Acquired Bullous Disease

摘要: Pemphigus is a group of autoimmune blistering diseases that affect the skin and mucous membranes has three major forms: pemphigus vulgaris, foliaceus, paraneoplastic pemphigus. It characterized histologically by intraepi-thelial blister formation caused loss cell-to-cell adhesion keratinocytes (acan-tholysis) immunopathologically presence tissue-bound circulating IgG autoantibodies directed against cell surface or desmogleins. Desmogleins (Dsg1 Dsg3) are target antigens in pemphigus, clinical pheno-type correlated with patient's antides-moglein autoantibody profile. Paraneoplastic occurs association underlying neoplasms shows severe oral conjunctival erosions polymorphous eruptions. Paraneoplastic humoral (IgG autoan-tibodies Dsg3, Dsg1, plakin molecules), as well cellular, autoimmunity. Bulbous pemphigoid subepidermal disease most commonly elderly. The presentation typically pruritic eruption widespread tense blisters. It vivo bound C3 at dermoepidermal junction. The bullous BP230(BPAG1 BP230) BP180(BPAG2 type XVII collagen), which found hemidesmosomes along basement membrane zone. Epidermolysis acquisita rare acquired associated VII collagen, component anchoring fibrils. Systemic corticosteroids mainstay therapy for disease, adjuvant therapies, including immunosup-pressive agents, plasmapheresis, high-dose intravenous immunogloblin, biological used cases.