Pemphigoid (MMP): A Chronic Autoimmune Disease

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Mucous membrane pemphigoid (MMP) encompasses a heterogeneous group of diseases which predominantly affects the elderly. The peak incidence is at roughly 70 years however, rare childhood cases have also been reported. There is no racial or ethnic predilection associated with MMP but the condition does appear to be twice as common in women than it is in men. For completeness, MMP is also termed cicatricial pemphigoid, oral pemphigoid and ocular pemphigoid. MMP is a chronic autoimmune disease that is recognized by sub-epithelial blistering lesions and eventual scarring of the mucous membranes, skin, or even both. Affected membranes may include the conjunctiva, oesophagus, trachea, nasopharynx, larynx, genitourinary tract, and anus. Of these, the most commonly affected are the oral mucosa and conjunctiva. In less common cases the skin may also be involved – 20-30% of cases …show more content…

It is an autoimmune disorder i.e. it occurs when the body produces autoantibodies against healthy ‘self’ tissue. In MMP, these produced autoantibodies react with target proteins (antigens) located in mucous membranes and skin tissue. More specifically, the autoantibodies attack the basement membrane zone (BMZ) of the epithelium. The BMZ functions in holding the outer layer of skin onto the underlying tissues therefore, when the BMZ is attacked and destroyed by the autoantibodies, the skin is no longer anchored down, allowing it to lift off and produce blisters.Immunological studies have revealed the precise targets of these autoantibodies are antigens which include BP180 and laminin 5 amongst other suggestions such as the subunits of the α6β4 integrins, Collagen VII and BP230. It has been suggested that affected individuals may have a genetic susceptibility to the development of some forms of MMP. Of course, the development of such cases would likely be multifactorial by requiring contribution of immunological, genetic, environmental, and other several

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