A Case of Pemphigus Vulgaris with Recurrent Laryngeal Edema

Abstract

Pemphigus is an autoimmune bullous disease characterized by lesions on the skin and mucous membranes. Characteristic clinical findings include painful and intractable erosions and ulcers in the oral cavity, but when lesions are not present on the skin but only on the oral mucosa, diagnosis is often difficult. Although laryngeal lesions are believed to be uncommon as the initial manifestation of pemphigus vulgaris, it is necessary to keep in mind the possibility.

We report a rare case of pemphigus vulgaris diagnosed in a patient who initially presented with recurrent laryngeal edema, followed subsequently by the appearance of mucosal lesions. The patient, a 75-year-old man, presented to the otorhinolaryngologist with a 2-month history of pharyngeal pain. Examination revealed only laryngeal edema, which improved after steroid administration. During the subsequent course, however, oral mucosal lesions appeared, which led to the diagnosis of pemphigus and treatment appropriate for this disease, and the patient was successfully treated without becoming severely ill.

In this case, there were no oral mucosal lesions or skin rash at the time of the initial examination, which made the diagnosis difficult. In the case of pemphigus vulgaris of the mucocutaneous type, since findings may initially appear only in the otorhinolaryngological field, it is important to keep in mind not only otorhinolaryngological diseases, but also systemic diseases and to seek dermatology referral for diagnosis and treatment when the disease follows an atypical course.