Synonyms

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

Disorder Subdivisions

• None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

• Pemphigus
• Erythema multiforme
• Pemphigoid, benign mucosal
• Dermatitis herpetiformis
• Epidermolytic hyperkerotosis
• Epidermolysis bullosa
• Epidermolysis bullosa acquista

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Bullous pemphigoid. Comparisons may be useful for a differential diagnosis:



Pemphigus is an uncommon, sometimes life-threatening disease in which blisters of varying sizes break out on the skin, the lining of the mouth, the vagina, the thin covering of the penis and other mucous membranes (thin moist layers that line the body's internal surfaces). The location and type of blister may vary according to the type of Pemphigus. If left untreated, Pemphigus can be a serious illness. Symptoms of this disorder include the appearance of soft blisters in the outer layers of the skin especially on the neck, scalp, mucous membranes, and/or the underarm and groin areas. (For more information on this disorder, choose "Pemphigus" as your search term on the Rare Disease Database.)



Erythema multiforme is an allergenic, inflammatory skin disorder characterized also by lesions on the skin and/or mucous membranes. The early symptoms of this disorder may include red, elevated spots (erythematous macules or papules) on the skin that may have fluid filled centers and that eventually grow into larger blisters. Affected areas generally include the hands, forearms, feet, and/or mucous membranes of the mouth, nose, and/or genitals. The skin lesions and blisters caused by Erythema Multiforme generally appear on both sides of the body and tend to heal in approximately 2 to 6 weeks. Erythema Multiforme may also cause fever, joint pain, cough, and a sore throat. (For more information on this disorder, choose "Erythema Multiforme" as your search term in the Rare Disease Database.)



Benign mucosal pemphigoid is a rare chronic disease characterized by blistering and scarring of the mucous membranes particularly in the mouth and membranes that surround the eyes (conjunctiva). Initial symptoms include redness and inflammation of these areas and scarring may occur on the membranes of the eyes. Blisters may also develop in the mucous membranes of the pharynx, esophagus, nose, urethra and/or vulva. (For more information on this disorder, choose "Benign Mucosal Pemphigoid" as your search term on the Rare Disease Database.



DermatitishHerpetiformis is a rare chronic skin disorder that is characterized by groups of severely itching blisters and elevated lesions. This disorder is often associated with a sensitivity to foods that contain gluten (gluten-sensitive enteropathy). The onset of Dermatitis Herpetiformis is generally slow in adults; children may also be affected. Small, discrete blisters and itchy smooth skin lesions that look like hives appear on the head, elbows, knees, lower back, and/or buttocks. Itching and burning may be almost intolerable, and the need to scratch may be overwhelming. (For more information on this disorder, choose "Dermatitis Herpetiformis" as your search term in the Rare Disease Database.)



Epidermolytic hyperkeratosis (bullous type) is a rare hereditary skin disorder characterized by the overgrowth of skin (hyperkeratosis) and abnormal redness of the skin (erythroderma). The symptoms of this disorder are present at birth and may range from mild to severe. The skin may appear to have warts or blisters, and to be thick over most of the body, particularly in the skin creases over joints. Epidermolytic Hyperkeratosis can be detected before birth by amniocentesis (microscopic examination of the fluid that surrounds the developing fetus). (For more information on this disorder, choose "Epidermolytic Hyperkeratosis" as your search term in the Rare Disease Database.)



Epidermolysis bullosa refers to a group of rare skin diseases characterized by fragile skin, blisters, and small fluid-filled lesions that develop following minor trauma to the skin. The mucous membranes are also involved in some forms of Epidermolysis Bullosa. Healing may be impaired. Blisters may leave multiple scars and/or damage the underlying muscle tissue. Most types of Epidermolysis Bullosa are inherited, and they usually first appear during childhood. (For more information on these disorders, choose "Epidermolysis Bullosa" as your search term in the Rare Disease Database.)



Epidermolysis bullosa acquista is a rare autoimmune disorder of the skin that typically affects middle-aged and elderly people. Trauma to the skin can cause blisters on the elbows, knees, pelvis, buttocks, and/or scalp. Increased levels of IgG (an immunoglobulin) are usually found around the blisters. After the blisters heal, scars usually remain. (For more information on this disorder, choose "Epidermolysis Bullosa Acquista" as your search term in the Rare Disease Database.)

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 bpemphigoid 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

@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

McKusick VA, Ed. ONLINE MENDELIAN INHERITANCE IN MAN (OMIM). The Johns Hopkins University. Dystonin; DST. Entry Number; 113810: Last Edit Date; 7/21/2006.



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

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.

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15475 Gratiot Avenue

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Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

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



NIH/NationaI Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

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Tel: (877)226-4267

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Email: NIAMSinfo@mail.nih.gov

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National Cancer Institute

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Tel: (301)435-3848

Tel: (800)422-6237

TDD: (800)332-8615

Email: cancergovstaff@mail.nih.gov

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Genetic and Rare Diseases (GARD) Information Center

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Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Email: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx

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



AutoImmunity Community

Tel: (919) 552-9057

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



Australasian Blistering Diseases Foundation

St. George Hospital

Department of Dermatology

Gray Street

KOGARAH NSW 2217

Australia

Tel: 02 9113 2543

Fax: 02 9113 2906