Practice Essentials

Bullous pemphigoid is a chronic, autoimmune, subepidermal, blistering skin disease that rarely involves mucous membranes. Bullous pemphigoid is characterized by the presence of immunoglobulin G (IgG) autoantibodies specific for the hemidesmosomal bullous pemphigoid antigens (BPAgs) BP230 (BPAg1) and BP180 (BPAg2). Occasionally, sublamina densa deposits are noted, related to anti-p200 antibody. If untreated, bullous pemphigoid can persist for months or years, with periods of spontaneous remissions and exacerbations. It can be fatal, particularly in patients who are debilitated.

Signs and symptoms

Bullous pemphigoid may present with several distinct clinical presentations, as follows:

Generalized bullous form - The most common presentation; tense bullae arise on any part of the skin surface, with a predilection for the flexural areas of the skin
Vesicular form - Less common than the generalized bullous type; manifests as groups of small, tense blisters, often on an urticarial or erythematous base
Vegetative form - Very uncommon, with vegetating plaques in intertriginous areas of the skin, such as the axillae, neck, groin, and inframammary areas
Generalized erythroderma form - This rare presentation can resemble psoriasis, generalized atopic dermatitis, or other skin conditions characterized by an exfoliative erythroderma
Urticarial form - Some patients with bullous pemphigoid initially present with persistent urticarial lesions that subsequently convert to bullous eruptions; in some patients, urticarial lesions are

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Media Gallery

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

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#author-disclosures{display:block}#author-disclosures.modal-layer{position:relative}#author-disclosures .modal-content{max-height:none}#author-disclosures .close-modal{display:none}

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.

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