Paraneoplastic pemphigus (Q43773)

Paraneoplastic pemphigus is a severe, often fatal autoimmune disease characterised by blisters and erosions not only on the skin and the mucous membranes but also involving other organs including the respiratory system. Auto-antibodies to a variety of plakin components of desmosomes and hemidesmosomes and to the protease inhibitor, Alpha-2-macroglobulin-like-1 protein, have all been implicated in its pathogenesis. It is strongly associated with lymphoproliferative disease.