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Case Reports
. 2014 Aug;6(4):295-8.
doi: 10.14740/jocmr1845w. Epub 2014 May 22.

IgG4-Related Esophageal Disease Presenting as Esophagitis Dissecans Superficialis With Chronic Strictures

Myriam Dumas-Campagna  1 , Simon Bouchard  1 , Genevieve Soucy  2 , Mickael Bouin  1
Affiliations
Case Reports

IgG4-Related Esophageal Disease Presenting as Esophagitis Dissecans Superficialis With Chronic Strictures

Myriam Dumas-Campagna et al. J Clin Med Res. 2014 Aug.

Abstract

IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmocytic infiltrate of IgG4-positive cells, storiform fibrosis and can be associated with tumefactive lesions. IgG4-related disease involving the upper gastrointestinal tract is rare and only two previous case reports have reported IgG4-related esophageal disease. We report the case of a 63-year-old female patient with a long-standing history of severe dysphagia and odynophagia with an initial diagnosis of reflux esophagitis. Symptoms persisted despite anti-acid therapy and control esophagogastroduodenoscopy (EGD) revealed endoscopic images consistent with esophagitis dissecans superficialis (sloughing esophagitis). An underlying autoimmune process was suspected and immunosuppressant agents were tried to control her disease. The patient eventually developed disabling dysphagia secondary to multiple chronic esophageal strictures. A diagnosis of IgG4-related disease was eventually made after reviewing esophageal biopsies and performing an immunohistochemical study with an anti-IgG4 antibody. Treatment attempts with corticosteroids and rituximab was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. Our case report describes this unique case of IgG4-related esophageal disease presenting as chronic esophagitis dissecans with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease.

Keywords: Autoimmune esophagitis; Esophagitis dissecans; IgG4-related disease; Sclerosing esophagitis.

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Figures

Figure 1
Figure 1
Barium swallow showing a short esophageal stenosis at the C5-C6 level and a long esophageal stenosis in the mid esophagus.
Figure 2
Figure 2
Histologic image of an esophageal biopsy (× 10) revealing a dense lymphoplasmocytic infiltrate. Hematoxylin phloxine saffron stain.
Figure 3
Figure 3
Histologic image (× 40) of the esophagus with many IgG4-positive plasma cells.
See this image and copyright information in PMC

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