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pemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. and
pemphigoidPemphigoid is a group of subepidermal, blistering autoimmune diseases that primarily affect the skin, especially the lower abdomen, groin, and flexor surfaces of the extremities. Here, autoantibodies (anti-BPA-2 and anti-BPA-1) are directed against the basal layer of the epidermis and mucosa. The condition tends to persist for months or years with periods of exacerbation and remission. Localized variants of the condition have been reported, most often limited to the lower extremities and usually affecting women. There are two predominant types of pemphigoid: mucous membrane pemphigoid (MMP) also called cicatricial pemphigoid, and bullous pemphigoid (BP). Pathogenesis and management are quite different for these conditions. Scar formation in mucous membrane pemphigoid can lead to major disability. patients and programs.
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