Esophageal ulcers in immunodeficiency with elevated levels of IgM and neutropenia

A 17-year-old boy with immunodeficiency, elevated levels of IgM,and neutropenia developed distal esophageal ulcers and stricture. Although his lower esophageal sphincter pressure was decreased, he had normal acid reflux test results, normal findings from acid clearing studies, and absence of diffuse esophagitis at esophagoscopy. Neutropenia and hypogammaglobulinemia are postulated as pathogenic factors in his esophageal ulcers.