  • Severe malnutrition caused by feeding difficulty, leading to failure to thrive
  • Squamous cell skin cancer

Calling your health care provider

If your infant has any blistering shortly after birth, call your health care provider. If you have a family history of epidermolysis bullosa and plan to have children, you may want to have genetic counseling.


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Review Date: 10/10/2010
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine. Also reviewed by Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)