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$Unique_ID{BRK04093}
$Pretitle{}
$Title{Pemphigoid, Benign Mucosal}
$Subject{Pemphigoid, Benign Mucosal Mucous Membrane Pemphigoid MMP Cicatricial
Pemphigoid CP Localized Cicatricial Pemphigoid (Brunsting-Perry Syndrome)
Vegetating Cicatricial Pemphigoid Intermittent Mucosal Pemphigoid Gingivitis
Bullous Pemphigoid Epidermolysis Bullosa Acquisita Pemphigus Vulgaris}
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
652:
Pemphigoid, Benign Mucosal
** IMPORTANT **
It is possible that the main title of the article (Benign Mucosal
Pemphigoid) is not the name you expected. Please check the SYNONYM listing
to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
Mucous Membrane Pemphigoid
MMP
Cicatricial Pemphigoid
CP
Disorder Subdivisions:
Localized Cicatricial Pemphigoid (Brunsting-Perry Syndrome)
Vegetating Cicatricial Pemphigoid
Information on the following diseases can be found in the Related
Disorders section of this report:
Intermittent Mucosal Pemphigoid
Gingivitis
Bullous Pemphigoid
Epidermolysis Bullosa Acquisita
Pemphigus Vulgaris
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.
Benign Mucosal Pemphigoid is a rare skin disease characterized by
blisters on the mucous membranes (the thin moist layer lining the body's
cavities). Mucous membranes of the mouth and the conjunctiva (the eyelids)
are commonly affected areas.
Symptoms
The first presenting symptom of Benign Mucosal Pemphigoid usually is a red
and blistered mouth.
The eye is a commonly affected area and may be the only area affected.
The eyes may become red and the conjunctiva inflamed. There may be
conjunctival scarring with formation of tissue between the eyelid and eyeball
(symblepharon).
Occasionally, blisters may occur in the mucous membranes of the pharynx
and esophagus (the continuous tube extending from in back of the nose to the
stomach), nose, vulva (female's external genitalia), and urethra (the tube in
which urine is released). Blisters infrequently occur on the skin.
Affected areas frequently develop scarring. Particular antibodies
(substances the body produces to fight invading organisms) such as IgA, IgG,
and C3 may be found in affected areas.
Benign Mucosal Pemphigoid usually has a long duration with frequent
remissions and recurrence.
DISORDER SUBDIVISIONS
Researchers suggest that Benign Mucosal Pemphigoid may be divided into
the following additional categories:
Localized Cicatricial Pemphigoid or Brunsting-Perry Syndrome is a chronic
scarring disease characterized by blisters usually occurring on the head and
neck. The blisters may be due to trauma or other local factors.
Vegetating Mucous Membrane Pemphigoid combines features of Benign Mucous
Membrane Pemphigoid and Pemphigus Vegetans. Pemphigus Vegetans is a
variation of Pemphigus Vulgaris (see Related Disorder section). It is
characterized by large, fast-growing blisters that usually occur in the
armpit and groin areas.
Causes
The exact cause of Benign Mucosal Pemphigoid is not known. It is thought to
be an autoimmune disorder. Autoimmune disorders are caused when the body's
natural defenses (antibodies) against invading organisms suddenly begin to
attack healthy tissue.
Affected Population
Benign Mucosal Pemphigoid usually affects middle-aged and elderly persons.
However, cases affecting children and adolescents have been reported. Males
and females are equally affected.
Related Disorders
Symptoms of the following disorders can be similar to those of Benign Mucosal
Pemphigoid. Comparisons may be useful for a differential diagnosis:
Gingivitis may resemble the red, blistered mouth common to Benign Mucosal
Membrane Pemphigoid. Gingivitis is a common condition characterized by red,
swollen gums that bleed easily when subjected to pressure. It usually begins
during puberty, persists throughout life with varying severity, and can be
caused by poor oral hygiene. There usually is little to no discomfort but
may progress slowly to periodontal disease. In Desquamative Gingivitis, the
reddened skin peels off in the form of scales.
Intermittent Mucosal Pemphigoid consists of blisters occurring in the
mouth which are few, widely separated in time, and heal without scar
formation.
Bullous Pemphigoid is a chronic skin disease usually affecting the
elderly. It is characterized by firm, large blisters that develop on normal-
appearing or reddened skin usually around cuts or scars. Within weeks,
blisters spread to skin of the flexor (muscles that contract or flex) areas,
groin, armpit, and the abdomen. Mucous membranes seldom are affected and
tend to heal quickly. The blisters have little tendency to spread, but heal
quickly when they do. There is, however, severe irritation. Bullous
Pemphigoid is considered to be an autoimmune disorder. (For more information
on this disorder, choose "Bullous Pemphigoid" as your search term in the
Rare Disease Database).
Epidermolysis Bullosa Acquisita is an acquired skin disorder usually
affecting the middle-aged and elderly. Injuries may cause blisters on the
skin of extensor areas including elbows, knees, pelvis, buttocks, and
sometimes the skull. Eyes may also be affected. The blisters tend to leave
scars after healing. There usually is IgG activity around the blisters.
Epidermolysis Bullosa Acquisita is thought to be an autoimmune disorder.
Pemphigus Vulgaris is a form of Pemphigus. Blisters may start as
isolated regions on the scalp, then in the mouth. The blisters may persist
for several months before appearing in other mucous membranes such as the
esophagus, nose, eyelids (conjunctiva), and rectum. The blisters are soft,
break easily, and heal poorly. Pressure on the border of the blisters causes
them to spread. Pressure on normal-looking skin can cause it to fall off
(Nikolsky's sign) and to blister. Pemphigus Vulgaris is an autoimmune
disorder that usually affects the middle-aged and elderly. (For more
information on this disorder, choose "Pemphigus" as your search term in the
Rare Disease Database).
Therapies: Standard
The usual treatment of Benign Mucosal Pemphigoid is various drugs, either
singly or in combinations.
Corticosteroids drugs are commonly prescribed. Topical corticosteroids
such as fluocinonide can relieve inflammation and itching. Systemic
corticosteroids such as prednisone relieve inflammation and can suppress the
immune system.
Immunosuppressive drugs such as cyclophosphamide or azathioprine may also
be used to treat Benign Mucosal Pemphigoid. The antibiotic drug dapsone may
also be used to relieve inflammation. All of these drugs must be carefully
monitored by a dermatologist for side effects. Other treatment is
symptomatic and supportive.
Therapies: Investigational
The drug aldesulfonsodium is being investigated to treat childhood Benign
Mucosal Pemphigoid.
This disease entry is based upon medical information available through
April 1989. 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 Benign Mucosal 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 (NIAMS)
Information Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 1370.
MUCOSAL INVOLVEMENT IN BULLOUS AND CICATRICIAL PEMPHIGOID. A CLINICAL
AND IMMUNOPATHOLOGICAL STUDY: V.A. Venning et al.; Br J Dermatol (January,
1988: issue 118(1)). Pp. 7-15.
OCULAR CICATRICIAL PEMPHIGOID WITH GRANULAR IgG AND COMPLEMENT
DEPOSITION: A.D. Proia et al.; Arch Ophthalmol (November, 1985: issue
103(11)). Pp. 1669-1672.
IMMUNOSUPPRESSIVE THERAPY IN OCULAR CICATRICIAL PEMPHIGOID: B.J. Mondino
and S.I. Brown; Am J Opthalmol (October, 1983: issue 96(4)). Pp. 453-459.