Other areas that may be affected include the nostrils. Although oral and ocular mucosae can both be affected in a given patient, patients with involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance, resulting in scarring and blindness. Any mucous membrane can be involved, but the most commonly involved sites in decreasing frequency include the oral mucosa 85%, conjunctiva 64%, skin 24%, pharynx 19%, external genitalia 17%, nasal mucosa 15%, larynx 8%, anus 4%, and esophagus 4%. Bullous pemphigoid and mucous membrane pemphigoid are suggested as variants of a single entity.
Introduction the treatment of oral mucosal disorders must be based on an early and correct diagnosis. Abstract mucous membrane pemphigoid mmp is a rare, chronic and vesiculobullous disorder that classified as autoimmune disease. Mucous membrane pemphigoid mmp is a rare, chronic and vesiculobullous disorder that classified as autoimmune disease. Mucous membrane pemphigoid an overview sciencedirect. Atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder of mucosa, characterized by subepithellial bullae.
Mucous membrane pemphigoid mmp refers to a heterogeneous group of rare, chronic, inflammatory, mucous membranedominated, subepithelial blistering diseases that manifest with a varying constellation of oral, ocular, cutaneous, genital, nasopharyngeal, esophageal, and laryngeal lesions. Pemphigus vulgaris and mucous membrane pemphigoid are mucous membrane disorders that, like other mucosal disorders, can be difficult to initially diagnose. Second, a patient has to find a doctor that has not only heard of the diseases, but knows how to treat them. Mucous membrane pemphigoid mmp is a rare predominantly mucosal subepidermal blistering disorder involving the oral mucosa, conjunctiva, anogenital tissues and upper aerodigestive tract, with occasional skin involvement. The characteristic pathologic feature that unites these disorders is the presence of linear deposits of immunoglobulin igg, iga, igm, or c3 in the epithelial basement.
Oral pemphigoid is considered to be an autoimmune disease. A 42yearold thai man presented to our faculty after. Mucous membrane pemphigoid mmp, previously known as cicatricial pemphigoid, is a systemic cicatrizing autoimmune disease characterized by chronic blistering of mucous membranes, including ocular, oral, genital, nasopharyngeal, anogenital, and laryngeal mucosa, with ocular 60. Mucous membrane pemphigoid mmp is a heterogeneous group of autoimmune chronic inflammatory, subepithelial blistering disorder, predominantly involving. Mucous membrane pemphigoid includes a heterogeneous group of blistering diseases, which have in common the involvement of the mucosae, a chronic course and a scarring tendency. These disorders are characterized by subepithelial blister formation and the deposition of immunoglobulins and complement within the epidermal andor mucosal basement membrane zone. Mucous membrane pemphigoid also known as mmp, cicatricial pemphigoid, benign mucous membrane pemphigoid, and mucosal pemphigoid. Other moist surfaces of the body known as mucous membranes can also be affected, and these include the surface layers of the eyes, inside the nose and the genitalia. Bullous pemphigoid and mucous membrane pemphigoid mmp are autoimmune blistering diseases that most commonly arise in older adults. Oct 31, 2012 we next considered the pemphigoid family, such as mucous membrane pemphigoid or cicatricial pemphigoid, because of the presence of oral mucosal lesions generalized erythema, desquamation, multiple large ulcers covered with yellowish slough tissue along the gingiva, and multiple tense bullae of the oral mucosa and tongue. The most commonly affected sites include the oral cavity and eye, followed by the. Mmp can progress to scarring in affected areas, which may lead to devastating complications including. Mucous membrane pemphigoid is a heterogeneous group of chronic inflammatory blistering diseases that affect oral, ocular, pharyngeal, laryngeal, genital, or anal mucosa.
Oral lesions in bullous pemphigoid jama dermatology jama. Mucous membrane pemphigoid genetic and rare diseases. Mucous membrane pemphigoid mmp, also referred to as cicatricial pemphigoid, is a rare bullous disease affecting predominantly mucosal sites and occasionally the skin in about 25% of cases. Mucous membrane pemphigoid mmp is 1 of the 2 most common chronic immunopath ogenic diseases to cause chronic oral blistering. Mucous membrane pemphigoid mmp is a heterogeneous group of chronic. Mucous membrane pemphigoid and oral blistering diseases. Classically, the variants of mmp are bullous pemphigoid and mucous membrane or cicatricial pemphigoid the latter being the most common presentation. There were no predisposing factors such as emotional stress, smoking, nutritional deficiency or systemic disease. Cp afects particularly mucous membranes but cutaneous involve ment can also be seen in some cases 99,100. Recommendations of an international panel of experts. The oral disease severity score odss is a disease scoring tool that has been validated for mmp, pv and a b figure 1 a,b mucous membrane pemphigoid. Mucous membrane pemphigoid most frequently affects the oral cavity 85% of affected individuals followed by the eye 65% of affected individuals.
