 | This articleneeds additional citations forverification. Please helpimprove this article byadding citations to reliable sources. Unsourced material may be challenged and removed. Find sources: "Mucous membrane pemphigoid" – news ·newspapers ·books ·scholar ·JSTOR(February 2009) (Learn how and when to remove this message) |
| Mucous membrane pemphigoid | |
|---|---|
| Other names | Cicatricial pemphigoid; MMP, Benign mucosal pemphigoid, Benign mucous membrane pemphigoid,[1] Ocular pemphigoid,[2] and Scarring pemphigoid[1]) |
| Specialty | Dermatology  |
Mucous membrane pemphigoid is a rare chronicautoimmune subepithelialblistering disease characterized by erosivelesions of themucous membranes andskin.[3] It is one of thepemphigoid diseases that can result in scarring.[4]
The autoimmune reaction most commonly affects theoral mucosa in the mouth, causing lesions in thegums (gingiva), known asdesquamative gingivitis. More severe cases can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals, anus, andcornea.[5] When the cornea of the eye is affected, repeated scarring may result in blindness.
Brunsting–Perry cicatricial pemphigoid is a rare variant of mucous membrane pemphigoid involving the scalp and the neck without mucosal involvement. It is proposed by some authors that this be called a variant ofepidermolysis bullosa acquisita.[6][1]
Nikolsky's sign (gentle lateral pressure) on unaffected mucosa or skin raises a bulla. If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In contrast, in Pemphigus, the epithelium tends to disintegrate rather than form a bulla.
Nikolsky's sign is present inpemphigus and mucous membrane pemphigoid, but not inbullous pemphigoid.
In mucous membrane pemphigoid, theautoimmune reaction occurs in the skin, specifically at the level of thebasement membrane, which connects the lower skin layer (dermis) to the upper skin layer (epidermis) and keeps it attached to the body.
When the condition is active, the basement membrane is dissolved by the antibodies produced, and areas of skin lift away at the base, causing hard blisters which scar if they burst. In other words, this is a desquamating/blistering disease in which theepithelium "unzips" from the underlying connective tissue, allowing fluid to gather that subsequently manifest asbullae, or blisters.
Various forms of MMP have been described with the most common being, classic/oral MMP where antibodies attack the C-Terminus of BPAG-2 (BP180).[7] In the ocular form, the antigen identified is theβ4 subuniti ofα6β4 integrin. One other notable form is the anti-epillegrin form of MMP, where the target antigen is Laminin 332.[8]
Diagnostic techniques:
The management depends upon the severity of the condition. For example, where there are lesions in the mouth alone,systemic drugs are less likely to be used. Where the condition is not limited to the mouth, or where there is poor response toTopical treatments, systemic drugs are more likely to be used.[9]
Simple measures that can be taken include avoidance of hard, sharp or rough foods, and taking care when eating. Goodoral hygiene is also usually advised, and professional oral hygiene measures such asdental scaling.[9]
Topical andintralesional (injected into the affected areas)corticosteroid drugs may be used, such asfluocinonide,clobetasol propionate ortriamcinolone acetonide.Oral candidiasis may develop with long term topical steroid use, and sometimesantimycotics such asmiconazole gel orchlorhexidine mouthwash are used to prevent this. Topicalciclosporin is sometimes used.
Dapsone is sometimes used as asteroid sparing agent. The dose is often increased very slowly in order to minimize side effects. Systemic steroids, such asprednisone orprednisolone may be needed in severe cases. Many other drugs have been used to treat mucous membrane pemphoid, includingazathioprine,cyclophosphamide,methotrexate,thalidomide,mycophenolate mofetil,leflunomide,sulphasalazine,sulphapuridine,sulphamethoxypiridazine,tetracyclines (e.g.minocycline,doxycycline) andnicotinamide.[9]
Plasmapheresis appears to help some cases. Sometimes surgical procedures are required to repair scars, prevent complications such as blindness, upper airway stenosis or esophageal stricture.[9]