NECROTIZING TRACHEOBRONCHITIS WITH PROGRESSIVE AIR-FLOW OBSTRUCTION ASSOCIATED WITH PARANEOPLASTIC PEMPHIGUS

Paraneoplastic pemphigus (PNP) is an autoimmune disease associated wit h leukemia and non-Hodgkin's lymphoma. A patient with stage IVB poorly differentiated lymphocytic lymphoma developed characteristic upper an d lower airway involvement with profound mucocutaneous erosion and tra cheobronchial epithelial desquamation. Immunofluorescence testing conf irmed autoantibody deposition along the basement membrane of bronchial epithelium. Disruption of the cellular adhesion mechanisms, including desmosomes, hemidesmosomes, and possibly the integrin submits, is pre sumed to have led to disruption and desquamation of the tracheobronchi al epithelial barrier, severe obstruction of the airways and hypoxia, and possibly bacterial superinfection. As far as can be determined, th e feature of airflow obstruction occurring in association with PNP has not been described. Physicians should be aware that these complicatio ns of PNP may rapidly lead to hypoxic respiratory failure and death.