Última actualización:
10/21/2025
Años publicados: 1986, 1988, 1992, 1993, 2000, 2006, 2007, 2018
NORD gratefully acknowledges Meredith Gaufin, MD Candidate, University of Utah School of Medicine, and Christopher M. Hull, MD, Department of Dermatology University of Utah, for assistance in the preparation of this report.
Bullous pemphigoid (BP) is a rare, autoimmune, chronic skin disorder characterized by blistering, urticarial lesions (hives) and itching. Less commonly these blisters can involve the mucous membranes including the eyes, oral mucosa, esophagus and genital mucosa. It typically presents in older adults as a generalized intensely itchy blistering skin condition.
The first symptom of BP is usually redness and itching of the skin. Within weeks to months, thin-walled, tense blisters with clear fluid centers (bullae) appear on the arms and legs (flexor surfaces), in the armpits (axillae), on the abdomen, and/or in the skinfolds of the groin. Mucous membranes may also be involved but are less commonly seen than skin blisters.
The blisters are usually tense (tight), and contain clear or blood-tinged fluid; they do not rupture easily with gentle contact. If the blisters do rupture, pain may occur but healing is usually rapid and resolves without scarring.
Bullous pemphigoid usually itches and in its early phase urticarial (hives) lesions may be present before blisters are noted.
Bullous pemphigoid is an autoimmune blistering disease. Autoimmune disorders are generated when the body’s natural defenses against “foreign” or invading organisms, attack healthy tissue for unknown reasons. In BP, an autoantibody binds to a component of the skin that holds the dermis and epidermis together, causing separation of these two layers, thus forming a blister. The autoantibodies recognize components of the basement membrane zone called BP antigen 1 and 2 (and in some cases other basement membrane zone antigens). These proteins are part of complexes that hold the skin together, called hemidesmosomes, and provide structural support to the skin. When the body “attacks” these proteins, the skin becomes more fragile and the clinical manifestations of BP become apparent.
Certain drug reactions can produce skin lesions that are very similar to BP. It is essential to determine whether the patient’s symptoms are adverse reactions to the pharmaceuticals, or whether the blisters are the result of an autoimmune reaction.
BP is a rare disorder that affects males and females in equal numbers. This disorder primarily affects the elderly, with an average age around 80 years old. Rare cases have been reported in infants and adolescents.
Recently, researchers have learned of an association between BP and neurological disorders. It is reported that between a third to a half of all BP patients have neurological diseases, such as dementia, Parkinson’s disease, stroke, epilepsy, and multiple sclerosis. These conditions normally occur before the onset of BP.
Diagnosis is made based on a combination of clinical interpretation and laboratory findings.
Treatment
The goal of treatment for bullous pemphigoid (BP) is to control symptoms such as itching and blistering, prevent complications and minimize treatment-related side effects. The approach depends on the severity of the disease, the person’s age and other existing health conditions.
For people with limited disease, strong topical corticosteroids such as clobetasol are often used and can be very effective. These creams or ointments help calm the inflammation and reduce the formation of new blisters. In cases where applying topical treatments is not practical or if the disease is more widespread, oral corticosteroids like prednisone may be prescribed. These medications can bring the disease under control quickly, but they are associated with serious side effects such as increased risk of infections, high blood pressure, diabetes, bone loss and even higher mortality, especially in older adults. To reduce these risks, the lowest possible dose is used for the shortest amount of time needed to control symptoms.
To avoid prolonged steroid use, additional medications may be introduced early in treatment. These include doxycycline, which has anti-inflammatory properties, as well as other immune-modulating drugs such as mycophenolate mofetil, azathioprine and methotrexate. These are known as steroid-sparing agents and are often used to help reduce the dose of steroids needed to maintain disease control.
