Ear, nose and throat manifestations in pemphigus vulgaris

Abstract

Background: Pemphigus vulgaris (PV) is an autoimmune disease characterized by mucocutaneous intraepithelial blisters and pathogenic autoantibodies against desmoglein 3. There are two clinical forms: mucosal (MPV) and mucocutaneous (MCPV). The frequency of ear, nose and throat (ENT) involvement in PV is not clearly defined. Only a few isolated individual cases have been reported.

Objectives: The objective of our study was to determine the incidence of ENT involvement in patients with PV.

Patients: We studied prospectively all 16 patients diagnosed with PV and treated in the Department of Dermatology of the University Clinic of Navarra between 2001 and 2005. They were 10 cases of MPV and six cases of MCPV. All patients were evaluated for ENT manifestations by endoscopic examination.

Results: Of the 16 patients, 13 presented with throat symptoms (81%), 12 pharyngeal (75%) and seven laryngeal symptoms (44%). Fourteen patients (88%) had active PV lesions on endoscopic evaluation (eight patients had active lesions on both pharyngeal and laryngeal mucosa, four had PV lesions only on laryngeal mucosa and two had PV lesions on pharyngeal mucosa). Laryngeal lesions were most commonly present in MPV patients. The frequency of nasal symptoms (38%) was lower than active PV lesions (62%) found on ENT examination. Oral symptoms and oral active PV lesions were the most frequent findings (94%). Only three patients with MCPV showed erosions on the external auditory canal.

Conclusions: As ENT endoscopy allows more extensive areas of mucosa to be examined than simple visual inspection, we recommend that it be included in the examination of all patients with PV. 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.