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Last updated:
September 10, 2018
Years published: 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 therapy is to reduce symptoms (itching and formation of new blisters). Local skin care with antibacterial ointment to cover eroded blisters is recommended for all patients to decrease the likelihood of developing a secondary bacterial infection. High potency topical corticosteroid creams are typically used as first-line treatment. If topical application is not an appropriate option (for example, the patient is unable to apply ointment), oral corticosteroids, such as prednisone, may be considered. Since the cumulative effects of long-term corticosteroid therapy are undesirable, treatment aims at the lowest dose over the shortest period of time. A 2010 Cochrane review summarized the available treatment data for BP and concluded that very potent topical steroids are effective and safe treatments for BP, but their use in extensive disease may be limited by side effects and practical factors, such as the need to cover large areas with ointment. Initial doses of prednisolone greater than 0.75 mg per kg per day do not give additional benefit and could cause more adverse reactions. Doses lower than 0.75 mg per kg per day may be adequate to control disease and reduce likelihood and severity of potential side effects.
An anti-inflammatory antibiotic, doxycycline, has been studied to treat BP. The benefit of using non-steroidal agents is their superior safety profile. This is often combined with a B vitamin called niacinamide.
In more severe cases, patients will often be treated with medications that suppress the immune system. Examples of these medications include mycophenolate mofetil, azathioprine, methotrexate, rituximab and others.
Because many patients with BP are elderly, decisions about whether to treat with drugs that alter the immune system (such as corticosteroids) must be individualized because it may make patients more susceptible to infections.
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: prpl@cc.nih.gov
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.
INTERNET
Kantor J. Bullous pemphigoid. Medical Encyclopedia. MedlinePlus Last updated:02 May 2017. www.nlm.nih.gov/medlineplus/ency/article/000883.htm Accessed 07/9/2018.
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Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
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