Bullous Pemphigoid

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Bullous pemphigoid facts

  • Bullous pemphigoid is an autoimmune skin disease characterized by blisters.
  • The cause of bullous pemphigoid is not known.
  • Bullous pemphigoid can involve the lining of the mouth and nose.
  • The diagnosis of bullous pemphigoid is ultimately confirmed by biopsy.
  • Treatment of bullous pemphigoid is individualized and depends on the location and severity of the disease.

What is bullous pemphigoid?

Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes (mucous membrane tissue) can be involved. The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer of the skin or mucous membranes. This layer of tissue is called the "basement membrane." These antibodies (immunoglobulins) bind to proteins in the basement membrane called hemidesmosomal BP antigens and this attracts cells of inflammation. The mucous membrane disease is also referred to separately as mucous membrane pemphigoid.

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Bullous Pemphigoid Symptoms & Signs

Blisters

Blisters can accompany some types of skin rashes and inflammatory conditions, including certain autoimmune diseases. Depending upon the cause of the blisters, blisters may occur singly or in groups. In contrast to abscesses and boils, which are collections of inflammatory fluid found deep in the tissues, blisters are found in the most superficial layer of skin.

What are bullous pemphigoid causes and risk factors?

A majority of those affected by bullous pemphigoid are 50 years of age or older. While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with a genetic predisposition to develop bullous pemphigoid.

Is bullous pemphigoid contagious?

No. Bullous pemphigoid cannot be transmitted from one person to another.

What types of specialists treat bullous pemphigoid?

Bullous pemphigoid is diagnosed and treated by dermatologists.

What are signs and symptoms of bullous pemphigoid?

Symptoms of bullous pemphigoid include intense itching and burning sensation of the skin. When the mucous membranes of the mouth are affected, it can cause pain, burning, peeling away of affected inner lining tissues, and sensitivity to acidic foods. Eating can be difficult, and involvement in the deeper areas of the throat can cause coughing. Involvement of the inner nose can cause nosebleeds. The disease typically worsens and improves over time.

How do health-care professionals diagnose bullous pemphigoid?

Bullous pemphigoid is often diagnosed by clinical examination and is confirmed by the results of a biopsy of involved tissue. The biopsy with traditional pathology evaluation can demonstrate inflammation of the affected skin levels. Additional testing of the biopsy specimen for antibody immune deposits can reveal the abnormal antibodies located in the basement membrane layer of skin or mucous membrane tissue. Blood testing for circulating basement membrane antibodies can also be helpful.

Bullous pemphigoid-like condition can sometimes be associated with other illnesses, including systemic lupus erythematosus (SLE or lupus) and cancer.

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What is the treatment for bullous pemphigoid?

Bullous pemphigoid can be chronic and mild without substantially disturbing the general health of affected individuals. It can also significantly affect daily lives in its more severe forms. Mild bullous pemphigoid can resolve with topical prescription corticosteroid creams but sometimes requires high doses of steroids taken internally. Severe bullous pemphigoid can also require immune-suppression drugs such as azathioprine (Imuran), mycophenolate (Cellcept), and methotrexate (Rheumatrex). The tetracycline derivatives such as minocycline or doxycycline have also been used as an option to reduce inflammation. Other treatments that have been used for severe disease include intravenous immunoglobulin infusions, typically given monthly.

Research has indicated that large quantities of high-potency topical corticosteroids applied to the body surface are safer in controlling localized bullous pemphigoid than oral corticosteroids. It was felt by the researchers that topical corticosteroids should now be the treatment of choice for bullous pemphigoid, particularly when the disease is not extensive.

Are there home remedies for bullous pemphigoid?

No. There are no home remedies for bullous pemphigoid other than preventing skin irritation and infection from scratching.

What is the prognosis of bullous pemphigoid?

The outlook for bullous pemphigoid is variable. As described above, the symptoms tend to wax and wane. In its most severe form it can be fatal without treatment, especially if involving the airways and pharynx.

Is it possible to prevent bullous pemphigoid?

No. There is no prevention for bullous pemphigoid.

REFERENCE:

Leiferman, Kristin M., et al. "Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid." UpToDate.com. Apr. 26, 2016. <http://www.uptodate.com/contents/epidemiology-and-pathogenesis-of-bullous-pemphigoid-and-mucous-membrane-pemphigoid>.

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Reviewed on 8/31/2016
References
REFERENCE:

Leiferman, Kristin M., et al. "Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid." UpToDate.com. Apr. 26, 2016. <http://www.uptodate.com/contents/epidemiology-and-pathogenesis-of-bullous-pemphigoid-and-mucous-membrane-pemphigoid>.

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