The Mechanism of Respiratory Failure in Paraneoplastic Pemphigus

Paraneoplastic pemphigus1 is an autoimmune disease that accompanies an overt or occult neoplasm and causes blisters. It is characterized by the presence of IgG autoantibodies that react against desmosomal and hemidesmosomal plakin proteins,2–5 desmosomal transmembrane proteins (desmogleins),6 and an unidentified 170-kd antigen. Blistering of stratified squamous epithelium results from acantholysis, the loss of cell–cell adhesion, induced by pathogenic antibodies against the desmogleins.6 The most commonly associated neoplasms are, in decreasing order of frequency, non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castleman's disease, thymoma, retroperitoneal sarcomas, and Waldenstrom's macroglobulinemia. Progressive respiratory failure with clinical features of bronchiolitis obliterans is frequently the cause . . .