Abstract： A 55-year-old female patient was referred with a complaint of oral ulcers and vegetative plaques on the scalp,neck and axillae for 4 years.Physical examination revealed bean-sized ulcers at the left rim of the tongue as well as hypertrophic and vegetative plaques with a small amount of exudates and an offensive odor on the scalp,neck and both axillae.Pathological examination showed hyperkeratosis,hyperacanthosis,vesicles caused by acantholysis in the upper part of basal layer,massive inflammatory infiltration of neutrophils and lymphocytes in dennis with neutrophil microabscess.Acantholytic cells were observed in the vesicles.Direct immunofluorescence revealed positive staining for immunoglobulin G (IgG) between epidermal prick cells.The diagnosis of pemphigus vegetans was confirmed based on the above findings.A combination therapy with intravenous methylprednisolone 40 mg once a day and oral acitretin 10 mg twice daily were subsequently initiated along with symptom-oriented approaches.After 20-day treatment,the lesions markedly improved with decreased exudates and flattened plaques.