Paraneoplastic pemphigus is a rare but severe autoimmune disease characterized by severe stomatitis and a variety of cutaneous manifestations in association with an underlying neoplasia. Pulmonary involvement may also occur. The pathogenesis involves the production of autoantibodies against desmogleins, plakins, and the protease inhibitor alpha-2-macroglobulin-like 1, but T-cell-mediated autoimmunity is also thought to play a role. Diagnosis usually relies on the demonstration of a specific subset of circulating autoantibodies in patient serum, although in a small subset of patients, these autoantibodies might be absent. Due to its rarity, there are no set of guidelines for treatment. The general approach includes a variety of immunosuppressive agents and treatment of the underlying neoplasia. Despite treatment, paraneoplastic pemphigus has high mortality rates, often due to sepsis, respiratory failure, or progression of the underlying malignancy.
|Titel||Autoimmune bullous diseases|
|Subtitel||Text and Review|
|Uitgeverij||Springer International Publishing|
|ISBN van elektronische versie||978-3-319-23754-1|
|ISBN van geprinte versie||978-3-319-23753-4|
|Status||Published - 2016|