In more severe or treatment-resistant cases, newer therapies are showing promise. In 2025, the U.S. Food and Drug Administration (FDA) approved dupilumab (Dupixent) for the treatment of adults with BP. This is a major development, as dupilumab is the first biologic medication FDA-approved for this condition. It works by blocking specific parts of the immune system that drive inflammation, and studies have shown that it provides rapid relief from itching, controls disease activity in a high percentage of patients, and has an excellent safety profile. Dupilumab is especially useful for older adults or individuals who cannot tolerate traditional immunosuppressive treatments.
Other advanced therapies, such as rituximab and omalizumab, may be considered in complex or refractory cases. These treatments are typically reserved for people who do not respond to standard approaches.
Because BP is often a chronic condition, regular follow-up is important. Doctors monitor the severity of the disease, check for medication side effects and adjust treatment as needed. Special attention is given to blood pressure, blood sugar levels, bone health and the function of the adrenal glands, which can be affected by steroid use.
Each treatment plan is carefully tailored to the individual, with the goal of controlling the disease effectively while minimizing the impact of treatment on overall health. The approval of dupilumab represents an important advancement, offering a targeted and safer option for many patients with this condition.
Research is ongoing into additional therapies, including JAK inhibitors and other biologic agents, which may offer new options in the future with fewer risks.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For more information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruiting Office:
Tollfree: (800) 411-1222
TTY: (866) 411- 1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Bolognia, Jean L., MD; Schaffer, Julie V., MD; Cerroni, Lorenzo, MD. Dermatology, 4th ed. Elsevier; 2018.
Habif TP. Clinical Dermatology: a Color Guide to Diagnosis and Therapy, 6th ed. Elsevier; 2016.
REVIEW ARTICLES
Kershenovich R, Hodak E, Mimouni D. Diagnosis and classification of pemphigus and bullous pemphigoid. Autoimmun Rev. 2014 Apr-May;13(4-5):477-81.
Schmidt E, Zillikens D. Pemphigoid diseases. Lancet. 2013 Jan 26;381(9863):320-32.
Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Immunol Allergy Clin North Am. 2012 May;32(2):217-32.
Venning VA, Taghipour K, Mohd Mustapa MF, Highet AS, Kirtschig G. British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. Br J Dermatol. 2012 Dec;167(6):1200-14.
Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges. 2011 Oct;9(10):844-56; quiz 857.
Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database Syst Rev. 2010 Oct 6;(10):CD002292.
ARTICLES
Santoro C. FDA Expands Dupilumab Approval as First New Biologic for Bullous Pemphigoid. AJMC. June 20, 2025. https://www.ajmc.com/view/fda-expands-dupilumab-approval-as-first-new-biologic-for-bullous-pemphigoid Accessed Oct 20, 2025.
Jiang X, Chen L, Zhu Y, Zhan S, Jin N, Cheng H. Effective response to high-frequency and prolonged dupilumab therapy in severe refractory bullous pemphigoid: A case study. Hum Vaccin Immunother. 2025;21(1):2503521. doi:10.1080/21645515.2025.2503521
Borradori L, Van Beek N, Feliciani C, et al. Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2022;36(10):1689-1704. doi:10.1111/jdv.18220
Chalmers JR, Wojnarowska F, Kirtschig G, et al. A randomised controlled trial to compare the safety, effectiveness and cost-effectiveness of doxycycline (200 mg/day) with that of oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetracyclines (BLISTER) trial. Health Technol Assess. 2017;21(10):1-90. doi:10.3310/hta21100
INTERNET
Oakley A. Bullous pemphigoid. DermNet. Available at: https://dermnetnz.org/topics/bullous-pemphigoid Last updated February 2025. Accessed Oct 20, 2025.
Valdebran M. Bullous Pemphigoid. Medscape Reference. Updated: Sep 02, 2025. Available at: https://emedicine.medscape.com/article/1062391-overview Accessed Oct 20, 2025.
Baigrie D, Nookala V. Bullous Pemphigoid. [Updated 2023 Mar 2]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK535374/ Accessed Oct 20, 2025.
Kantor J. Bullous pemphigoid. MedlinePlus. Review date 5/31/2023. www.nlm.nih.gov/medlineplus/ency/article/000883.htm Accessed Oct 20, 2025.
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