Mucous membrane pemphigoid mmp mmp is a chronic autoimmune disease of unknown etiology that manifests in the form of subepithelial blisters. Mucous membrane pemphigoid mmp is a rare group of chronic autoimmune disorders. Ocular mmp ocmmp also encompasses the conditions linear immunoglobulin a disease, mucosal dominated epidermolysis. 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 and oral blistering diseases. Mucous membrane pemphigoid mmp is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin332. Lowpower photomicrograph showing separation of the epithelium and connective tissue, resulting in a subepithelial blister. Mounting evidence supports the concept of immunoregulatory dysfunction. Bullous pemphigoid is a rare and chronic autoimmune disorder characterised by subepidermal blisters that predominantly involves the skin and less commonly the mucous membrane. Like all patients at that time, i was welcomed into the fold by the ippfs founder, janet segall, who nurtured the organization and all involved as if they were her own family. Mmp is characterized by the formation of autoantibodies to the basement membrane zone bmz.
The patient also had blistering lesions on the roof of his mouth, trunk, and buttocks as well as. However, in reporting the results of the first international consensus on mucous membrane pemphigoid, chan and others4 recommended the term mucous. What is mucous membrane pemphigoid mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. To be a member of this group you must have a form of pemphigoid or an associated illness or be a supporter. This is the result of autoantibodies formed against. There is limited evidence that mucous membrane pemphigoid involving the eyes responds best to treatment with cyclophosphamide combined with corticosteroids. Ocular mucous membrane pemphigoid mmp is a rare, immunomediated chronic progressive condition of the conjunctiva characterized by blisters developing from subepithelial tissue through disruption of the adhesions between the conjunctival epithelium and the subepithelium. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular andor digestive sequelae with important morbidity. Mucous membrane pemphigoid and oral blistering diseases carey. Mucous membranes that may be involved include the oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus. A clinical formocular cicatricial pemphigoid ocpmay result in blindness if it involves the eyes. Ocular mucous membrane pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with opacification of the cornea. 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 membrane zone.
Mucous membrane pemphigoid genetic and rare diseases. Ocular mucous membrane pemphigoid mmp is a rare, immunomediated chronic progressive condition of the conjunctiva characterized by blisters developing from sub epithelial tissue through. Mucous membrane pemphigoid nord national organization for. Atenolol is commonly used by patients with hypertension, angina pectoris, or myocardial infarction. Pathogenesis of mucous membrane pemphigoid dermatologic. 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 that predominantly affects the mucous membranes, including the conjunctiva and occasionally the skin.
Safe and effective adjuvant or steroidsparing agents are needed. 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. Mucous membrane pemphigoid is a challenging disease to treat, even more so when the primary specialty physician is unable to visualize the affected areas. 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.
Various basement membrane zone components have been identified as targets of autoantibodies in mmp. It triggers a type ii hypersensitivity, which leads to the binding of autoantibodies against the basement membrane zone. There have been reports of various adverse effects associated with the use of atenolol including bullous pemphigoid. Mucous membrane pemphigoid in a patient with chronic. Mucous membrane pemphigoid mmp is an autoimmune blistering disease that is notoriously difficult to treat.
Mucous membrane pemphigoid mmp is a systemic cicatrizing autoimmune disease that primarily affects orificial mucous membranes, such as the conjunctiva, the nasal cavity, the oropharynx, and the genitalia. Mucous membrane pemphigoidassociated paronychia with. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder that causes subepithelial damage and scarring of mucosal surfaces with or without skin involvement. Mucous membrane pemphigoid in a patient with hypertension. Although it primarily affects the conjunctiva, other mucous membranes can be involved. Pdf mucous membrane pemphigoid is a rare mucocutaneous disorder that has a predilection for the mucous membranes. Successful treatment of mucous membrane pemphigoid with. Mucous membrane pemphigoid mmp is a heterogeneous group of. Mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. 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. It predominantly affects middleaged to elderly individuals, and occurs slightly more often in females. Mucous membrane pemphigoid mmp this form of the disease affects the eyes, mouth, and throat. Mucous membrane pemphigoid mmp is an autoimmune blistering disorder of mucous membranes that is characterized by subepithelial bullae chan et al 2002.
Other sites of mucous membrane involvement include the pharynx and. Bullous pemphigoid bp this strain is limited to the skin. 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. Mucous membrane pemphigoid mmp is an autoimmune disease of unknown etiology. Pdf treatment strategies in mucous membrane pemphigoid. Ocular involvement occurs in about 70% of all mmp cases. Outcomes for mucous membrane pemphigoid depend on early. Like other forms of pemphigoid, the disorder is characterized by the formation of autoantibodies against structural proteins of the dermalepidermal junction. Patients with cutaneous involvement present with tense blisters and erosions, often on the head and the neck or at sites of trauma. 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. A 42yearold thai man presented to our faculty after. T1 the management of mucous membrane pemphigoid and pemphigus. Treatment of mucous membrane pemphigoid with janus kinase. Pdf management of ocular mucous membrane pemphigoid.
