Medical Care

As in other autoimmune bullous diseases, the goal of therapy is to decrease blister formation, to promote healing of blisters and erosions, and to determine the minimal dose of medication necessary to control the disease process. Therapy must be individualized for each patient, keeping in mind preexisting conditions and other patient-specific factors.

Consultations

Treatment of patients with bullous pemphigoid requires coordination of care between the dermatologist and the patient's primary care provider. Patients with oral disease may require an otolaryngologist and/or a dentist for evaluation and care. An ophthalmologist should evaluate patients with suspected ocular involvement and those requiring prolonged high-dose steroids.

Diet

The lesions may flare in patients with oral disease after eating hard and crunchy foods, such as chips, raw fruits, and vegetables.

For patients treated with systemic corticosteroid for longer than 1 month, a combined supplement of calcium and vitamin D should be instituted to prevent osteoporosis. The dosage and the frequency are stated in the recommendations established by the American College of Rheumatology Task Force in 1996.^[64]

In addition to calcium and vitamin D supplementation, patients on long-term treatment with systemic corticosteroids should be taking bisphosphonate, a specific inhibitor for osteoclast-mediated bone resorption (eg, alendronate).

Activity

Patients should be instructed to avoid direct physical trauma to their skin surfaces. For example, localized bullous pemphigoid has rarely been described peristomally.

Complications

Secondary infection may occur because of the presence of multiple erosions and immunosuppressants used to control the disease. These infections may be either systemic or localized to the skin. Cutaneous infection increases the risk of scarring and delays wound healing.

Malignancies due to immunosuppressants have been reported. Case-control series in patients with bullous pemphigoid have failed to detect an increased incidence of malignancy in patients with bullous pemphigoid when compared with age- and sex-matched controls

Bone marrow suppression may occur in patients receiving immunosuppressants.

Growth restriction may occur in children receiving systemic corticosteroids and immunosuppressants. Adrenal insufficiency may occur following prolonged use of glucocorticoids. Osteoporosis and bone fractures may result following the use of systemic corticosteroids. Associated neurodegenerative disease may be antibody-mediated.^[65]

There may be a higher incidence of squamous carcinoma and lymphoma in patients with pemphigoid.^[66]

Medication

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Media Gallery

Direct immunofluorescence study performed on a perilesional skin biopsy specimen from a patient with bullous pemphigoid detects a linear band of immunoglobulin G deposit along the dermoepidermal junction.
Indirect immunofluorescence study performed on salt-split normal human skin substrate with the serum from a patient with bullous pemphigoid detects immunoglobulin G class circulating autoantibodies that bind to the epidermal (roof) side of the skin basement membrane.

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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: American Medical Association, Illinois State Medical Society, Society for Investigative Dermatology, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Microcirculatory Society, Alpha Omega Alpha, American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Board of Dermatology<br/>Author for: Up-to-date.

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

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Federation for Medical Research, American Society for Clinical Investigation, Society for Investigative Dermatology

Disclosure: Received consulting fee from Novan for consulting; Received consulting fee from Stieffel, a GSK company for consulting; Received salary from Society for Investigative Dermatology for board membership.

Bullous Pemphigoid Treatment & Management

Medical Care

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Diet

Activity

Complications

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