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. Mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Mucous membrane pemphigoid most frequently affects the oral cavity 85% of affected individuals followed by the eye 65% of affected individuals. Paraneoplastic mucous membrane pemphigoid with ocular and. Mmp can progress to scarring in affected areas, which may lead to devastating complications including. 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. Inclusion criteria were clinical findings of oral mmp verified by histological and immunofluorescent examination. Mucous membrane pemphigoid an overview sciencedirect topics.
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%. Mucous membrane pemphigoid mmp is a heterogeneous group of autoimmune chronic inflammatory, subepithelial blistering disorder, predominantly involving. Disease onset is usually between 40 and 70 years and oral lesions are seen. Silverman sj, gorsky m, lozadonur f, liu a oral mucous membrane pemphigoid a study of 65 patients oral surg oral med oral patho 1986. 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. 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. There have been reports of various adverse effects associated with the use of atenolol including bullous pemphigoid. Mucous membrane pemphigoid nord national organization for. Cp afects particularly mucous membranes but cutaneous involve ment can also be seen in some cases 99,100. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder of mucous membranes that is characterized by subepithelial bullae chan et al 2002. Pdf oral mucous membrane pemphigoid two case reports with. Cicatricial pemphigoid an overview sciencedirect topics. 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. Those who have the disease affecting only the oral mucosa andor the.
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. Bullous pemphigoid and mucous membrane pemphigoid are suggested as variants of a single entity. Successful treatment of mucous membrane pemphigoid with. Oral mucosa is the most common site being affected in mucous membrane pemphigoid. Mucous membrane pemphigoid mmp is a rare autoimmune bullous disease caused by various autoantibodies. Mmp can affect various mucous membranes but predominantly occurs in the oral cavity. 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. A clinical study of oral mucous membrane pemphigoid a. Ocular and oral mucous membrane pemphigoid cicatricial. Oral manifestations oral lesions are the initial manifestation of mmp in 90% of cases. Oral lesions in bullous pemphigoid jama dermatology jama. First, one has to live with painful and debilitating blisters on their skin andor mucous membranes. Mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. The oral mucosa, conjunctiva, and, less frequently, the nose, oesophagus, larynx and genitals may be affected.
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. Mucous membrane pemphigoid an overview sciencedirect. In both these cases, the disease was limited to the oral cavity with no other mucosal or skin lesions. May 12, 2012 oral pemphigoid pemphigoid of the mouth is an uncommon blistering condition which affects primarily the lining of the mouth and gums. Limited cutaneous involvement typically localized to the head, neck, or upper. 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.
In most of cases oral lesions may be initial manifestation or the only sign of the disease. 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. Oral mucous membranes consist of an epithelial covering of underlying connective tissues fig. Oral biopsy is suggested as a simple diagnostic test. Pdf diagnosis of oral mucous membrane pemphigoid by means.
However, in reporting the results of the first international consensus on mucous membrane pemphigoid, chan and others4 recommended the term mucous. Other moist surfaces of the body known as mucous membranes can. Mucous membrane pemphigoid mmp is a rare, chronic and vesiculobullous disorder that classified as autoimmune disease. Pdf mucous membrane pemphigoid mmp is a heterogeneous group of autoimmune chronic inflammatory, subepithelial blistering disorder. Second, a patient has to find a doctor that has not only heard of the diseases, but knows how to treat them. Mmp can progress to scarring in affected areas, which may lead to devastating complications. It is predominantly seen in the fifth decade of life. 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. Pdf mucous membrane pemphigoid is a rare mucocutaneous disorder that has a predilection for the mucous membranes.
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. 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. For many patients, the search for a correct diagnosis involves months or even years of. Mucous membrane pemphigoid mmp is a heterogeneous group of chronic. 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.
Pemphigus vulgaris and mucous membrane pemphigoid are mucous membrane disorders that, like other mucosal disorders, can be difficult to initially diagnose. 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. Herein, we describe a novel therapeutic approach that is based on 2 reports in the literature of. Atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. Division of oral medicine and dentistry, brigham and womens hospital, 75 francis s treet, boston, ma 02115, 617 7326570.
