A Rare Case of Idiopathic Hypereosinophilic Syndrome Involving the Oral Cavity Associated with the Esophagus and Gastrointestinal Tract

Abstract

We report the rare case of HES involving oral cavity associated with esophagus, and gastrointestinal tract, which we succeeded in diagnosing precisely through a biopsy specimen obtained from the lip. A 64-year-old man had dysphagia, swelling of the oral mucosa and the posterior cervical muscles, accompanied by an abdominal pain and diarrhea. Peripheral blood cell count showed marked eosinophilia. Case ReportA 64-year-old man had a 9-day history of dysphagia and swelling of the oral mucosa and posterior cervical muscles. Also he had abdominal pain and diarrhea for 4 days.He had bronchial asthma for the previous 1 year, which was controlled with regular inhalations of budesonide and oral theophyline and occasional inhalations of salbutamol. There were no recent infections or relevant travel history.At admission, he was unwell with a low-grade fever up to 37.1°C, swelling of oral mucosa (Fig. 1), and frequent bowel movement. Because of dysphagia and severe swelling of the oral mucosa, he could not eat any food.Laboratory examinations revealed an excess of white blood cells, 17,800/mm , with marked eosinophilia, and 52.7% eosinophils. Serum IgE concentrations were also elevated at 1,785 IU/ml (normal <310 IU/ml). Radioallergosorbent tests for multiple allergens were negative.Other normal laboratory values included antinuclear antibody, anti-DNA antibody, and complement titer.

Eosinophilic disorders of the gastrointestinal tract: imaging features

“…The involvement of the pharynx and larynx in eosinophilic esophagitis is extremely rare in adults (14). Airway involvement is reported more commonly in children with eosinophilic esophagitis, and 15% of children with eosinophilic esophagitis have airway and extra-intestinal symptoms (15).…”

Eosinophilic esophagitis—established facts and new horizons

“…Hypereosinophilic syndrome (HES) is defined as a an eosinophil count more than 1500 cells/ml without secondary causes for at least 1 month and evidence of end organ manifestation attributable to the eosinophilia [ 147 ]. It is characterized by multiorgan system infiltration by eosinophils, but may involve esophageal tissue resulting in EE with dysphagia [ 148 , 149 ].…”

Hypereosinophilic Syndrome vs Eosinophilic Gastroenteritis

“…In the case of Watanabe et al (8), eosinophilic infiltrations occurred in the oral cavity as well as in the esophagus and gastrointestinal tract, consisted of eosinophilic infiltration deeply in muscle layer. They defined this patient as idiopathic hypereosinophilic syndrome, rather than eosinophilic gastroenteritis (8).…”