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$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.