Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. It is one of the pemphigoid diseases that can result in scarring. Signs and symptoms
The autoimmune reaction most commonly affects the oral mucosa in the mouth, causing lesions in the gums (gingiva), known as desquamative gingivitis. More severe cases can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals, anus, and cornea. 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 of epidermolysis bullosa acquisita.Nikolskys 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. Nikolskys sign is present in pemphigus and mucous membrane pemphigoid, but not in bullous pemphigoid. Pathophysiology
In mucous membrane pemphigoid, the autoimmune reaction occurs in the skin, specifically at the level of the basement membrane, which connects the lower skin layer (dermis) to the upper skin layer (epidermis) and keeps it attached to the body.
The dose is often increased very slowly in order to minimize side effects. Systemic steroids, such as prednisone or prednisolone may be needed in severe cases. Many other drugs have been used to treat mucous membrane pemphoid, including azathioprine, cyclophosphamide, methotrexate, thalidomide, mycophenolate mofetil, leflunomide, sulphasalazine, sulphapuridine, sulphamethoxypiridazine, tetracyclines (e.g. minocycline, doxycycline) and nicotinamide. Other treatments
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. See also
Gestational pemphigoid
List of cutaneous conditions
List of target antigens in pemphigoid
List of immunofluorescence findings for autoimmune bullous conditions

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