A Case of Mucosal Dominant Pemphigus Vulgaris

抄録

Pemphigus is an intractable disease of the oropharyngeal mucosa and is classified into vulgaris and foliaceus. The incidence is relatively low and early diagnosis of pemphigus vulgaris is difficult due to the clinical and pathological diversity of this disease.
We report herein on a case of mucosal dominant pemphigus vulgaris occurring in the oropharyngeal and laryngeal mucosa. A 73-year-old woman complaining of a blister on oral mucosa visited an otolaryngologist and was diagnosed as having stomatitis. Despite standard treatment, the lesion did not improve and skin erosion was observed. The patient then visited a dermatologist and was referred to the dermatology department of our hospital with the diagnosis of pemphigus vulgaris. Since she complained of a sore throat, swallowing pain and hoarseness, she was asked to have an examination at our department.
Local examination showed multiple erosion in the oropharyngeal and laryngeal mucosa. A lower lip biopsy was performed and the anti-desmoglein antibody titer in the serum was examined. The histopathological findings were consistent with pemphigus vulgaris and an increased anti-desmoglein antibody 3 titer was observed. The patient then underwent corticosteroid treatment and her symptoms remarkably improved. Although pemphigus vulgaris is rarely seen by otolaryngologists, it is suggested that pemphigus vulgaris should be considered as one of the differential diagnosis when intractable stomatitis is observed.