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Hello! I would like to learn something new. Given that only begins to study medicine interested me the next area. My current occupation is about bullous pemphigoid and pemphigus vulgaris. In that case I would like to know some basic info about this disease. And what is the incidence and mortality in the world? Thanks for your possible answer about this.

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Hi! Bullous pemphigoid is an autoimmune skin disorder producing chronic, pruritic bullous eruptions in elderly patients. Diagnosis is by skin biopsy. Corticosteroids are used initially. Most patients require long-term maintenance therapy, for which a variety of drugs can be used. In bullous pemphigoid, antibodies are directed against the basement membrane zone of the epidermis, causing separation between the epidermis and dermis. Bullous pemphigoid must be distinguished from pemphigus vulgaris which is an uncommon, potentially fatal, autoimmune disease characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. Diagnosis is by skin biopsy with direct immunofluorescence testing. Treatment is with corticosteroids and sometimes immunosuppressants. Pemphigus vulgaris usually occurs in middle-aged or elderly patients and is rare in children. One variant, paraneoplastic pemphigus, occurs in older patients with malignancy (primarily lymphoreticular); outcome is poor. The disease is characterized by the presence of autoantibodies directed against intercellular adhesion molecules desmoglein-1 and desmoglein-3 in the epidermis. They are Ca-dependent cadherins, involved in adhesion and cell signaling between epidermal cells. Acantholysis result from either direct inhibition of function of the desmogleins by autoantibody binding or from autoantibody-induced cell signaling that results in down-regulation of cell-cell adhesion and formation of blisters. These autoantibodies are present in both serum and skin during active disease. Any area of stratified squamous epithelium may be affected, including mucosal surfaces.
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