Pdf oral mucous membrane pemphigoid two case reports with. Blistering mucocutaneous diseases of the oral mucosa a. Background mucous membrane pemphigoid mmp, also known as cicatricial pemphigoid, is a serious, autoimmune, blistering disorder that can result in blindness and other complications as a result of scarring of the mucous membranes. Other moist surfaces of the body known as mucous membranes can. Pdf mucous membrane pemphigoid mmp is a heterogeneous group of autoimmune chronic inflammatory, subepithelial blistering disorder. Depending on the site affected, serious sequelae may include ocular damage and blindness, airway erosions and destruction, and strictures of the esophagus or anogenital regions.
A clinical study of oral mucous membrane pemphigoid. Oral mucous membrane pemphigoid two of the patients were young a female aged 16 years and a male aged 19 years. Oral mucous membranes consist of an epithelial covering of underlying connective tissues fig. Cicatricial pemphigoid an overview sciencedirect topics.
Paraneoplastic mucous membrane pemphigoid with ocular. Other names include cicatricial pemphigoid, oral pemphigoid and ocular pemphigoid. For many patients, the search for a correct diagnosis involves months or even years of. First, one has to live with painful and debilitating blisters on their skin andor mucous membranes. Definitions and outcome measures for mucous membrane pemphigoid. Quarterly international pemphigus pemphigoid foundation. Nov 14, 2014 mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Bajpai m, pardhe n 2017 oral mucous membrane pemphigoid without skin involvement treated by intravenous immunoglobulins.
What is mucous membrane pemphigoid mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. Oral lesions in bullous pemphigoid jama dermatology. Mucous membrane pemphigoid mmp is a rare, chronic, autoimmune, subepithelial blistering and erosive disease that affects the mucosal surfaces of the mouth gingiva, movable mucosa, tongue, and palate, eyes, nose, nasopharynx, hypopharynx, larynx, esophagus, genitals, andor anus picture 1ac. Ocular and oral mucous membrane pemphigoid request pdf. Pemphigus vulgaris pv and mucous membrane pemphigoid mmp are among the diseases that pose the greatest diagnostic difficulties. Oral mucosa the oral cavity is lined with an uninterrupted mucous membrane, which is continuous with the skin near the vermilion border of the lips and with the pharyngeal mucosa in the region of the soft palate and anterior pillars of fauces. However, in reporting the results of the first international consensus on mucous membrane pemphigoid, chan and others4 recommended the term mucous membrane pemphigoidbecause the disease may. Blood examinations highlighted ac antipbga2, and the gingival. Mucous membranes that may be involved include the oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus. Silverman sj, gorsky m, lozadonur f, liu a oral mucous membrane pemphigoid a study of 65 patients oral surg oral med oral patho 1986. The cases were retrieved from the archives of ondokuz mayis university and gulhane military medical academy, turkey, between 1997 and 2002.
To the best of our knowledge we present the first case report of atenololinduced mucous membrane pemphigoid. Mucous membrane pemphigoid is a heterogeneous subepithelial blistering disease predominantly affecting oral, ocular mucous membrane and occasionally the skin. Mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Pdf mucous membrane pemphigoid is a rare mucocutaneous disorder that has a predilection for the mucous membranes. Scarring occurs with less frequency in cases of mmp that only involve the mucous membranes of the mouth oral mucosa. Mucous membrane pemphigoid mmp mmp is a chronic autoimmune disease of unknown etiology that manifests in the form of subepithelial blisters. Mucous membrane pemphigoid is a heterogeneous subepithelial blistering disease that predominantly affects the mucous membranes, including the conjunctiva and occasionally the skin.
Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the mucous membranes. Mucous membrane pemphigoid and oral blistering diseases carey. Division of oral medicine and dentistry, brigham and womens hospital, 75 francis s treet, boston, ma 02115, 617 7326570. Ocular and oral mucous membrane pemphigoid cicatricial. Like other forms of pemphigoid, the disorder is characterized by the formation of autoantibodies against structural proteins of the dermalepidermal junction. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus.
For the mild oral mucosa lesion, high potency topical steroids such as 0. The natural history of mucous membrane pemphigoid is not well understood as only a few small case series have been published. There have been reports of various adverse effects associated with the use of atenolol including bullous pemphigoid. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder of mucous membranes that is characterized by subepithelial bullae chan et al 2002.
