Epidermolysis Bullosa Acquisita Treatment & Management
- Author: Daniel L Croom, MD; Chief Editor: Dirk M Elston, MD more...
Patients with epidermolysis bullosa acquisita (EBA) may require therapy with oral corticosteroids, anti-inflammatory agents, and immunosuppressants.[26, 27, 28, 29, 30]
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.
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.
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.
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.
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. 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.
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. 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.
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.
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