Medical Care

Patients with epidermolysis bullosa acquisita (EBA) may require therapy with oral corticosteroids, anti-inflammatory agents, and immunosuppressants.^{[35, 36, 37, 38, 39]}

For patients who are on long-term systemic corticosteroid treatment, daily calcium, vitamin D, and potentially bisphosphonate therapy are important for reducing steroid-induced osteoporosis.

All forms of epidermolysis bullosa acquisita may generate significant quality-of-life considerations. The “autoimmune bullous disease quality of life” (ABQOL) and “treatment-based autoimmune bullous disease quality of life” (TABQOL) are two surveys that assess disease impact and track patient progress to treatment.^{[40, 41]}

Consultations

Coordination of care with the patient's primary care provider is important for monitoring adverse effects of therapy and the overall health of the patient.

Because epidermolysis bullosa acquisita (EBA) can involve esophageal mucosa and 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 esophageal lesions or inflammatory bowel disease, patients with epidermolysis bullosa acquisita should be referred to a gastroenterologist.

In patients with oral involvement, coordination of care with the patient's dentist is recommended.

In patients with ocular involvement, consultation with an ophthalmologist is recommended.

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.

Activity

Because epidermolysis bullosa acquisita (EBA) 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.

Complications

Infection is a possible consequence of open erosions and wounds in the setting of immunosuppression.

Malignancies may occur, secondary to chronic inflammation and immunosuppressive treatments.

Bone marrow suppression is possible, secondary to medication.

Growth retardation may occur, secondary to medications used during childhood.

Complications secondary to prednisone treatment for epidermolysis bullosa acquisita (EBA) include adrenal insufficiency, osteoporosis, and cataracts.

The chronic inflammation and scar formation on patients' extensor surfaces can severely hinder the daily activities of patients.^[42]The scarring nature of epidermolysis bullosa acquisita can lead to nail destruction and hair loss.

Oropharyngeal mucous membrane involvement can lead to periodontal disease, oral mucosal erosions, and esophageal strictures, which limit oral intake, and supraglottic stenosis with airway compromise. Ocular involvement can lead to obstruction of nasolacrimal ducts, conjunctival scarring, and blindness.

Prevention

It is important to monitor and prevent complications of prolonged systemic corticosteroid therapy. The American College of Rheumatology 2010 guidelines for prevention and treatment of glucocorticoid-induced osteoporosis recommend calcium and vitamin D supplementation, lifestyle modification counseling, and consideration of bisphosphonate therapy.^[43]The indication for bisphosphonate therapy is based on risk stratification considering age, steroid dose, duration of treatment, and Fracture Risk Assessment Tool (FRAX) score. Prolonged corticosteroid treatment is also a risk factor for cataracts, and patients should be screened by an ophthalmologist.

Long-Term Monitoring

Patients with epidermolysis bullosa acquisita (EBA) should be monitored regularly by physicians with experience in treating autoimmune skin disease. During the active disease stage, patients should be monitored by their physicians on a monthly basis. When in remission, patients should be monitored by their physicians annually.

Medication

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Media Gallery

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.

of 2

Author

Jacob Reinhart, MD Undersea Medical Officer, United States Navy

Disclosure: Nothing to disclose.

Coauthor(s)

Kristina Marie Dela Rosa, MD Dermatologist, Insight Dermatology, San Diego, CA

Kristina Marie Dela Rosa, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Jaggi Rao, MD, FRCPC Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, Director of Dermatology Residency Program, University of Alberta Faculty of Medicine and Dentistry, Canada

Jaggi Rao, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Dermatology Association, Canadian Medical Association, Canadian Medical Protective Association, Pacific Dermatologic Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Ponciano D Cruz, Jr, MD Professor and Vice-Chair, Paul R Bergstresser Chair, 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: Received consulting fee from RCTS for independent contractor; Received honoraria from Mary Kay Cosmetics for consulting; Received grant/research funds from Galderma for principal investigator.

Daniel L Croom, MD Undersea Medical Officer, Naval Special Warfare Center

Disclosure: Nothing to disclose.

Acknowledgements

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.

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.

The view(s) expressed herein are those of the authors and do not reflect the official policy or position of Naval Special Warfare Center, the U.S. Navy Medical Department, the U.S. Navy Office of the Surgeon General, the Department of the Navy, Department of Defense, or the U.S. Government.