Idiopathic Esophageal Ulcer

A 36-year-old homosexual man presented with a 2-week history of odynophagia to liquids and solids; he had no dysphagia or heartburn. The patient, who had been seropositive for HIV for 3 years, had refused all antiretroviral drugs and prophylactic agents against opportunistic infections.

Lucia C. Fry, MD, and Klaus E. Mönkemüller, MD, of Chandler, Ariz, noted white plaques consistent with thrush that covered the tongue and oropharynx. The patient’s CD4+ cell count was 50/µL.

Esophagogastroduodenoscopy demonstrated a well-circumscribed ulcer with raised borders in the mid esophagus (arrow). Histopathologic examination of esophageal biopsy specimens showed an ulcer bed of granulation tissue; no cytoplasmic inclusions, granulomas, vasculitic changes, or microorganisms were seen. Immunohistochemistry stains were negative for intracytoplasmic and intranuclear inclusions; special stains yielded no fungi or mycobacteria. Idiopathic esophageal ulcer (IEU) was diagnosed.

Candida species are the most common causes of esophagitis in HIV-infected patients; however, these fungi rarely cause esophageal ulcers. Cytomegalovirus (CMV) causes about 50% of esophageal ulcers in HIV-infected patients; herpes simplex virus (HSV) is a less common cause of ulcerative esophagitis in these patients. About 25% of cases of esophageal ulcers have no specifically identified microbial cause.

The pathogenesis of IEU is unknown. Esophageal ulcers may be present at the time of HIV seroconversion; however, they usually occur in patients with severe immunodeficiency who have CD4+ cell counts lower than 50/µL.

Odynophagia and dysphagia are the main symptoms of IEU and of ulcerative esophagitis from other causes. The diagnosis of IEU is one of exclusion; multiple biopsies of the ulcer margins and base are needed to exclude an infectious process. Oral prednisone, 40 mg/d tapered to 10 mg/wk for 1 month, is the most common therapy. This agent has a healing rate of more than 90% and provides significant pain relief within days.¹ Concurrent therapy with a systemic azole preparation can be used to prevent Candida esophagitis and treat it in patients who are coinfected with Candida and CMV or HSV.

This patient’s symptoms resolved during the first 3 days of treatment with prednisone. He was also given fluconazole for the thrush. At follow-up 2 months later, the patient was asymptomatic.