$Unique_ID{BRK03550} $Pretitle{} $Title{Bullous Pemphigoid} $Subject{Bullous Pemphigoid Pemphigoid Old Age Pemphigus Parapemphigus Senile Dermatitis Herpetiformis Benign Pemphigus Pemphigus Erythema Multiforme Pemphigoid, Benign Mucosal Dermatitis Herpetiformis Epidermolytic Hyperkeratosis Epidermolysis Bullosa Epidermolysis Bullosa Acquista} $Volume{} $Log{} Copyright (C) 1986, 1988, 1992, 1993 National Organization for Rare Disorders, Inc. 54: Bullous Pemphigoid ** IMPORTANT ** It is possible that the main title of the article (Bullous Pemphigoid) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Pemphigoid Old Age Pemphigus Parapemphigus Senile Dermatitis Herpetiformis Benign Pemphigus 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 Hyperkeratosis Epidermolysis Bullosa Epidermolysis Bullosa Acquista General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Bullous Pemphigoid is a rare, 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 under surfaces of the arms and legs (flexor surfaces), in the armpits (axillae), on the abdomen, and/or around the groin. Bullous Pemphigoid usually does not affect the ears or the mucous membranes, such as the lining of the mouth. When these areas are affected, they generally heal rapidly. The blisters are usually large and rigid, 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. Other symptoms of Bullous Pemphigoid may include an elevation of white blood cell count (detected by laboratory test), but the patient is not likely to have a fever. A test in which a fluorescent dye is used to stain antibodies (immunofluorescent microscopic examination) may reveal blisters under the top layers of skin and the binding of IgG antibodies to the bottom layers of the skin membranes. When fluid in the blisters (serum) is analysed, it contains these antibodies in approximately 70 percent of the people with Bullous Pemphigoid. The importance of the antibody testing (titres) in relation to the severity of the symptoms is unknown; tests that measure antibody activity do not seem to reflect disease activity and/or severity of 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 although it has been suggested that may be an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms, suddenly begin to attack perfectly healthy tissue. Certain drug reactions can produce skin lesions that are very similar to those of Bullous Pemphigoid. It is important to determine whether side effects of pharmaceuticals may be causing the patient's symptoms, or whether the blisters are caused by Bullous Pemphigoid. Affected Population 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 a group of rare autoimmune skin disorders characterized by the development of blisters in the outer layers of the skin (epidermis) and 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 allergic inflammatory skin disorder characterized by lesions that develop 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 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.) Therapies: Standard 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. Therapies: Investigational 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 This disease entry is based upon medical information available through January 1993. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Bullous Pemphigoid, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References TEXTBOOK OF DERMATOLOGY, Vol II, 3rd ed. A. Rook et al.; Oxford and Blackwell Scientific Publications, 1979. MENDELIAN INHERITANCE IN MAN, 10th Ed.: Victor A. McKusick, Editor: Johns Hopkins University Press, 1992. Pp. 171. CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 2282, 2309. THE MERCK MANUAL, 16th Ed.: Robert Berkow Ed.; Merck Research Laboratories, 1992. Pp. 2444. CLINICAL DERMATOLOGY, 2nd Ed.; Thomas P. Habif, M.D., Editor: The C.V. Mosby Company, 1990. Pp. 415-416. MECHANISM OF LESION PRODUCTION IN PEMPHIGUS AND PEMPHIGOID. William Sams Jr.; J Am Acad Dermatol (April 1982;(4 part 1)): Pp. 431-52. AZATHIOPRINE IN DERMATOLOGY. I.R. Younger; J Am Acad Dermatol (Aug 1991; 25(2 Pt 1)): Pp. 281-286. BULLOUS PEMPHIGOID. N. Korman; J Am Acad Dermatol (May 1987; 16(5.1)). Pp. 907-924. BULLOUS PEMPHIGOID AND CICATRICIAL PEMPHIGOID. G.J. Anhalt; J Autoimmun (Feb 1991; 4(1)). Pp. 17-35.