Mucous membrane pemphigoid genetic and rare diseases. The involvement of skin is only seen in one quarter of the patients with mmp. Mmp is an uncommon blistering conditionwhich most frequently affects the lining of the mouth and gums. Other areas that may be affected include the nostrils. Pdf diagnosis of oral mucous membrane pemphigoid by. Mucous membrane pemphigoid mmp is a group of immunobullous diseases involving the mucosa and skin. Oral mucous membrane pemphigoid without skin involvement. To the best of our knowledge we present the first case report of atenololinduced mucous membrane pemphigoid. Pemphigus vulgaris pv and mucous membrane pemphigoid mmp are among the diseases that pose the greatest diagnostic difficulties. Bullous pemphigoid and mucous membrane pemphigoid mmp are autoimmune blistering diseases that most commonly arise in older adults. Mucous membrane pemphigoid in a patient with hypertension. Mucous membrane pemphigoid also known as mmp, cicatricial pemphigoid, benign mucous membrane pemphigoid, and mucosal pemphigoid. The most commonly affected sites include the oral cavity and eye, followed by the. 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.
Other names include cicatricial pemphigoid, oral pemphigoid and ocular pemphigoid. Mucous membrane pemphigoid progresses slowly, rarely remits spontaneously, and often responds incompletely to treatment. Blistering mucocutaneous diseases of the oral mucosa a. 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. Although oral and ocular mucosae can both be affected in a given. Mucous membrane pemphigoid mmp is 1 of the 2 most common chronic immunopath ogenic diseases to cause chronic oral blistering. 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. The natural history of mucous membrane pemphigoid is not well understood as only a few small case series have been published.
There are many difficult aspects of life with pemphigus and pemphigoid pp. The histopathology of oral lesions in bullous pemphigoid is similar to that of the skin lesions. Oral mucous membrane pemphigoid two of the patients were young a female aged 16 years and a male aged 19 years. 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. A 42yearold thai man presented to our faculty after. Mucous membranes that may be involved include the oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus. Summary the autoimmune blistering disorders present with variable frequency in the oral cavity. Quarterly international pemphigus pemphigoid foundation. Mucous membrane pemphigoid and oral blistering diseases carey.
Blood examinations highlighted ac antipbga2, and the gingival. Nov 14, 2014 mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. For an unknown reason, antibodies natural substances important in. Oral pemphigoid is considered to be an autoimmune disease. The condition is broadly heterogeneous in terms of site and severity of involvement in. The cases were retrieved from the archives of ondokuz mayis university and gulhane military medical academy, turkey, between 1997 and 2002. Mucous membrane pemphigoid is an autoimmune condition, so most therapies involve the use of immunosuppressive drugs. 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 is a heterogeneous group of chronic inflammatory blistering diseases that affect oral, ocular, pharyngeal, laryngeal, genital, or anal mucosa. Scarring occurs with less frequency in cases of mmp that only involve the mucous membranes of the mouth oral mucosa. Recommendations of an international panel of experts. Oral mucous membrane pemphigoid two case reports with varied.
Potential sequelae include painful mucosal erosions, vision loss and laryngeal stenosis. 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. 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. Bajpai m, pardhe n 2017 oral mucous membrane pemphigoid without skin involvement treated by intravenous immunoglobulins.
Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the mucous membranes. A clinical study of oral mucous membrane pemphigoid a alkan. For an unknown reason, antibodies natural substances important in defending your. A clinical study of oral mucous membrane pemphigoid. For the mild oral mucosa lesion, high potency topical steroids such as 0. Like other forms of pemphigoid, the disorder is characterized by the formation of autoantibodies against structural proteins of the dermalepidermal junction. Jan, 2016 there are many difficult aspects of life with pemphigus and pemphigoid pp. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus.
What is mucous membrane pemphigoid mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. 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 msd manual professional edition. Paraneoplastic mucous membrane pemphigoid with ocular. Management of mucous membrane pemphigoid in a joint oral. Ocular and oral mucous membrane pemphigoid request pdf. This study aimed to evaluate the diagnostic value of mmpspecific autoantibodies in patient sera. Mucous membrane pemphigoid mmp mmp is a chronic autoimmune disease of unknown etiology that manifests in the form of subepithelial blisters. Oral lesions in bullous pemphigoid jama dermatology.
Mucous membrane pemphigoid and oral blistering diseases. 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. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder of mucosa, characterized by subepithellial bullae. 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. 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. Mucous membrane pemphigoid is a heterogeneous subepithelial blistering disease predominantly affecting oral, ocular mucous membrane and occasionally the skin. 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. Corticosteroids are the traditional mainstay of treatment, but other immunosuppressants, such as topical tacrolimus, are sometimes used offlabel to some benefit. Mucous membrane pemphigoid mmp is an uncommon chronic blistering disease that usually affects patients over the age of 50. In part 2 of this 2part article, we discuss the other.