Current treatment of autoimmune blistering diseases

M Kasperkiewicz, E Schmidt - Current drug discovery …, 2009 - ingentaconnect.com
M Kasperkiewicz, E Schmidt
Current drug discovery technologies, 2009ingentaconnect.com
Autoimmune bullous diseases are a heterogeneous group of disorders that can be
subdivided according to the level of split formation in the intraepidermal blistering
pemphigus diseases and subepidermal bullous disorders, latter including pemphigoid
diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. In the majority of
autoimmune bullous disorders, disease activity can be sufficiently controlled by systemic
corticosteroids in combination with further immunosuppressants/immunomodulants such as …
Autoimmune bullous diseases are a heterogeneous group of disorders that can be subdivided according to the level of split formation in the intraepidermal blistering pemphigus diseases and subepidermal bullous disorders, latter including pemphigoid diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. In the majority of autoimmune bullous disorders, disease activity can be sufficiently controlled by systemic corticosteroids in combination with further immunosuppressants/immunomodulants such as dapsone, doxycycline, methotrexate, azathioprine, or mycophenolate mofetil. In contrast, in pemphigus, mucous membrane pemphigoid, and epidermolysis bullosa acquisita, treatment is challenging and only in a minority of patients, conventional immunosuppressive therapy induces clinical remission. Since only a few years ago, only cyclophosphamide and high-dose intravenous immunoglobulin were available as potent second-line therapies. Meanwhile, immunoadsorption and the monoclonal anti-CD20 antibody rituximab have been established as further therapeutic options. Here, both conventional therapies and novel treatment regimens for autoimmune blistering diseases are discussed.
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