Abstract
A 66-year-old woman with a 2-week history of sore throat that did not improve with a course of antibiotic treatment prescribed at a neighborhood clinic, visited our department. Examination revealed hemorrhagic erosions in the oral cavity, pharynx, larynx. We suspected mucosal erosions secondary to infection, and started the patient on injectable antibiotic treatment on an outpatient basis, however, the erosions remained persistent. Since antibody tests for various potentially causative viruses as well as bacterial culture tests were negative, the mucosal lesions were excluded as being caused by bacterial/viral infections. Since pemphigus was suspected based on the Nikolsky phenomenon being elicited by suction of the buccal mucosa, we obtained biopsy specimens from the lesions. Histopathology confirmed the presence of acantholysis, and immunostaining of the specimens revealed IgG deposition around the spinous cells. We further confirmed the diagnosis of mucosal-dominant pemphigus vulgaris based on blood test results that were negative for anti-desmoglein 1 antibody and positive for anti-desmoglein 3 antibody. Pemphigus vulgaris tends to present with oral mucosal lesions as the earliest symptom, although the diagnosis tends to be delayed in patients presenting with only oral involvement as compared with that in patients presenting with skin lesions. One of the reasons why the diagnosis of pemphigus vulgaris could be made relatively early in our case was that we were able to elicit the Nikolsky phenomenon by suctioning the buccal mucosa which seemed to be normal. Thus, attempts to elicit the Nikolsky phenomenon may be an important diagnostic tool for otolaryngologists faced with pemphigus vulgaris patients presenting in the early stage of the disease.
