2022 Volume 21 Issue 2 Pages 108-113
A 52-year-old man had undergone medications of oral prednisolone and cyclosporine for pemphigus vulgaris for 7 years. Mucocutaneous bulla and erosions recurred gradually, Pemphigus Disease Area Index (PDAI) was moderate with 10 points on the skin and two points on the mucous membrane, but it was intractable. After hospitalization, sore throat and dyspnea required endoscopy. It revealed mucosal erosions, ulcers, and lacerations over the entire length of the esophagus with Dsg1 and Dsg3 antibodies elevated to 630 units and 2,540 units, respectively. The esophageal lesions were coincident with mucosal pemphigus vulgaris. Oral corticosteroids and azathioprine, plasmapheresis, and high-dose intravenous immunoglobulin reduced mucosal lesions 41 days later. Our case emphasizes the necessity of performing endoscopy to examine esophageal involvement in cases of severe mucosal pemphigus vulgaris. Moreover, aggressive initial treatment is important in severe cases. Skin Research, 21 : 108-113, 2022
