History

The onset of bullous pemphigoid may be either subacute or acute, with widespread, tense blisters. Significant pruritus is frequently present and may be the only manifestation of the disease, especially in older patients.^[56]In some patients, the blisters arise after persistent urticarial lesions.

Bullous pemphigoid has been reported following several nonbullous, chronic, inflammatory skin diseases, such as lichen planus and psoriasis.

Bullous pemphigoid has been reported to be precipitated by ultraviolet irradiation, x-ray therapy, and exposure to some drugs.

Drugs associated with bullous pemphigoid include furosemide, ibuprofen and other nonsteroidal anti-inflammatory agents, captopril, penicillamine, and antibiotics.

Bullous pemphigoid has been reported to develop shortly after vaccination, particularly in children.^[57]

Physical Examination

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

Generalized bullous form of bullous pemphigoid: The generalized bullous form is the most common presentation. Tense bullae arise on any part of the skin surface, with a predilection on the flexural areas of the skin. Oral and ocular mucosa involvement rarely occurs and, when seen, is of minor clinical significance. The bullae can occur on normal-appearing, as well as erythematous, skin surfaces. The bullae usually heal without scarring or milia formation.
Vesicular form of bullous pemphigoid: The vesicular form is less common. It manifests as groups of small, tense blisters, often on an urticarial or erythematous base.
Vegetative form of bullous pemphigoid: The vegetative form is very uncommon, with vegetating plaques in intertriginous areas of the skin, such as the axillae, the neck, the groin, and inframammary areas. This form of bullous pemphigoid closely resembles pemphigus vegetans.
Generalized erythroderma form of bullous pemphigoid: This rare presentation can resemble psoriasis, generalized atopic dermatitis, or other skin conditions characterized by an exfoliative erythroderma. Patients with this variant may develop vesicles or bullae.
Urticarial form of bullous pemphigoid: Some patients with bullous pemphigoid initially present with persistent urticarial lesions that subsequently convert to bullous eruptions. In some patients, urticarial lesions are the sole manifestations of the disease. In a report from China, about 30% of bullous pemphigoid patients were recorded with initial nonbullous forms of clinical presentation, such as erythema, papules, and plaques.^[58]
Nodular form of bullous pemphigoid: This rare form, termed pemphigoid nodularis, has clinical features that resemble prurigo nodularis, with blisters arising on normal-appearing or nodular lesional skin.
Acral form of bullous pemphigoid: In childhood-onset bullous pemphigoid associated with vaccination, the bullous lesions predominantly affect the palms, the soles, and the face.
Infant form of bullous pemphigoid: For the infant patients affected by bullous pemphigoid, the blisters tend to occur frequently on the palms, soles, and face, affecting the genital areas rarely. Sixty percent of these infant patients have generalized blisters.^[1]

Differential Diagnoses

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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 Clinical Presentation

History

Physical Examination

References

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