Necrotizing Tracheobronchitis With Progressive Airflow Obstruction Associated With Paraneoplastic Pemphigus

• Published in: Chest Vol. 112; no. 6; pp. 1704 - 1707
• Main Authors: Osmanski, James P., Fraire, Armando E., Schaefer, Oren P.
• Format: Journal Article
• Language: English
• Published: Northbrook, IL Elsevier Inc 01.12.1997; American College of Chest Physicians; Elsevier B.V
• Subjects: airway obstruction; Airway obstruction (Medicine); Airway Obstruction - diagnosis; Airway Obstruction - etiology; autoantibody; Autoimmune Diseases - complications; Autoimmune Diseases - diagnosis; Biological and medical sciences; Biopsy; Bronchi - pathology; Bronchitis - complications; Bronchitis - diagnosis; Case studies; Causes of; Complications and side effects; desmosome; Diagnosis, Differential; hemidesmosome; Hemidesmosomes; Humans; Hypoxia - diagnosis; Hypoxia - etiology; Lymphoma, Non-Hodgkin - complications; Lymphoma, Non-Hodgkin - diagnosis; Male; Medical sciences; Middle Aged; Necrosis; necrotizing tracheobronchitis; non-Hodgkin's lymphoma; paraneoplastic pemphigus; Paraneoplastic syndromes; Paraneoplastic Syndromes - complications; Paraneoplastic Syndromes - diagnosis; Pemphigus; Pemphigus - complications; Pemphigus - diagnosis; Pneumology; Pulmonary manifestations of general diseases; Respiratory system : syndromes and miscellaneous diseases; Skin - pathology; Tracheitis - complications; Tracheitis - diagnosis; airway obstruction; autoantibody; non-Hodgkin's lymphoma; necrotizing tracheobronchitis; paraneoplastic pemphigus; desmosome; hemidesmosome; Human; Diseases of the trachea; Respiratory disease; Leukemia; Malignant hemopathy; Non Hodgkin lymphoma; Obstruction; Case study; Respiratory tract; Pathology; Optical microscopy; Association; Tracheitis; Lymphoproliferative syndrome; Bronchitis; Immunofluorescence
• ISSN: 0012-3692; 1931-3543
• DOI: 10.1378/chest.112.6.1704

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.