IgG4-Related Disease with Esophageal Involvement

We report the case of a 56-year-old man who presented with dysphagia and weight loss. An esophagoduodenoscopy revealed a severe esophageal stricture in the distal esophagus. After surgical resection, the final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammatory cells with an increase and predominance of IgG4-positive plasma cells. He did not, however, have any other symptoms indicative of systemic autoimmune disease or connective tissue disorders. Histologically, abundant infiltration of IgG4-positive plasma cells and lymphocytes was observed. The patient was diagnosed with IgG4-related disease, definitive, with esophageal involvement.