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Necrotizing Tracheobronchitis With Progressive Airflow Obstruction Associated With Paraneoplastic Pemphigus

James P. Osmanski, II; Armando E. Fraire; Oren P. Schaefer
Author and Funding Information

Affiliations: From the Division of Pulmonary, Allergy and Critical Care Medicine, University of Massachusetts Medical Center, Worcester,  From the Division of Anatomic and Surgical Pathology, University of Massachusetts Medical Center, Worcester

Affiliations: From the Division of Pulmonary, Allergy and Critical Care Medicine, University of Massachusetts Medical Center, Worcester,  From the Division of Anatomic and Surgical Pathology, University of Massachusetts Medical Center, Worcester


1997 by the American College of Chest Physicians


Chest. 1997;112(6):1704-1707. doi:10.1378/chest.112.6.1704
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Abstract

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.


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