Epidermolysis Bullosa Acquisita Treatment & Management

  • Author: Lawrence S Chan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 15, 2011
 

Medical Care

Patients with epidermolysis bullosa acquisita (EBA) may require therapy with oral corticosteroids and immunosuppressants.

  • For patients who are on long-term systemic corticosteroid treatment, daily calcium and vitamin D supplements are essential for reducing steroid-induced osteoporosis.
  • In addition, patients who are on long-term systemic corticosteroid treatment should take bisphosphonate, a class of specific inhibitor of osteoclast-mediated bone resorption (eg, alendronate [Fosamax]).
  • For patients who do not respond to oral corticosteroids and immunosuppressives, physicians could also consider using some other newer, but not-yet-proven therapeutic options, including intravenous immunoglobulin and intravenous infusion of anti-CD20 antibodies (rituximab).[23, 24, 25, 26, 27]
  • Additionally, see the complete clinical trial, Phase II Pilot Study of Extracorporeal Phototherapy for Epidermolysis Bullosa Acquisita.
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Consultations

Consultation and coordination of the patient's care with the patient's primary care provider are important for monitoring side effects of therapy and the overall management of the patients.

  • Because epidermolysis bullosa acquisita is strongly associated with inflammatory bowel disease, patients with epidermolysis bullosa acquisita should be asked about symptoms related to their GI tract. If there are any symptoms suggestive of inflammatory bowel disease, patients with epidermolysis bullosa acquisita should be referred to gastroenterologist for consultation.
  • In patients with oral involvement, coordination of care with the patient's dentist also is recommended. These patients may have difficulty with regular dental care and prophylaxis may induce exacerbations of oral blistering.
  • In patients with ocular involvement, consultation with an ophthalmologist is recommended.
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Diet

In patients with oral involvement, hard or brittle foods and foods with high acid content (eg, tomatoes, orange juice) should be avoided. Ingestion of these foods may traumatize mucosa and precipitate new lesions.

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Activity

Because epidermolysis bullosa acquisita primarily affects trauma-prone skin surfaces, patients are instructed to avoid direct physical trauma to their skin surfaces. When physical activities are planned, patients are instructed to use protective pads to cover their extensor skin surfaces. Additionally, gentle but thorough daily oral hygiene should be encouraged.

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Contributor Information and Disclosures
Author

Lawrence S Chan, MD  Dr Orville J Stone Professor of Dermatology, Head, Department of Dermatology, University of Illinois College of Medicine

Lawrence S Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Dermatological Association, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, Microcirculatory Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

David Woodley, MD  Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California

David Woodley, MD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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Direct immunofluorescence performed on perilesional skin biopsy specimen from a patient with epidermolysis bullosa acquisita detects a linear band of immunoglobulin G deposit along the dermoepidermal junction.
Indirect immunofluorescence performed on salt-split normal human skin substrate using serum from a patient affected with epidermolysis bullosa acquisita detects immunoglobulin G class circulating autoantibodies that bind to the dermal (base) side of the basement membrane.
 
 
 
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