Necrotizing tracheobronchitis with progressive airflow obstruction associated with paraneoplastic pemphigus
Division of Pulmonary; Department of Pathology
Airway Obstruction; Anoxia; Autoimmune Diseases; Biopsy; Bronchi; Bronchitis; Diagnosis, Differential; Humans; Lymphoma, Non-Hodgkin; Male; Middle Aged; Necrosis; Paraneoplastic Syndromes; Pemphigus; Skin; Tracheitis
Life Sciences | Medicine and Health Sciences
Paraneoplastic pemphigus (PNP) is an autoimmune disease associated with leukemia and non-Hodgkin's lymphoma. A patient with stage IVB poorly differentiated lymphocytic lymphoma developed characteristic upper and lower airway involvement with profound mucocutaneous erosion and tracheobronchial epithelial desquamation. Immunofluorescence testing confirmed autoantibody deposition along the basement membrane of bronchial epithelium. Disruption of the cellular adhesion mechanisms, including desmosomes, hemidesmosomes, and possibly the integrin subunits, is presumed to have led to disruption and desquamation of the tracheobronchial epithelial barrier, severe obstruction of the airways and hypoxia, and possibly bacterial superinfection. As far as can be determined, the feature of airflow obstruction occurring in association with PNP has not been described. Physicians should be aware that these complications of PNP may rapidly lead to hypoxic respiratory failure and death.
Chest. 1997 Dec;112(6):1704-7.
Osmanski, James P. II; Fraire, Armando E.; and Schaefer, Oren P., "Necrotizing tracheobronchitis with progressive airflow obstruction associated with paraneoplastic pemphigus" (1997). Open Access Articles. 429.