First, one has to live with painful and debilitating blisters on their skin andor mucous membranes. It is the most common type of the pemphigoid group, representing 80% of subepidermal immunobullous cases. The oropharynx is the most common site of initial presentation, followed by ocular, nasopharyngeal, anogenital, skin, laryngeal, and esophageal involvement. Mucous membrane pemphigoid an overview sciencedirect. Herein, we describe a novel therapeutic approach that is based on 2 reports in the literature of. However, mucous membrane pemphigoid with mild to modest inflammatory activity responds to dapsone in most participants and may therefore be best treated with dapsone due to its lower side effect profile compared to. Classically, the variants of mmp are bullous pemphigoid and mucous membrane or cicatricial pemphigoid the latter being the most common presentation. It is generally con sidered a disease of middle age, and is twice as com mon in women with no racial or ethnic predilection.
Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. Pdf colchicine in the management of mucous membrane. Mucous membrane pemphigoid mmp is the designation for a class of rare autoimmune blis tering disorders in which autoantibodies directed against structural. Second, a patient has to find a doctor that has not only heard of the diseases, but knows how to treat them. Pemphigoid diseases including bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear iga dermatosis, lichen planus pemphigoides, and antip200 pemphigoid are a subgroup of autoimmune bullous skin diseases characterized by an autoantibody response toward structural components of the hemidesmosome resulting in subepidermal. If it involves the deeper parts of the throat, it may cause respiratory compromise.
Limited cutaneous involvement typically localized to the head, neck, or upper. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Mucous membrane pemphigoid also known as mmp, cicatricial pemphigoid, benign mucous membrane pemphigoid, and mucosal pemphigoid. The term ocular cicatricial pemphigoid ocp refers to mucous membrane pemphigoid that clinically presents as a chronic cicatrizing scarring conjunctivitis. These disorders are characterized by vesiculobullous lesions that. These include the rare autoimmune blistering diseases mucous membrane pemphigoid mmp, pemphigus vulgaris pv, linear iga disease.
The most commonly affected sites include the oral cavity and eye, followed by the. Is the initiation of noninvasive dental hygiene procedures contraindicated. Review article ced clinical and experimental dermatology cpd mucous membrane pemphigoid and oral blistering diseases b. The most characteristic clinical feature of benign mucous membrane pemphigoid is the predilection for recurring bullae of. The diagnosis and blistering mechanisms of mucous membrane. At present, immunoprecipitation is regarded as the technique of choice for identifying the patient au toantibody target antigens.
Patients with ocular mmp, in many cases, develop profound conjunctival scarring and visual impairment. Mucous membrane pemphigoid is extremely rare in the lower respiratory tract, and when these lesions are discovered, it often is in the face of lifethreatening respiratory distress. Mucous membrane pemphigoid mmp is a mucous membranedominated autoimmune subepithelial blistering disease that is caused by autoantibodies against various autoantigens in basement membrane zone bmz proteins, including collagen xvii col17. Ocular mucous membrane pemphigoid eye disorders merck. Blistering mucocutaneous diseases of the oral mucosa a. Mucous membrane pemphigoid mmp is a heterogeneous group of autoimmune subepithelial blistering diseases affecting primarily mucous membranes showing marked degree of clinical and immunological variability. Its management requires longterm oral corticosteroids. Mucous membrane pemphigoid mmp, a chronic systemic autoimmune blistering disease that affects any mucous membrane, is characterized by.
Mucous membrane pemphigoid mmp is an autoimmune blistering disorder that is characterized by subepithelial bullae. I was diagnosed with mucous membrane pemphigoid in august, 2007, and was very lucky to fi nd the ippf. Cicatricial pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Other areas that may be affected include the nostrils. Mucous membrane pemphigoid mucous membrane pemphigoid mmp is an autoimmune, chronic inflammatory, subepithelial blistering disease. Mucous membrane pemphigoid is a bullous dermatosis characterised clinically by blistering of the mucous. Clinicians face diagnostic problems in detecting circulating antibodies and targeted antigens in mmp. Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting muco sal surfaces. Mucous membrane pemphigoid, previously known as cicatricial pemphigoid, is a very rare subepidermal autoimmune blistering disease which predominantly affects the mucous membranes such as the inner linings of the mouth, eyes and genitalia. N2 mucous membrane pemphigoid and pemphigus vulgaris are autoimmune blistering disorders in which many similar drugs and therapeutic strategies are utilized. Mucous membrane pemphigoid mmp usually runs a chronic and potentially devastating course. Paraneoplastic mucous membrane pemphigoid with ocular and.
Cicatricial pemphigoid cp refers to a group of rare chronic autoimmune blistering diseases that predominately affects the mucous membranes, including the conjunctiva, and occasionally the skin. Ocular cicatricial pemphigoid discussion ocular cicatricial pemphigoid is a systemic autoimmune disease. We report on a 58 years old man with severe mmp who presented with onychomadesis. Mucous membrane pemphigoid involving the trachea and. To our knowledge, mucous membrane pemphigoid associated paronychia and onychomadesis have not been reported before. Patients typically have skin lesions, some also have mucous membrane lesions.153 523 1010 406 1105 183 155 1313 1401 670 1143 1472 1478 486 234 60 1439 449 268 59 985 1378 306 240 1107 1374 1021 1361 1036 509 792 1179 251 1300 1055 1085 1211 122 858 100 755 262 1170 692 823 1076 267