SESSION TITLE: Obstructive Airway Disease Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Cicatricial pemphigoid is a chronic inflammatory disease that frequently involves the eyes and upper airway mucosa but very rarely involves the lower airways. We report a patient with CP involving the eyes and sinuses, with worsening obstructive lung disease.
CASE PRESENTATION: A 52-year-old man with biopsy-proven CP involving the eyes and paranasal sinuses was referred for pulmonary consultation for poorly controlled asthma. His CP had been treated with prednisone, mycophenolate, cyclophosphamide and multiple eye surgeries but despite this had progressed to blindness. Direct immunofluorescence of a nasal biopsy showed linear deposits of IgA and trace C3 at the basement membrane zone consistent with CP. He was started on rituximab with improvement in sinusitis. However, cough, exertional dyspnea, and obstructive airway disease worsened despite increasing doses of prednisone. There was no evidence for upper airway obstruction. Bronchoscopic evaluation revealed severe diffuse erythema and edema and patchy whitish plaques from the mid-trachea to the fourth generation airways, as well as scarring and stenosis of some subsegmental bronchi. Standard pathologic evaluation demonstrated focal acute inflammation, and microbiology studies were negative. Histological and immunofluorescence staining were consistent with lower airway involvement with CP.
DISCUSSION: CP is a heterogeneous group of chronic autoimmune subepithelial blistering diseases primarily involving mucosal surfaces of the oral cavity, conjunctivae of the eye, or mucous membranes of the nose, larynx, pharynx, esophagus, anus, and genitals. Ocular involvement with scarring can lead to blindness, and laryngotracheal involvement can result in life-threatening stenosis and obstruction. Diagnosis is based on clinical assessment of typical mucocutaneous lesions as well as biopsy with direct immunofluorescence showing linear IgG, IgA, and/or C3 staining along the basement membrane zone. Involvement of the lower airways is very rare, and in this patient was clinically manifest as steroid-resistant asthma. To our knowledge there are no other case reports of DIF microscopy consistent with CP from bronchoscopic biopsies.
CONCLUSIONS: Lower airway involvement from CP represents a rare but clinically important entity that can mimic asthma. Evaluation of chronic cough and dyspnea in a patient with CP should involve diagnostic bronchoscopy and endobronchial biopsy with immunofluorescence.
Reference #1: Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013; 381: 320-332.
DISCLOSURE: The following authors have nothing to disclose: Margaret Zambon, Serban Staicu, Michael Nead, Mark Frampton
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