Bullous Pemphigoid

Bullous Pemphigoid

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Bullous Pemphigoid is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Benign Pemphigus
  • Old Age Pemphigus
  • Parapemphigus
  • Pemphigoid
  • Senile Dermatitis Herpetiformis

Disorder Subdivisions

  • None

General Discussion

Bullous Pemphigoid is a rare, autoimmune, chronic skin disorder characterized by blistering. This disorder occurs most frequently in elderly people. Generalized blistering occurs in and under the upper layers of the skin and usually subsides spontaneously within several months or years. However, symptoms may recur. In some rare cases of Bullous Pemphigoid, complications such as pneumonia may develop.

Symptoms

The first symptom of bullous pemphigoid (BP) is usually redness of the skin surrounding a lesion, scar, and/or the navel. Within weeks, thin walled blisters with clear fluid centers (bullae) appear on the undersurfaces of the arms and legs (flexor surfaces), in the armpits (axillae), on the abdomen, and/or around the groin. These blisters are create red, itchy patches. Unlike pemphigus, BP blisters usually do not affect the mucous membrane lining the mouth; if they do they heal rapidly.



The blisters are usually hard and tight, and contain clear or blood-tinged fluid; they do not rupture easily. If the blisters do rupture, pain may occur but healing is usually rapid.



Bullous pemphigoid usually itches and in its early phase, itching and hive-like patches may be the only symptoms.



After a few months, the symptoms of bullous pemphigoid often disappear spontaneously, but they may recur for no apparent reason.

Causes

The exact cause of bullous pemphigoid is not known; increasingly, however, it is believed to be an autoimmune disorder. Autoimmune disorders are generated when the body's natural defenses (e.g., the immune system with its antibodies) against "foreign" or invading organisms, attack healthy tissue for unknown reasons.



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.



In 1990 it was determined that the gene for Bullous Pemphigoid is located on chromosome 6 and has been mapped to 6p12-p11.

Affected Populations

Bullous pemphigoid is a rare disorder that affects males and females in equal numbers. This disorder primarily affect the elderly.

Standard Therapies

Doctors with some experience with BP can usually recognize the blisters that characterize the disorder. Others will study one or more skin biopsies to determine the layer(s) of skin involved and the appearance of the antibody deposits between the outer and inner layers of the skin.



Treatment

Mild cases of bullous pemphigoid may not require any treatment and remission may take place within months or years. More serious bouts of the disorder are treated with steroids, usually prednisone over a longish period of time. Since the cumulative effects of long-term steroid therapy are undesirable treatment aims at the lowest dose over the shortest period of time.



Other medications have been tried with only fair results. These include: tetracycline antibiotics, dapsone, methotrexate, and high dose immuno-globulin.



Corticosteroid drugs are given to people with bullous pemphigoid to help reduce the number of blisters. The dosage of these immune suppressant drugs is lower for people with BP than the dosage prescribed to treat pemphigus. Prednisone can be discontinued in approximately 50 percent of cases of BP because the patients eventually go into remission. The remainder of patients may require maintenance therapy. Because many people with Bullous Pemphigoid are elderly, decisions about whether to treat with drugs that alter the immune system (such as corticosteroids) must be individualized because it may make fragile patients more susceptible to infections.

Investigational Therapies

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 web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources contact:

www.centerwatch.com



Duke University Medical Center in collaboration with Genentech, a biotechnology company, is sponsoring a Phase I and Phase II clinical trial to measure the safety and efficacy of treating bullous pemphigoid with rituximab, a monoclonal antibody that has been used to treat some cancers.

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:830.



Kasper, DL, Fauci AS, Longo DL, et al. Eds. Harrison's Principles of Internal Medicine. 16th ed. McGraw-Hill Companies. New York, NY; 2005:303, 313.



James WD, Berger T, Elston DM. Eds. Andrew's Diseases of the Skin: Clinical Dermatology. 10th ed. Saunders Elsevier. Philadelphia, PA. 2006:466-69.



REVIEW ARTICLES

Schmidt E, Hunzelmann N, Zillikens D, Brocker EB, Goebeler M. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol. 2006;31:503-08.



McCuin JB, Hanlon T, Mutasim DF. Autoimmune bullous diseases: diagnosis and management. Dermatol Nurs. 2006;18:20-25.



Ahmed AR. Uses of intravenous immunoglobulin therapy in autoimmune blistering diseases. Int Immunopharmacol. 2006;6:557-78.



Joly P, Fontaine J, Roujeau JC. The role of topical steroids in bullous pemphigoid in the elderly. Drugs Aging. 2005;22:571-76.



Khumalo N. Kirtschig G, Middleton P, Hollis S, Wojnarowska F, Murrell D. Interventions for bullous pemphigoid. Cochrane Database Syst Rev. 2005 Jul 20;CD002292



FROM THE INTERNET

McKusick VA, Ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Dystonin; DST. Entry Number; 113810: Last Edit Date; 7/21/2006.



Kantor J. Bullous pemphigoid. Medical Encyclopedia. MedlinePlus Last updated:04 October 2006. 3pp.

www.nlm.nih.gov/medlineplus/ency/article/000883.htm

Accessed 11/7/2006

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



National Cancer Institute

6116 Executive Blvd Suite 300

Bethesda, MD 20892-8322

USA

Tel: (301)435-3848

Tel: (800)422-6237

TDD: (800)332-8615

Email: cancergovstaff@mail.nih.gov

Internet: http://www.cancer.gov



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



Australasian Blistering Diseases Foundation

St. George Hospital,

Department of Dermatology

Gray Street

Kogarah

Sydney, NSW 2217

Australia

Tel: 0291132088

Fax: 0291132886

Email: info@blisters.org.au

Internet: http://www.blisters.org.au/BDHome.html



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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