Mucosal manifestations of pemphigus vulgaris in ear, nose, and throat; before and after treatment

BackgroundPemphigus vulgaris (PV) is a life-threatening autoimmune bullous disease, mediated by autoantibodies directed against antigens on the keratinocyte cell surface of stratified squamous epithelia. The frequency of ear, nose, and throat (ENT) involvement in PV is not clearly identified.ObjectiveWe sought to evaluate the ENT involvement in new patients with PV examined by ENT endoscopy before and after treatment.MethodsThis study included 41 new patients with PV. All patients were evaluated for ENT manifestations by otoscopic and endoscopic examination before treatment. After these examinations, the patients received proper treatment according to standard protocols. Thirty patients were re-evaluated by endoscopic examination after clinical remission of mucocutaneous signs.ResultsA total of 41 (33 [80.5%] male and 8 [19.5%] female) patients with mean age of 44.56 ± 12.76 years entered this study. In ENT examination of patients before the treatment, 11 (26.8%) patients showed ear, 15 (36.6%) nasal, 37 (90.3%) oral, 25 (61%) pharyngeal, and 24 (58.5%) laryngeal involvement. Thirty patients underwent the posttreatment ENT examination. We found ear signs in 2 (6.7%), nasal involvement in 1 (3.3%), oral signs in 4 (13.3%), pharyngeal manifestations in 6 (20%), and laryngeal signs in 3 (10%) patients after treatment. The treatment was significantly effective in the improvement of mucosal lesion in different sites (P < .01).LimitationEleven patients refused to undergo posttreatment ENT examination.ConclusionENT evaluation may be worthwhile to evaluate the disease extension in patients with PV more definitely and exclude other potential etiologies in recalcitrant patients.