Background: The presence of lesions of pemphigus vulgaris in the larynx and nasal cavity has been reported in individual case reports. However, the frequency with which these sites are involved is not known. Objective: Our purpose was to investigate the incidence of laryngeal and nasal disease involvement in patients with pemphigus vulgaris. Methods: This was a retrospective analysis conducted in a referral private practice in an academic department of dermatology. A total of 53 sequential patients with pemphigus vulgaris diagnosed by clinical, histologic, and immunofluorescence criteria were selected on the basis of having been treated by one of us (J. C. B.) during most of their illness. Patients' charts were reviewed for documentation of laryngeal and nasal symptoms, ear/nose/throat evaluation, and response to treatment. Results: Twenty-six (49%) of the patients complained of laryngeal or nasal symptoms at some time during the course of their disease. Twenty-one patients had laryngeal symptoms and 12 had nasal symptoms. More than 80% of patients with laryngeal or nasal symptoms had evidence of pemphigus involvement based on ear/nose/throat examination or rapid response to increased doses of corticosteroids, and 2 patients had candidiasis confirmed by fungal culture. Conclusion: These observations indicate that laryngeal and nasal symptoms are common in pemphigus vulgaris. In the majority of cases, this appears to be a result of involvement with the disease or with candidiasis. (J Am Acad Dermatol 2001;44:609-11.)