S130 – Otolaryngologic Manifestations in Penphigus Vulgaris

Abstract

Objectives Pemphigus vulgaris is an autoimmune disease characterized by mucocutaneous intraepithelial blisters and pathogenic autoantibodies against desmoglein 3. There are 2 clinical forms: mucosal and mucocutaneous. The frequency of ear, nose and throat involvement in pemphigus vulgaris is not clearly defined. Only a few individual cases have been reported. The objective of this study was to determine the incidence of otolaryngologic involvement in patients with pemphigus. Methods We have studied prospectively all 18 patients diagnosed with PV and treated by Otolaryngology and Dermatology departments of the University Hospital of Navarra between 2001 and 2007. They were 10 cases of mucosal pemphigus and 8 cases of mucocutaneous pemphigus. All patients were evaluated by endoscopic examination. Results 15 patients presented with throat symptoms (83%), 13 pharyngeal (72%), and 8 laryngeal symptoms (44%). 16 patients (88%) had active pemphigus vulgaris lesions localized in pharyngeal and laryngeal mucosa. Laryngeal lesions were most commonly present in mucocutaneous patients. The frequency of nasal symptoms (38%) was lower than active pemphigus vulgaris lesions (63%). Oral symptoms and oral active lesions were the most frequent findings (94%). Only in 3 patients were sown erosions on the external auditory canal. Conclusions Endoscopic evaluation in patients affected by pemphigus vulgaris allows to study more extensive areas of mucosa. By obtaining more complete information concerning the extent of the disease, a more accurate diagnosis can be made, better choice of drug and dose may be decided, and ultimately, response to treatment may be improved.