A rare case of hematemesis

A 39-year-old woman presented with intermittent hematemesis, odynophagia, and repeated oral blistering in the previous 3 years. Gastroscopy did not disclose any lesion during advancement of endoscope but did reveal a diffused hematoma at the upper part of the esophagus when the endoscope was withdrawn (A). Furthermore, circumferential mucosal exfoliation occurred when a biopsy was performed at the site of hematoma (B). Histologic examination of the biopsy specimen showed oral blistering (C, H&E, orig. mag. ×100) and circumferential exfoliated esophageal mucosa (D, H&E, orig. mag. ×200), suggestive of pemphigus vulgaris (PV). In addition, the result of testing for anti–desmoglein-3 antibodies was positive. No other potential cause of the hematoma was found except PV. We also excluded other common reasons for the hematemesis. Therefore, the patient’s condition was diagnosed as PV with esophageal involvement. The patient was treated with 50 mg/d of prednisone, and all symptoms were relieved after 2 weeks. After the treatment, the patient received 3 follow-up gastroscopy examinations, with no hematoma found. PV with esophageal involvement is rather rare. The patient did not have any skin lesions and presented with intermittent hematemesis, which has not yet been reported, to our knowledge. This case reminds us that PV with esophageal involvement should be considered in patients with unexplained hematemesis, especially when gastroscopy shows esophageal hematoma and mucosal exfoliation even in the absence of skin lesions.