Paraneoplastic pemphigus

Paraneoplastic pemphigus is a rare but severe potentially fatal 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 macroglobuline 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 it's rarity, there are no guidelines for the treatment of PNP. 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.