Sclerosing Esophagitis with IgG4-positive Plasma Cell Infiltration

Abstract

The patient was a 76-year-old woman who had noticed slight difficulty in swallowing in the 3 years prior to this presentation. Her dysphagia progressed while she was hospitalized following cervical cancer surgery. Esophagogastroduodenoscopy and an esophagram showed circumferential erosion and a stricture of the thoracic esophagus. Esophageal resection was performed; the resected specimens showed a stricture and wall thickening. Histologically, transmural hyperplasia, which consisted of inflammatory granulation tissue with the abundant infiltration of IgG4-positive plasma cells and lymphocytes, was observed. The patient was diagnosed with probable IgG4-related disease. IgG4-related esophageal disease presenting as esophageal lesions alone is a very rare condition.

Keywords: IgG4-positive plasma cell; IgG4-related disease; esophageal stenosis; esophagitis.

Figure 1.

Esophagogastroduodenoscopy showed circumferential erosion and stricture of the thoracic esophagus. The surface of the stenotic lesion was relatively smooth, and the marginal mucosa was cloudy. Several ulcer scars were observed at the oral mucosa.

Figure 2.

a: A gross examination of the resected specimens showed a stricture with mucosal erosion and transmural wall hypertrophy measuring 50 mm (in length) in the middle of the thoracic esophagus. The stricture was contracted without deep ulceration. b, c: The histopathological features. A low magnification view revealed marked inflammatory granulation tissue and fibrosis from the epithelium to the proper muscle layer. High magnification revealed abundant plasma cells and fibrosis in the submucosal layer (Hematoxylin and Eosin staining, original magnification 2b: ×1.25, 2c: ×20). d: An immunohistochemical study showed IgG4-positive plasma cells at a level of >10/high powered field and the ratio of IgG4+/IgG+plasma cells was >40%.