$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.