The moist lining of the oral cavity that is in continuation with the exterior surface of skin on one end and oesophagus on the other end is. In part 2 of this 2part article, we discuss the other. Mucous membrane pemphigoid mmp is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity. Although oral and ocular mucosae can both be affected in a given. Cicatricial pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin that can result in scarring 581 it is one of the pemphigoid diseases. Oral mucosa the moist lining of the oral cavity is called oral mucosa or oral mucous membrane the function of oral mucosa is protection, sensation and secretion it is continuous with the skin outside the oral cavity and differs from it in appearance and structure dr. Paraneoplastic mucous membrane pemphigoid with ocular and. Mucous membrane pemphigoid is an autoimmune condition, so most therapies involve the use of immunosuppressive drugs.
Mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. Mucous membrane pemphigoid mmp is an uncommon chronic blistering disease that usually affects patients over the age of 50. Inclusion criteria were clinical findings of oral mmp verified by histological and immunofluorescent examination. Mmp can progress to scarring in affected areas, which may lead to devastating complications. Division of oral medicine and dentistry, brigham and women. Management of mucous membrane pemphigoid in a joint oral. Oral mucous membrane pemphigoid two case reports with varied. For an unknown reason, antibodies natural substances important in. Oral biopsy is suggested as a simple diagnostic test. The oral mucosa is often the initial site of mmp lesions, so it is important that dentists as well as physicians are aware of the symptoms and signs. May 12, 2012 oral pemphigoid pemphigoid of the mouth is an uncommon blistering condition which affects primarily the lining of the mouth and gums. Mucous membrane pemphigoid mmp is a group of immunobullous diseases involving the mucosa and skin. Mmp can affect various mucous membranes but predominantly occurs in the oral cavity. Patients are instructed to apply the ointment or gel 23 times a day after drying the oral mucosa to enhance the adherence of mediation to oral mucosa.
Potential sequelae include painful mucosal erosions, vision loss and laryngeal stenosis. Oral manifestations oral lesions are the initial manifestation of mmp in 90% of cases. Limited cutaneous involvement typically localized to the head, neck, or upper. Oral mucosa is the most common site being affected in mucous membrane pemphigoid. This study aimed to evaluate the diagnostic value of mmpspecific autoantibodies in patient sera. Corticosteroids are the traditional mainstay of treatment, but other immunosuppressants, such as topical tacrolimus, are sometimes used offlabel to some benefit. Pdf diagnosis of oral mucous membrane pemphigoid by. There are many difficult aspects of life with pemphigus and pemphigoid pp.
A clinical study of oral mucous membrane pemphigoid a. Mucous membrane pemphigoid in a patient with hypertension. Pdf diagnosis of oral mucous membrane pemphigoid by means. Mucous membrane pemphigoid msd manual professional edition. The histopathology of oral lesions in bullous pemphigoid is similar to that of the skin lesions. Jan, 2016 there are many difficult aspects of life with pemphigus and pemphigoid pp. Areas commonly involved are the oral mucosa lining of the mouth and conjunctiva mucous membrane that coats the inner surface of the eyelids and the outer surface of the eye. Mucous membrane pemphigoid mmp, also known as cicatricial pemphigoid, is defined as a heterogeneous group of putative autoimmune subepithelial blistering diseases primarily affecting any mucous membranes, including the oral cavity, ocular mucosae, or mucous membranes of the nose, larynx, esophagus, rectum, penis, and vagina. Mucous membrane pemphigoid progresses slowly, rarely remits spontaneously, and often responds incompletely to treatment. Successful treatment of mucous membrane pemphigoid with. Summary the autoimmune blistering disorders present with variable frequency in the oral cavity.
For an unknown reason, antibodies natural substances important in defending your. A clinical study of oral mucous membrane pemphigoid a alkan. Herein, we describe a novel therapeutic approach that is based on 2 reports in the literature of. The involvement of skin is only seen in one quarter of the patients with mmp. It is predominantly seen in the fifth decade of life. The diagnosis of mucous membrane pemphigoid was suspected after a positive pinch test. In both these cases, the disease was limited to the oral cavity with no other mucosal or skin lesions. The condition is broadly heterogeneous in terms of site and severity of involvement in. Mucous membrane pemphigoid mmp is a rare autoimmune bullous disease caused by various autoantibodies. The oral mucosa, conjunctiva, and, less frequently, the nose, oesophagus, larynx and genitals may be affected. Those who have the disease affecting only the oral mucosa andor the. Disease onset is usually between 40 and 70 years and oral lesions are seen. Jan 24, 2020 mucous membrane pemphigoid mmp refers to a heterogeneous group of rare, chronic, inflammatory, mucous membrane dominated, subepithelial blistering diseases that manifest with a varying constellation of oral, ocular, cutaneous, genital, nasopharyngeal, esophageal, and laryngeal lesions.
1271 1301 1343 786 359 1169 1424 937 842 861 1001 829 493 682 442 3 1064 354 1283 1329 1531 1438 1216 953 286 153 969 1427 1216 321 54 578 317