Les maladies bulleuses auto-immunes

摘要: Autoimmune blistering diseases are characterized by the production of pathogenic autoantibodies that responsible for formation epidermal blisters. Major advances in understanding pathogenesis these disorders have allowed development new therapeutic agents. Recent epidemiologic data showed bullous pemphigoid mainly affects elderly patients. Bullous is often associated with degenerative neurologic disorders. A major increase incidence has been observed France. Treatment based on superpotent topical corticosteroids. The role desmosomal proteins demonstrated initiation, propagation and persistence autoimmune response pemphigus. Several studies shown a correlation between anti-desmoglein antibody titers disease activity. Pemphigus susceptibility genes identified. Oral corticosteroids remain mainstay pemphigus treatment. Dramatic long-lasting improvement recently obtained rituximab recalcitrant types Other junctional rare entities, whose prognosis can be severe. Their diagnosis improved use immunological assays immunoelectronic microscopy. Immunosupressants widely used severe order to prevent mucosal sequelae.