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Bullous Pemphigoid

 

Synonyms

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

Disorder Subdivisions

• Autoimmune disorder

Related Disorders List

 

• 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 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. Unlike Pemphigus, Bullous Pemphigoid 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 a autoimmune reaction by the body against the glue holding the skin together; . 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 similart to Bullous Pemphigod. It is essential to determine whether side effects of the pharmaceuticals may be causing the patient’s symptoms, or whether the blisters are caused by Bullous Pemphigoid.

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.

Dermatitis Herpetiformis 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

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 Bullous Pemphigoid than the dosage prescribed to treat Pemphigus. Corticosteroid drugs (usually prednisone) can be discontinued in approximately 50 percent of cases of Bullous Pemphigoid 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

Immunosuppressive drugs such as azathioprine and methotrexate, have been used on an experimental basis in combination with corticosteroid drugs to treat Bullous Pemphigoid.

Cyclosporine (Sandimmune) may be of potential benefit for treating a number of autoimmune skin diseases including Pemphigus, Bullous Pemphigoid, Posterior Uveitis, and Behcet's Syndrome. However, careful monitoring by a physician of people taking cyclosporine is necessary to guard against possible toxic side effects. Relapses of Bullous Pemphigoid can occur when the drug therapy is stopped. More research is needed before cyclosporine can be recommended as a treatment for all but the most severe cases of Bullous Pemphigoid.

Clinical trials are underway to study possible new therapies for Bullous Pemphigoid. Interested persons may wish to have their physician contact:

W.R. Gammon, M.D.
Dept. of Dermatology
University of North Carolina
137 NC Memorial Hospital
Chapel Hill, NC 27514

References

McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 113810; Last Update: 7/21/99.

TEXTBOOKS
Thoene JG., ed. Physicians’ Guide to Rare Diseases. Montvale, NJ: Dowden Publishing Company Inc; 1995:651

Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:968.

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

Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:1647-51.

Habif TP, ed. Clinical Dermatology. 2nd ed. St. Louis, MO: The C.V. Mosby Company; 1990:415-16.

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2205-06.


REVIEW ARTICLES
Roeder C, et al., Psoriatic erythroderma and bullous pemphigoid treated successfully with acitretin and azathioprine. Eur J Dermatol. 1999;9:537-39.

Nousari HC, et al., Pemphigus and bullous pemphigoid. Lancet. 1999;354:667-72.

Trueb RM, et al., Childhood bullous pemphigoid: report of a case with characterization of the targeted antigens. J Am Acad Dermatol. 1999;40:338-44.

Scott JE, et al., The blistering diseases. Med Clin North Am. 1998;82:1239-83.

JOURNAL ARTICLES
Korman NJ., New and emerging therapies in the treatment of blistering diseases. Dermatol Clin. 2000;105:113-23.

Kuboki T, et al., [Recurrent bleeding tendency due to factor VIII inhibitor during therapy for bullous pemphigoid. Rinsho Ketsueki. 1999;40:1087-92.

Mainguy G, et al., Regulation of epidermal bullous pemphigoid antigen 1 (BPAG1) synthesis by homeoprotein transcription factors. J Invest Dermatol. 1999;113:643-50.

Kirtschig G, et al., IgA basement membrane zone autoantibodies in bullous pemphigoid detect epidermal antigens of 270-280 kDa, 230 kDa, and 180 kDa molecular weight by immunoblotting. Clin Exp Dermatol. 1999;24:302-07.

Egan CA, et al., The immunoglobulin A antibody response in clinical subsets of mucous membrane pemphigoid. Dermatology. 1999;198:330-35.

Rhodes LE, et al., Blister fluid cytokines in cutaneous inflammatory bullous disorders. Acta Derm Venereol. 1999;79:288-90.

Jung M, et al., Increased risk of bullous pemphigoid in male and very old patients: A population-based study on incidence. J Am Acad Dermatol. 1999;41 (2 Pt 1):266-68.