Medication Summary

Treatment is directed at reducing the inflammatory response and autoantibody production. Although target-specific therapy is the "Holy Grail" for immunodermatologists, non–target-specific treatments are currently used. The most commonly used medications are anti-inflammatory agents (eg, corticosteroids, tetracyclines, dapsone) and immunosuppressants (eg, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide). Evidence suggests that strong topical corticosteroid treatment may achieve disease control while avoiding systemic adverse effects from systemic corticosteroids.^{[5, 4, 67, 68]}Omalizumab and dupilumab have been used as corticosteroid-sparing agents.^{[69, 70, 71]}

Initial treatment with doxycycline was found to be effective and was associated with a lower incidence of adverse effects compared with prednisone.^{[6, 7, 8]}

Proper treatments of bullous pemphigoid depend on the severity of the disease. For localized disease, topical steroids plus the systemic anti-inflammatory (tetracycline and nicotinamide) may be sufficient. Effects of monotherapy with nicotinamide are unknown. For more severe cases, systemic steroids along with immunosuppressives may be needed to control the disease. If the disease is difficult to control, consider treatment with an anti-CD20 antibody (rituximab), which is relatively specific in targeting the antibody-producing B cells.^{[72, 73, 74, 75, 76, 77, 78, 79, 80]}

Class Summary

These agents inhibit the inflammatory process by inhibiting specific cytokine production and vascular permeability. They may also stabilize granulocyte membranes and prevent release of key enzymes.

Prednisone (Deltasone)

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Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. When taken orally, it is used alone or in conjunction with other immunosuppressive agents for treating bullous pemphigoid.

Tetracycline (Sumycin)

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Although an antibiotic, tetracycline has proven effective in some cases of bullous pemphigoid either alone or in conjunction with niacinamide (2 g/d). Efficacy may be due to anti-inflammatory properties.

Clobetasol (Temovate)

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Clobetasol is a class I superpotent topical steroid; it suppresses mitosis and increases the synthesis of proteins that decrease inflammation and cause vasoconstriction. It is useful in treating localized bullous pemphigoid or in conjunction with low-dose systemic corticosteroids to treat the generalized disease.

Class Summary

For patients in whom steroids or other anti-inflammatory agents have not caused a response or for those unable to tolerate prednisone, immunosuppressants are useful adjuvants.

Azathioprine (Imuran)

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Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, RNA, and proteins. It may decrease the proliferation of lymphocytes. It is for use alone or in conjunction with prednisone.

Rituximab (Rituxan)

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Rituximab is a genetically engineered chimeric murine/human monoclonal antibody against human CD20, a molecule present in normal and malignant B lymphocytes. It is described in case reports as a promising biological treatment for B-lymphocyte–mediated diseases (eg, pemphigus vulgaris).

Questions

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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 Medication

Medication Summary

Anti-inflammatory agents